AJCM Winter 2012

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American Journal of Clinical MedicineÂŽ

Owned and Published by the American Association of Physician Specialists, Inc. Peer Reviewed. Listed in Google Scholar and BioMedLib

Winter 2012 • Volume Nine, Number One

Featured in this issue 8 The Rural Health Landscape in Jeopardy

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About Rural Health in America

11 Medical Education Cost & Debt Impacting Future Rural Physicians

14 Anaphylaxis: Diagnosis and Management in the Rural Emergency Department

22 Investigating a Rural Rotation in the Mississippi Delta: A Qualitative Study in Medical Education

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Preconception Counseling to Prevent the Complications of Obesity during Pregnancy


CALL FOR PAPERS American Journal of Clinical Medicine

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Owned and Published by the American Association of Physician Specialists, Inc.

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Primary Ma of the Liv ture Cystic Ter er: Rep ort of a atoma Rare Cas e The Rol e in Pelvic of Physician Exp Examin ation Acc erience uracy

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The American Journal of Clinical Medicine® (AJCM®) is the official, peer-reviewed journal of the American Association of Physician Specialists, Inc. (AAPS), an organization dedicated to promoting the highest intellectual, moral, and ethical standards of its members. Its diversity incorporates physicians that represent a broad spectrum of specialties including anesthesiology, dermatology, diagnostic radiology, disaster medicine, emergency medicine, family medicine/OB, family practice, geriatric medicine, hospital medicine, internal medicine, obstetrics and gynecology, ophthalmology, orthopedic surgery, plastic and reconstructive surgery, psychiatry, radiation oncology, general surgery, and urgent care medicine.

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To further the goals of AAPS, which include providing education for its members and promoting the study, research, and improvement of its various specialties, the AJCM® invites submissions of high-quality review articles, clinical reports, case reports, or original research on any topic which has potential to impact the daily practice of medicine. Publication in the AJCM® is one of the criteria to qualify for the prestigious Degree of Fellow within the Academies of Medicine of the AAPS.


AJCM

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Sounding Board: “Doctor, my legs are swelling” Thomas G. Pelz, DO

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Preconception Counseling to Prevent the Complications of Obesity during Pregnancy Merri M. Paden, MD Daniel M. Avery Jr., MD

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Heterotaxy Syndrome in a Young Adult Andres Abreu, DO, MS Maria Luisa Nieto-Morales, MD, Ph.D Margarita Fuentes, MD

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Daniel M. Avery Jr., MD Daniel M. Avery, III, MD John T. McDonald, Jr., MD Joseph C. Wallace, MD

In This Issue RURAL Health SECTION The Rural Health Landscape in Jeopardy

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Alan Morgan, CEO National Rural Health Association

About Rural Health in America From The National Organization of State Offices of Rural Health

Medical Education Cost & Debt Impacting Future Rural Physicians

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Medical Ethics Without the Rhetoric

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Adenocarcinoma Arising in a Three Millimeter-Thick Endometrium

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C. Cory Smith, RN, PhD(c)

Case Report: Fetal Heart Rate Misrepresented by Maternal Heart Rate: A Case of Signal Ambiguity Ikechukwu Emereuwaonu, MD

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M. Scott Linscott, MD

Investigating a Rural Rotation in the Mississippi Delta: A Qualitative Study in Medical Education

Mark Pastin, PhD

Daniel M. Avery, Jr., MD Daniel M. Avery, III, MD Marion D. Reed, MD Joseph C. Wallace, MD

C. Cory Smith, RN, PhD(c)

Anaphylaxis: Diagnosis and Management in the Rural Emergency Department

Case Report: Cellulitis Following Newborn Circumcision

Orthopedic Issues in Family & Emergency Medicine - Managing Common Fractures: The Heel Michael Jerkins, Medical Student Ravi Kumar Singh, MD

22

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Case Report: Unusual Presentation of Aggressive Neuroendocrine Tumor Brian W. Harris, PhD, MD, FAAR Jordan Gularek, DO


American Association of Physician Specialists, Inc.® 5550 West Executive Drive Suite 400 Tampa, Florida 33609-1035 Phone: 813-433-2277 Fax: 813-830-6599 www.aapsus.org

AAPS Board of Directors William G. Anderson, DO Scott G. Barnes, DO A. Robert Cerrato, DO, JD Michael A. Cohen, DO Brian J. Feaver, MD, FAASFP Kenneth M. Flowe, MD, FAAEP Joseph C. Gallagher, DO, FAASOS Edna Z. Mahmood, MD, FAASS Jerry R. Majers, DO, FAAIM, FAAGM Stephen A. Montes, DO, FAASOS Douglas L. Marciniak, D.O, FAAIM Thomas G. Pelz, DO, FAAIM Atwood L. Rice, III, MD, JD Anthony P. Russo, Jr., DO Susan M. Sarracino, MD Betsy B. Schenck, DO Craig S. Smith, MD Louis W. Sullivan, MD James W. Terbush, MD Martin E. Thornton, DO

AAPS Staff William J. Carbone Chief Executive Officer Nadine B. Simone Executive Assistant Andrea N. Balboa Assistant Director of Certification Christina Stebbins Manager of Test Development Susan LoBianco Certification Coordinator Marilyn D. Whitfield Certification Coordinator Maria F. Valente Certification Coordinator Esther L. Berg Director of CME, Meetings & Membership Keely M. Clarke CME, Meetings & Membership Coordinator Anthony J. Durante Director of Finance & Operations Georgine C. Wasser Finance & Operations Coordinator Debi S. Colmorgen Communications Coordinator Bruce Cotton Director of Governmental Affairs Lauren E. Withrow Governmental Affairs Coordinator James G. Marzano Director of Public Relations & Marketing Jeffery L. Morris, Jr., JD Director of Communications & External Affairs

W

elcome to the American Journal of Clinical Medicine® (AJCM®). This issue is focused on Rural Health Issues. The Journal is dedicated to improving the practice of clinical medicine by providing up-to-date information for today’s practitioners. The AJCM is the official journal of the American Association of Physician Specialists, Inc. (AAPS), an organization dedicated to promoting the highest intellectual, moral, and ethical standards of its members, and whose diversity incorporates physicians that represent a broad spectrum of specialties including anesthesiology, dermatology, diagnostic radiology, disaster medicine, emergency medicine, family medicine obstetrics, family practice, geriatric medicine, hospital medicine, internal medicine, obstetrics and gynecology, ophthalmology, orthopedic surgery, plastic and reconstructive surgery, psychiatry, radiation oncology, general surgery, and urgent care medicine. Part of the mission of the AAPS is to provide education for its members and to promote study, research, and improvement of its various specialties. In order to further these goals, the AJCM invites submissions of high-quality review articles, clinical reports, case reports, or original research on any topic that has potential to impact the daily practice of medicine. Publication of a peer-reviewed article in the AJCM is one of the criteria needed to qualify for the prestigious Degree of Fellow in the Academies of Medicine of the AAPS. Articles that appear in the AJCM are peer reviewed by members with expertise in their respective specialties. Manuscripts submitted for publication should follow the guidelines in The International Committee of Medical Journal Editors: “Uniform requirements for manuscripts submitted to biomedical journals” (JAMA, 1997; 277:927-934). Studies involving human subjects must adhere to the ethical principals of the Declaration of Helsinki, developed by the World Medical Association. By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of their article that might create any potential conflict of interest. More detailed information is included in the AJCM Manuscript Criteria and Information on pages 64 and 65. All articles published, including editorials, letters, and book reviews, represent the opinions of the authors and do not reflect the official policy of the American Association of Physician Specialists, Inc., or the institution with which the author is affiliated, unless this is clearly specified. ©2012 American Journal of Clinical Medicine® is published by the American Association of Physician Specialists, Inc. All rights reserved. Reproduction without permission is prohibited. Although all advertising material is expected to conform to ethical standards, acceptance does not imply endorsement by the American Journal of Clinical Medicine® and the American Association of Physician Specialists, Inc.

Editor-In-Chief

Wm. MacMillan Rodney, MD, FAAFP, FACEP

Senior Editor

Kenneth M. Flowe, MD, FAAEP

Managing Editor

Esther L. Berg, MEd

Editorial Board

Harold M. Bacchus, Jr., MD, FAAFP Gilbert Daniel, MD, FAAR Michael K. Garey, MD Beverly R. Goode-Kanawati, DO Thomas G. Pelz, DO, FAAIM Cyril H. Wecht, MD, JD

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AJCM is peer reviewed and listed in Google Scholar and BioMedLib


AJCM

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Welcome to the American Journal of Clinical Medicine® (AJCM®)-

Welcome to the first issue of the American Journal of Clinical Medicine® 2012. Since 1975, my goals have been to provide exemplary continuing health care for my patients and outstanding educational experiences for my students, residents, and colleagues. It is an honor and privilege to serve as editor of the American Journal of Clinical Medicine (AJCM). I have served as an author and/or editor for over 200 publications and compliment my predecessors and staff for establishing and maintaining the AJCM. Thank you. Authors who produce quality manuscripts must be solicited, encouraged, nurtured, and published. Writing is a labor of love. The only thing more difficult than writing is rewriting. Welcome to the world of publication. All will benefit through the prosperity of the AJCM enterprise. In 2009, I proposed a circular hierarchy of seven activities. These activities are: patient care, education, scholarship, administration, ambassadorship, leadership, and legacy. The AJCM is now being cited as part of the international community of scholars. Our 2009, 2010, and 2011 editions are listed by Google Scholar and BioMedLib. These internet search engines are more extensive than PubMed. This gives published authors international recognition as achieving a universal academic standard of excellence. With this membership, AJCM joins the major leagues of science, and published work will last as long as our civilization does. WE must fund our efforts and make the transition to multimedia via the internet. This will require committed editorial board members who unselfishly devote themselves to timely peer review of manuscripts. WE must maintain an impeccable reputation for quality and integrity. WE should be part of a national campaign supporting recognition of the AJCM as a medical journal offering useful information for the practicing physician. There so many worthy causes, but the AJCM should be a journal which emphasizes the issues which make the AAPS so important for physicians who have been challenged by training cartels and economic credentialing monopolies. Our research should demonstrate the role of privilege based on MERIT. The AAPS and the AJCM will develop a nationally recognized leadership position through recognition of its authors and their published research. In keeping with the 2011 link between the AAPS and the National Rural Health Association, the AJCM 2012 will focus its efforts on building ABPS, the logical solution for rural communities and frontier hospitals. These include mission hospitals. Two special groups in ABPS merit mention – BCEM (Board of Certification in Emergency Medicine) and BCFMOB (Board of Certification in Family Medicine and Obstetrics). As co-director of a fellowship in Emergency Medicine (BCEM), in 1992 the faculty recognized that the AAPS was the only organization with the vision for this innovation. In 1999, other faculty designed and built a private practice hoping to improve upon the seemingly insolvable problems witnessed during my career as a Professor and Chair in several medical schools. This clinical group, Medicos, is built on a foundation of family medicine but combines Public Health, OB, cesareans, emergency care, office surgery, and primary care. This identity is surgical family medicine obstetrics, and we relate to the ABPS Board of Certification in Family Medicine and Obstetrics. Without grants or charity, these faculty groups fund an active teaching program and clinical research encompassing almost all of the established specialties. AAPS, ABPS, and AJCM will strive for leadership in providing physicians for these underserved communities and developing countries. “Medicos-where 10 percent of the information makes over 90 percent of the difference and where, through Grace, twice the service is provided at less than half of the cost.”

Wm. MacMillan Rodney MD, FAAFP, FACEP Editor, American Journal of Clinical Medicine® Member, American Board of Family Medicine Obstetrics


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American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One


Rural Health is Important to AAPS

The Rural Health Landscape in Jeopardy Alan Morgan, CEO National Rural Health Association 

About Rural Health in America From The National Organization of State Offices of Rural Health 

Medical Education Cost & Debt Impacting Future Rural Physicians C. Cory Smith, RN, PhD(c) 

Anaphylaxis: Diagnosis and Management in the Rural Emergency Department M. Scott Linscott, MD 

Investigating a Rural Rotation in the Mississippi Delta: A Qualitative Study in Medical Education C. Cory Smith, RN, PhD(c) 


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American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

The Rural Health Landscape in Jeopardy Alan Morgan, CEO National Rural Health Association

Rural America is not simply a “small” version of urban America. It is a unique health care delivery environment. Rural is that place where those most in need of health care services oftentimes have the fewest options available when it comes to accessing care. Sixty-two million Americans live and work in rural areas of the United States, and a substantial number of these areas are federally designated as Health Profession Shortage Areas. The challenge from a national perspective is how best to address the health care needs of this important population group. Unfortunately, with the current national fiscal crisis, we may be headed in the wrong direction. The Budget Control Act of 2011 calls for a $1.2 to $1.5 trillion reduction in federal expenditures over the next 10 years. The Joint Select Committee on Deficit Reduction – or “super committee” – has been formed and tasked with developing policies that will produce these reductions. And rural health has been targeted in various proposals to this committee and to Congress as a whole. The specific payment reductions advocated by each proposal vary, and a number of groups have put forward various ideas. Proposals include: • The Congressional Budget Office (CBO): Eliminate alternative hospital designations: Critical Access Hospital (CAH), Sole Community Hospital (SCH), and Medicare Dependent Hospital (MDH). This plan would reduce hospital payments by $3.8 billion in fiscal year (FY) 2012 increasing to $9.5 billion in reduced payments in FY 2021. Total cut to rural facilities over 10 years: $62.2 billion.

• President Obama: Starting in FY 2013, the President’s plan would end add-on payments for physicians and hospitals in frontier states, reduce CAH reimbursement to 100% of reasonable cost, and end CAH reimbursement for facilities located ten miles or less from another hospital. Total cut to rural facilities over 10 years: $6 billion • House Republican leadership: While specifics of the proposal were not released, House Republican leadership sought to cut $2 billion from frontier state add-on payments and $14 billion from rural hospital reimbursement structures. Total cut to rural facilities over 10 years: $16 billion. • Ways and Means Democratic staff: The Democratic staff from the House Ways and Means Committee embraced the CBO recommendation. They erroneously argued that the elimination of these payment structures would be more equitable and in line with other payment reforms. Total cut to rural facilities over 10 years: $62.2 billion. • Sequestration: If the super committee fails to produce sufficient savings, Medicare reimbursements will be “sequestered.” An automatic cut of two percent will be instituted on all providers. Total cut to rural facilities over 10 years: $5.9 billion. These various proposals to the super committee include billions in reimbursement cuts to rural health care. These proposals erroneously claim that they eliminate “higher than necessary reimbursement” to rural facilities. However, small rural hospitals, known as Critical Access Hospitals (CAHs), account for only 5.3 percent of all hospital spending while providing care for 8.7% of Medicare adjusted patient days. Proposed cuts will exacerbate significant funding shortfalls for rural hospitals that are providing efficient services to the most vulnerable beneficiaries.


American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

Currently, 41% of all CAHs operate at financial lose. If these proposals to cut billions in Medicare reimbursements go into effect, many more CAHs will lose money. Some will have to close their doors, further jeopardizing access to inpatient and emergency care. Congress created the CAH designation in 1997 to prevent hospital closures, like those that occurred in the 1980s and 1990s. In those two decades, 360 rural facilities closed. From 2000 to 2009, only 43 CAHs closed proving that the CAH program is a safety net program that is working. CAHs are vital access points for rural seniors who are, per capita, older, poorer, and sicker than their urban counterparts. Recent reports show that Medicare contributes over 40% of all revenue to CAHs, compared to 32% for urban facilities. Medicare cuts to rural hospitals will disproportionately harm these vital safety net facilities.

The National Rural Health Association (NRHA) and its more than 21,000 hospital, physician, practitioner, and patient members are urging Congress to protect the rural health care safety net. Again, this fight is about much more than simply the existence of rural hospitals. It’s about the future of health care access in small towns across America. It’s not an easy fight, but most certainly a cause worth fighting for as a nation. For more information on NRHA’s efforts to protect rural health, visit www.RuralHealthWeb.org. Alan Morgan is CEO of the National Rural Health Association (NRHA) located in Washington, DC. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

Are You Published? Had any interesting cases lately?  Consider submitting your Case Reports to the American Journal of Clinical Medicine® (AJCM®).  Case Reports are one of the AJCM’s most popular features. Case Reports are short, didactic case studies of 1,000 – 1,500 words. They are easy to write and AJCM readers love them.  Submit your Case Reports and other articles to the AJCM. Articles are cited in GoogleScholar.com and BioMedLib.com. We also welcome your . . . • Review articles

• Clinical reports

• Original research

Sounding Board and Letters to the Editor are your opportunities to share your thoughts, concerns, or suggestions to the AJCM readers. Publication in the AJCM is one of the criteria to qualify for the prestigious Degree of Fellow within the AAPS Academies of Medicine.

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American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

About Rural Health in America General Statistics • Approximately 62 million people – nearly one in five Americans – live in rural and frontier areas. • Rural Americans reside in 80 percent of the total U.S. land area but only comprise 20 percent of the U.S. population. • There are 4,118 primary care Health Professional Shortage Areas (HPSAs) in rural and frontier areas of all U.S. states and territories compared to 1,960 in metropolitan areas. • The average median income for rural U.S. residents is $40,615 compared to $51,831 for urban residents. • Approximately 15.4 percent of rural U.S. residents live in poverty compared to 11.9 percent of urban residents.

Rural Health Workforce • There is a more holistic, patient-centered approach to health care in rural communities – providers have the opportunity to provide more comprehensive care to their patients. • Despite this opportunity, only nine percent of all physicians and 12 percent of all pharmacists practice in those settings. • There were 55 primary care physicians per 100,000 residents in rural areas in 2005, compared with 72 per 100,000 in urban areas – a figure which decreases to 36 per 100,000 in isolated, small rural areas. • There are only half as many specialists per 100,000 residents in rural areas compared to urban areas.

Healthcare/Health Insurance Accessibility • While nearly 85 percent of U.S. residents can reach a Level I or Level II trauma center within an hour, only 24 percent of residents living in rural areas can do so within that time frame – this despite the fact that 60 percent of all trauma deaths in the United States occur in rural areas,. • Approximately 21.9 percent of residents in remote rural counties are uninsured, compared to 17.5 percent in rural counties adjacent to urban counties and 14.3 percent in urban counties. • Rural residents spend more on health care out of pocket than their urban counterparts; on average, rural residents pay or 40 percent of their health care costs out of their own pocket compared with the urban share of one-third. One in five rural residents spends more than $1,000 out of pocket in a year.

Rural Hospitals • Rural hospitals are sources of innovation and resourcefulness that reach beyond geographical boundaries to deliver quality care. They are also typically the economic foundation of their communities – every dollar spent on rural hospitals generates about $2.20 for the local economy. • Twelve percent of rural hospitals indicate they are not considering HIT investments because of cost concerns compared to 3 percent of urban hospitals.

• Rural areas average about 30 dentists per 100,000 residents; urban areas average approximately twice that number.

• Critical Access Hospitals care for a higher percentage of Medicare patients than other hospitals because rural populations are typically older than urban populations.

• Only 10 percent of psychologists/psychiatrists and 20 percent of masters-level social workers work in rural areas.

State Offices of Rural Health • All 50 states maintain a State Office of Rural Health (SORH). • SORHs serve as a clearinghouse of information and innovative approaches to rural health services delivery; coordinate state activities related to rural health in order to avoid duplication of efforts and resources; and identify Federal, State and nongovernmental programs that can provide technical assistance to public/private nonprofit entities serving rural populations. • Collectively, SORHs provided technical assistance to more than 28,000 rural communities during the 2010-2011 fiscal year. From The National Organization of State Offices of Rural Health


American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

Medical Education Cost & Debt Impacting Future Rural Physicians C. Cory Smith, RN, PhD(c)

The concept of a rural physician shortage is not a recent discovery. The shortage has existed for more than 80 years despite the fact that, in general, people living in rural areas have a greater need for medical care, being older, sicker, and poorer than their non–rural peers.1,2 The American Association of Medical Colleges suggests the future rural physician workforce is likely to decline even further, with only three percent of recent medical students planning to practice in small towns and rural areas.3 Whitcomb argues the factors associated with this decline include the decreasing number of physicians entering family medicine and primary care, the changing lifestyle preferences of younger physicians, and the increasing level of medical student debt.4 In 1984, 87% of medical students had debt at an average of $22,000 for those attending public medical school and an average of $27,000 for students attending private school.5 In 2007, 87% of medical students had debt at an average of $145,000 for students attending public medical schools and an average of $180,000 for students attending private schools.5 Recent research found that approximately 23% of medical students, a number that in 2008 was more than double the percentage in 2004, have more than $200,000 in debt.5 Jolly suggests that if debt levels continue to increase at current rates, graduates of the class of 2033 will use more than 25% of their after–tax income to repay medical school loans for 25 working years.6 As of now there is inconclusive evidence that shows a strong definite relationship between medical school debt and career choice. However, Weeks and Wallace maintain it is common knowledge that primary care careers have a lower financial return on investment in education than medical and surgical specialization.7 Residency programs of higher income special-

ties consistently fill with US medical graduates at higher rates, suggesting that US students are sensitive to income in making career choices. Phillips, Weismantel, Gold, and Schwenk suggest that students with high debt may also be influenced both toward and away from primary care specialties, because primary care physicians have lower salaries than specialists but typically shorter residency training.8 Numerous scholars have tried to explore the role of medical school training experiences, educational debt, and specialty reimbursement differences in hopes of understanding the complex interplay of factors that go into choosing a career in primary care.9 One study conducted by Palmeri et al. at Dartmouth University analyzed the economic impact of a primary care career.9 The research team used economic modeling, which employed a variety of factors, to develop a net income and expense model. Palmeri attempted to account for the variability of factors by looking at best, worst, and average expense scenarios. Palmeri also used published retrospective data from the Bureau of Labor Statistics, the 2007 Physician Compensation Survey, the National Association of Realtors, the College Board and US News and World Report regarding medical student debt, physician reimbursement, retirement planning, college savings, and cost-of-living expenses to develop their models. Palmeri reported results that showed primary care physicians in the first three to five years following residency will continue to have expenses that exceed their earnings. The study conducted by Palmeri aligns with those in recent publications noting that primary care physicians do not begin to have a positive cumulative net worth until approximately age 33.10 Palmeri also stated that the first few years after residency will be financially lean but feasible, except in the high expense mod-

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American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

el, if the newly practicing physician reins in expenses by extending educational debt repayment from 10 to 20 years. However, Palmeri points out two caveats to their findings. First, the primary care physician should have a starting wage at approximately $130,000. Secondly, their income quickly has to increase to the mean of $188,272. Consequently, Palmeri states that physicians cannot make the decision to follow a primary care career path lightly. Palmeri argues that the declining interest in the gatekeeping disciplines of internal medicine, family medicine, and pediatrics suggests that students intuitively grasp this problem, thus making the career choice disincentives explicit. Palmeri adds that sub-specialization allows a budget surplus for discretionary spending, ranging from modest to substantial, and the financial situation of those who pursue subspecialization contrasts sharply with the dilemma of primary care physicians who face difficult economic choices. With evidence that supports the increase in cost of a medical education affecting specialty career choices, one must also question the implications of this increase affecting rural career choices. Moreover, literature was included that not only focused on present-day medical students but included the future medical school class composition. Magnus and Mick argued that high tuition may restrict access to medical education for those from low-income families, underrepresented minority groups, and residents of rural areas.11 Furthermore, Bergen emphasized the advantages of a diverse, representative medical student body.12 Bergen agreed with Magnus and Mick in that Bergen felt that the advantages of creating a diverse student body are often cited as showing that students from underrepresented groups are more likely to treat ethnic minorities, practice in rural communities, and work in socioeconomically depressed areas.12 Research shows that medical students from rural backgrounds are more likely to eventually practice in rural communities.13 Woloschuk, Lemay, and Wright from the University of Calgary, School of Medicine, focused research on the financial state of medical students from rural backgrounds during tuition fee deregulation in Canada.14 Dhalla, Kwong, and Streiner stated that the proportion of students with rural backgrounds who apply to medical school is not reflective of the population that resides in rural Canada.15 Across Canada, tuition fees for medical schools have been on the rise.14 In a study including twelve Canadian medical schools Dhalla et al. found that a greater proportion of students from rural backgrounds came from families of lower socioeconomic status (parental income < $40,000). The results also discovered that the mean projected education debt of graduation was significantly greater for rural background medical students compared with their metropolitan peers.14 Furthermore, Woloschuk et al. also noted that medical students from rural areas had significantly greater education debt at entry than medical students from metropolitan backgrounds. Woloschuk et al. maintain that rising tuition may not only serve as a deterrent to qualified candidates but may limit the diversity of medical students. Duffin suggested in prior work that this lack of

diversity ultimately has implications for the profession’s ability to understand and solve major health issues.16 Woloschuk et al. also stated just as tuition fees increase, a threshold may eventually be reached whereby some students may consider the financial realities of entering medical school to be overwhelming. The findings of this research also discovered that medical students from rural backgrounds were significantly older than their peers from metropolitan backgrounds, suggesting that it takes students longer to attain medical school entry requirements and the finances to proceed with medical training.14 Woloschuk et al. suggested the rise in medical school tuition may be especially detrimental to students from rural backgrounds, as financial support from parents may not be as readily available to these students as it is to their non-rural peers. Interestingly, the above-mentioned Canadian-based study is in concordance with older US data that shows as tuition fees rose in the 1970s and 1980s, the socioeconomic status (SES) of students enrolling in medical school increased.17 In fact, Rosinski conducted a study in 1965 that found that medical schools with the lowest fees attracted the greatest number of students with a lower SES.18 Ready and Nickens, health policy educators at the University of Michigan, suggest that medical school admissions policies involve a paradox regarding student diversity.19 Ready and Nickens also stated that medical school administrators have launched campaigns to recruit more ethnic minority medical students. As suggested above, the key motivation has been the growing evidence that minority physicians disproportionately serve poor and ethnic minority communities.20 Magnus et al. felt that committees stress racial diversity but downplay socioeconomic diversity. Magnus et al. also hypothesized that recruiting more physicians from disadvantaged backgrounds would improve access to health care because a disproportionate percentage of such physicians would establish practices in their home communities. In comparison to the Canadian study conducted by Woloschuk et al., US medical students with a lower SES also suffer from high amounts of undergraduate debt.11 Takagi argued that access to postsecondary education has declined for disadvantaged students in part as a result of more stringent federal criteria for determining who is financially independent and thus eligible for more aid.21 Therefore, in most instances it may not be the fault of many medical school admissions committees for not choosing to diversify their student bodies, but rather some disadvantaged students being deterred from the potentially overwhelming medical education debt. In addition, Ballantine argued that the higher admissions requirements and expectations have naturally led some medical schools to recruit students from wealthier geographic areas, which in most instances are non-rural areas.22 C. Cory Smith, RN, PhD(c), is the Director of Performance Improvement at the Mississippi State Department of Health and a doctoral candidate in the School of Graduate Studies in Health Sciences at the University of Mississippi Medical Center in Jackson, Mississippi.


American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

References 1.

Pusey WA. Medical education and medical service, I: The situation. JAMA. 1925;84:281-285.

2.

Human Resources and Services Administration, 2011. HPSA by state and county. Retrieved from http://hpsafind.hrsa.gov/HPSASearch.aspx.

3.

Association of American Medical Colleges, 2005. Association of American Medical Colleges Matriculation Questionnaire 2005: All Schools. Retrieved from http://www.aamc.org/data/msq/allschoolreports/ msq2005.pdf.

and wealth gap between specialists and primary care physicians? Health Affairs. 2010;29:933-940. 11. Magnus SA, Mick SS. Medical schools, affirmative action, and the neglected role of social class.American Journal of Public Health. 2000;90:1197-1201. 12. Bergen SS. Underrepresented 2000;284:1138-1139.

minorities

in

medicine.

JAMA.

13. Rabinowitz HK, Diamond JJ, Veloski J, Gayle JA. The impact of multiple predictors on generalist physicians’ care of underserved populations. The American Journal of Public Health. 2000;90(8):1225-128. 14. Woloschuk W, Lemay JF, Wright B. What is the financial state of medical students from rural backgrounds during tuition fee deregulation. Canadian Journal of Rural Medicine. 2010;15(4):156-160. 15. Dhalla IA, Kwong JC, Streiner DL. Characteristics of first-year students in Canadian medical schools. Canadian Medical Association Journal. 2002;166:1029-35.

4.

Whitcomb ME. (2005). The challenge of providing doctors for rural America. Academic Medicine. 2005;80:715-716.

5.

Association of American Medical Colleges, 2011. Association of American Medical Colleges Matriculation Questionnaire 20011: All Schools. Retrieved from http://www.aamc.org/data/msq/allschoolreports/ msq2011.pdf.

6.

Jolly P. Medical School Tuition and Young Physicians Indebtedness. Health Affairs. 2007;24(2):527-535.

18. Rosinski EF. Social class of medical students. A look at an untapped pool of possible medical school applicants. JAMA.1965;193:95-98.

7.

Weeks WB, Wallace AE. The more things change: revisiting a comparison of educational costs and incomes of physicians and other professionals. Academic Medicine. 2002;77:312-319.

19. Ready T, Nickens H. Affirmative action and project 3000 by 2000. Academic Medicine. 2000;71:656-657.

8.

Phillips J, Weismantel D, Gold K, Schwenk T. Medical student debt and primary care specialty intentions. Family Medicine. 2010;42(9): 616-622.

9.

Palmeri M, Pipas C, Wadsworth E, Zubkoff M. Economic impact of a primary care career: a harsh reality of medical students and the nation. Academic Medicine. 2010;85(11):1692-1697.

10. Vaughn B, DeVrieze S, Reed SD, Schulman KA. Can we close the income

16. Duffin J. What goes around comes around: a history of medical tuition. Canadian Medical Association Journal. 2001;164:50-6. 17. Kassebaum DG, Szenas PL, Schuchert MK. On rising medical student debt: in for a penny, in for a pound. Academic Medicine. 1996;7:11241134.

20. Tekian A. A thematic review of the literature on underrepresented minorities and medical training, 1981-1995: securing the foundations of the bridge to diversity. Academic Medicine. 1997;71:140-146. 21. Takagi DY. We should not make class a proxy for race. Chronicle of Higher Education. 1995;41:52. 22. Ballatine JH. The sociology of education: A systematic analysis. (3rd ed.). Englewood Cliffs, New Jersey: Prentice Hall Inc;1993.

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Anaphylaxis: Diagnosis and Management in the Rural Emergency Department M. Scott Linscott, MD

Abstract It is not uncommon for patients to present to a rural emergency department with one of the syndromes of anaphylaxis: urticaria, angioedema, and severe anaphylaxis. Therefore, it is important for the rural health care provider to be able to recognize these anaphylaxis syndromes and manage them appropriately. Urticaria and/or angioedema may present as isolated entities or may be combined with asthma and/or hypotension to produce severe anaphylaxis. The mainstays of treatment of urticaria are the H1 and H2 receptor anatagonists, most effective when given in combination intravenously. Acute angioedema may occur with or without urticaria and is treated with epinephrine. In severe anaphylaxis, urticaria and/or angioedema are accompanied by acute bronchospasm and or hypotension. Immediate therapy with parenteral epinephrine as well as rapid fluid resuscitation is critical for its successful management. Patients with angioedema or severe anaphylaxis should be given epinephrine auto-injectors prior to discharge. Follow-up with an allergist is recommended for most patients with anaphylaxis.

Introduction A 16-year-old female was diagnosed as having acute cystitis. She was given sulfamethoxazole trimethaphan double strength one b.i.d. for three days. On the second day of therapy she presented to the emergency department with a generalized patchy erythematous, raised rash involving her neck, chest, back, abdomen, and all extremities. The rash was very pruritic. A 35-year-old male had a history of lip and tongue swelling when eating peanuts. He was offered a cookie by his aunt who

assured him that she had used no peanuts in its preparation. Within five minutes he developed swelling of his face, lips, and tongue. His aunt rushed him to the emergency department. Upon arrival in the emergency department his voice was very hoarse and he was beginning to develop inspiratory stridor. A 23-year-old male IV drug abuser presented to the emergency department with an abscess on his left arm with surrounding cellulitis. He had a history of developing “hives” to some antibiotic, but he did not remember the name of the antibiotic. The abscess was incised and drained, and the patient given cefazolin 1 g IV and Bactrim DS 2 tablets orally. Ten minutes later he complained of diffuse itching, and his skin became very erythematous. The patient was wheezing, his blood pressure decreased from 130/82 to 70/30, and his pulse increased from 70 to 140 beats per minute. These patients may all present to a rural emergency department, and all represent varying degrees of anaphylaxis: urticaria, angioedema, and severe anaphylaxis (with asthma and/ or anaphylactic shock). Therefore, it is important that the rural health care provider understand their pathophysiology, clinical presentation, differential diagnosis, and management. According to a multidisciplinary panel of experts who met in 2006, anaphylaxis is defined as a serious allergic reaction that is rapid in onset and may cause death. This same group published new diagnostic criteria for anaphylaxis.1 This group established three criteria, each reflecting a different clinical presentation of anaphylaxis. They stated that anaphylaxis is very likely when any one of the following criteria is met:


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Criterion 1 – Acute onset of an illness (over minutes to several hours) involving the skin, mucous membranes, or both (e.g., generalized urticaria, pruritis or flushing with swelling of the lips, tongue, or uvula) and at least one of the following: a. Respiratory compromise (e.g., wheezing/bronchospasm, stridor, dyspnea, hypoxemia, and decreased peak expiratory flow) b. Decreased blood pressure or associated symptoms of end-organ dysfunction (e.g., syncope, hypotonic collapse, incontinence) Criterion 2 – Two or more of the following that occur rapidly after exposure to an allergen that is likely for that patient (may occur minutes to hours after allergen exposure): a. Involvement of the skin and/or mucous membranes (e.g., generalized urticaria, flushing with pruritis, and swollen lips, tongue, uvula) b. Respiratory compromise (e.g., wheezing/bronchospasm, stridor, dyspnea, hypoxemia, and decreased peak expiratory flow) c. Decreased blood pressure or associated symptoms (syncope, hypotonia, collapse, incontinence) d. Persistent gastrointestinal symptoms (e.g., vomiting, cramping, abdominal pain) Criterion 3 – Decreased blood pressure after exposure of a known allergen for that patient (minutes to hours). Decreased blood pressure is defined in adults as a systolic BP of less than 90 mmHg or >30% decrease from that patient’s baseline. In infants and children, decreased BP is defined as low systolic BP of less than 70 mmHg from one month up to one year; less than (70mmHg + [2 x age]} from one to ten years; less than 80 mmHg from 11 to 17 years. The author disagrees with this definition of anaphylaxis for the following reasons: 1) This definition is much too restrictive. Some patients may present with generalized urticaria and go on to develop angioedema and/or severe anaphylaxis over minutes to hours. Other patients may present with generalized urticaria and never go on to develop the more serious syndromes that meet the expert panel’s criteria for the diagnosis of “anaphylaxis.” 2) It is more reasonable to consider all of the IgE-mediated syndromes (urticaria, angioedema, and severe anaphylaxis) as “anaphylaxis,” even though some patients with urticaria and angioedema may not fulfill all the criteria for “anaphylaxis” recommended by the expert panel. Therefore, this article will discuss the pathophysiology, clinical presentations, differential diagnosis, and management of all three syndromes of anaphylaxis: acute urticaria, acute angioedema, and severe anaphylaxis (asthma and/or hypotension [anaphylactic shock]). Emphasis will be on clinical presentations, differential diagnosis, and management, since these are the most important for the physician practicing in a rural environment.

ACUTE URTICARIA Pathophysiology Acute urticaria results from the release of histamine, bradykinin, leukotriene C4, and other vasoactive substances from mast cells and basophils in the dermis. These substances cause extravasation of fluid into the dermis that lead to the formation of the urticarial “wheal.” The intense pruritis is caused by the histamine released into the dermis. Histamine is the ligand for two membrane-bound receptors, the H1 and H2 receptors. The activation of the H1 receptors on endothelial cells in the capillaries leads to increased capillary permeability and results in plasma leaking from the capillaries, resulting in the urticarial wheal. The activation of the H2 histamine receptors in the arterioles and venules leads to arteriolar and venule dilation, adding to the capillary leak and increasing the size of the urticarial wheal.2 There are several mechanisms causing this process. The socalled “Type 1” response is initiated by antigen-mediated IgE immune complexes that bind and cross-link Fc receptors on the surface of mast cells and basophils, thus causing degranulation and histamine release. This type of urticaria is the most common and may be caused by infections, insect stings, foods (particularly shellfish, eggs, nuts, berries, chocolate, cheeses, and tomatoes), drugs (NSAIDs, penicillins, sulfonamides, codeine, etc.), latex exposure, emotional stress, exercise, and pregnancy (e.g., pruritic urticarial papules and plaques of pregnancy [PUPPP]). The so-called “Type 2” response is mediated by cytotoxic T cells that cause deposits of immunoglobulins, fibrin, and complement around arterioles leading to urticarial vasculitis. The so-called “Type 3” response is caused by immune complexes than cause the urticaria and is seen in patients with systemic lupus erythematosis and other autoimmune diseases. The type 3 response is often chronic and recurrent. To date, no reliable tests exist to identify if these chronic urticarias are autoimmune or non-autoimmune in a specific patient.3 Complement-mediated urticarias include viral and bacterial infections, serum sickness, transfusion reactions, certain drugmediated reactions (opioids, succinylcholine, vancomycin, and other drugs) as well as radiocontrast agents. In the physical urticarias, some physical stimulus causes urticaria. These stimuli include immediate pressure urticaria, delayed pressure urticaria, cold urticarial, and cholinergic urticarial.8 The remainder of this discussion will be limited to IgE-mediated acute urticaria, the least life-threatening of the anaphylaxis syndromes.

Clinical Presentation Patients with urticaria (or “hives,” the lay term for urticaria) have varying clinical presentations. Patients receiving opioids intravenously may have an urticarial eruption in the distribution of the vein proximal to the injection site. Patients with known allergens, who are exposed to these allergens, may pres-

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ent with a diffuse urticarial rash covering almost their entire body associated with wheezing and tightness in the throat. The urticarial lesions are usually raised and intensely pruritic. They may present as multiple isolated lesions, or the lesions may become confluent, producing a very large “wheal.” The lesions are usually intensely pruritic. Urticarial lesions that are painful are suggestive of urticarial vasculitis. Urticaria is diagnosed clinically, based on a detailed history and finding the classical urticarial wheals on physical examination.4 An attempt should be made to elicit signs and symptoms of a generalized allergic reaction. A history of a hoarse voice or throat tightness, nausea, vomiting, or crampy abdominal pain may suggest angioedema in addition to the urticarial.5 Symptoms such as chest tightness, wheezing or difficulty breathing may suggest a more severe, generalized anaphylactic reaction. Symptoms such as lightheadedness with hypotension would also suggest a more severe anaphylactic reaction such as anaphylactic shock.6 Patients should be queried regarding a previous history of urticaria in the past as well as a history of systemic diseases, such as lupus erythematosus, that may be associated with urticaria. Also, a history of unusual food intake as well as recent drug exposure, use of new hair dyes, or insect stings should be sought out. Physical examination should include looking for swelling of the lips, tongue, or uvula as well as careful auscultation of the lungs for wheezing. Also, the patient’s vital signs should be taken immediately and, if the patient is hypotensive, large bore IVs as well as systemic epinephrine should be considered.7

Differential Diagnosis There are several conditions that may mimic various features of urticaria. The presence or absence of pruritus may be used to narrow the differential diagnosis. Nonpruritic conditions that may mimic urticaria are viral exanthems, such as erythema infectiosum (fifth disease), measles, and the rash associated with Epstein-Barr viral infection. Also, Sweets syndrome is associated with a nonpruritic rash similar to urticaria.8 Pruritic conditions that may be confused with urticaria include contact dermatitis, atopic dermatitis, insect bites, erythema multiforme, toxic drug eruptions, and cutaneous small vessel vasculitis.8,9

Management Urticaria Without Symptoms or Signs of Angioedema or Anaphylactic Shock 1. H1 and H2 antihistamines. If the urticaria is mild, oral therapy with the first generation (sedating) H1 antihistamines, such as diphenhydramine (Benadryl, etc.), chlorpheniramine (Chlor-Trimeton, etc.), and hydroxyzine (Vistaril, Atarax, etc.), or second gen-

eration (“non-sedating”) H1 antihistamines, such as cetirizine (Zyrtec), levocetirizine (Xyzal), loratadine (Claritin, Tavist, etc.), and fexofenadine (Allegra), may resolve the urticarial.10 However, if these oral drugs do not resolve the urticaria, giving diphenhydramine intravenously (12.5-25mg in adults) combined with an H2 antihistamine IV (ranitidine [Zantac] 50mg or famotidine [Pepcid] 20mg) has been demonstrated to be more effective in treating acute urticaria than the H1 antihistamines given IV alone.11,12 2. Glucocorticoids. Although probably not necessary in many cases of acute, non-vasculytic urticaria, these drugs have been shown to decrease itching and lead to a more rapid resolution of the urticaria than placebo, when used in combination with hydroxyzine.13 3. Epinephrine. There are no studies that show a benefit of epinephrine in acute urticaria that is not associated with angioedema or severe anaphylaxis with asthma and/or anaphylactic shock.

Urticaria With Symptoms of Angioedema and/or Asthma and/or Anaphylactic Shock 1. With angioedema. These patients should be treated with the combination of H1 and H2 antihistamines IV in addition to intramuscular epinephrine (0.3-0.5mg). The antihistamines alone are not effective in treating angioedema.14 2. With asthma. These patients probably have systemic anaphylaxis and should be given epinephrine 3-0.5 mg IM as well as albuterol nebulization in addition to the H1 and H2 antihistamines. 3. With hypotension (anaphylactic shock). These patients should be treated with very large volumes of crystalloid (NS) as well as an infusion of epinephrine IV (see severe anaphylaxis management below). If the urticaria does not respond to the epinephrine, intravenous H1 and H2 blockers may be employed (see management of severe anaphylaxis management below).

Disposition and Follow-up 1. If the patient has only acute urticaria and responds to therapy with H1 and H2 blockers (cessation of itching and the urticarial wheals should have become less intensely erythematous, but may still be present), they should be observed for 30-60 minutes. If they remain pruritis free and the wheals have not reappeared or become more erythematous, they may be discharged on a regimen of H1 and H2 blockers orally (hydroxizine 50 mg q 6 hours for 24 hours and then q 6 hours prn itching with ranitidine 150 mg bid for 24 hours prn itching. Although recommended by some authors, the H2 blockers have not been demonstrated to add to the effect of the H1 blockers when given orally, although


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randomized controlled trials have not been published. Also, because of the results of a single trial, many “experts” recommend giving prednisone orally in a tapering daily dose of 40-40-20-20-10-10mg and off.13 2. If the patient has angioedema in addition to the urticaria, particularly if it involves the larynx, the patient should be observed for two to four hours to be certain that the angioedema does not recur and, if discharged, treated as above for urticaria plus the patient should be given two epinephrine auto-injectors or their equivalent to take home with them and told to use them should they become hoarse. Another option would be to admit the patient to the hospital under observation status, although this is not necessary if the patient has isolated urticaria/angioedema that has completely resolved with antihistamine and epinephrine therapy.14 3. If the patient has urticaria plus severe anaphylaxis (asthma, hypotension, etc.), the patient should be admitted to a monitored unit (an ICU, if available) because of the possibility of biphasic anaphylaxis (see below). 4. If discharged, the patient should follow up with their primary care provider within one week. The primary care provider may elect to refer the patient to an allergist for further work-up to determine if the patient has allergens that could have precipitated the urticaria.

Angioedema Pathophysiology Angioedema is a localized subcutaneous or submucosal swelling resulting from extavasation of fluids into interstitial tissues. Angioedema, like urticaria, may occur in isolation or may be accompanied by urticaria, asthma, or anaphylactic shock. Angioedema may be thought of as urticaria of deeper tissues, rather than involving the dermis and epidermis. Much of the pathophysiology of urticaria also applies to angioedema. Exposure of the vasculature to inflammatory mediators causes venule dilation and increased permeability of capillaries. Angioedema results from loss of vascular integrity allowing intravascular fluids to extravasate locally into the skin and subcutaneous tissues, especially the mucous membranes. The known causes of angioedema can be divided into three groups, depending upon the underlying mechanism: 1. Mast cell-mediated etiologies in which angioedema results from release of mast cell mediators that increase vascular permeability. Mast cell-mediated angioedema is associated with urticaria and or pruritus in most cases.15 2. Bradykinin-mediated etiologies in which angioedema results from the generation of bradykinin and complement-arrived mediators that increase vascular permeability. This form of angioedema may be caused by ACE-inhibitors and is not associated with urticaria

and or pruritus and is treated differently than the IgEmediated angioedema.16 3. Etiologies of unknown mechanism. Mast cell-mediated etiologies are similar to those of urticaria. Activated mast cells release inflammatory mediators, including histamine, heparin, leukotriene C4, and prostaglandin D2, which causes dilation of the venules in the dermis and enhance capillary permeability, with resultant tissue edema. One of the mast cell-mediated etiologies is allergic reactions. Acute angioedema, with or without other symptoms of allergic reactions may be triggered by drugs, latex, foods, exercise, insect stings, etc. Angioedema resulting from allergic reaction is usually accompanied by other signs and symptoms, including urticaria, pruritus, flushing, throat tightness, bronchospasm, and hypotension. However, allergic angioedema may occur in isolation, although this is uncommon.15 Angioedema may also be due to direct release by mast cells of inflammatory mediators by certain medications such as opioids and radiocontrast media. This type of angioedema is accompanied by urticaria in most cases. IgE is not involved, and skin testing or in vitro testing is not helpful in identifying a cause. Nonsteroidal anti-inflammatory drugs, such as aspirin and ibuprofen, may cause acute urticaria/angioedema. This adverse effect of NSAIDs is believed to be due to the pharmacologic properties of the medications on mast cells. NSAID administration results in increased formation of inflammatory mediators, leading to angioedema in some individuals. Patients who have developed acute angioedema to COX 1 and COX 2 inhibitors (ibuprofen, naproxen, aspirin, etc.) usually can be given a COX 2 inhibitor (celecoxib, etc.) without the patient developing angioedema.17 Other non-allergic causes of angioedema include angiotensin II receptor blockers that may cause angioedema by a non-bradykinin mechanism.18 Other drugs have been demonstrated to cause angioedema by a non-IgE mediated mechanism. Finally, there is hereditary and acquired angioedema, neither of which is IgE mediated. These non-allergic angioedemas are beyond the scope of this discussion and can be read about elsewhere.19, 20

Clinical Presentation Mast cell-mediated angioedema usually begins with ten minutes of exposure to the allergen and builds over a few hours, then resolves in 24-48 hours. The most commonly affected areas are the mucous membranes with swelling of the lips, tongue, and uvula. Angioedema of the larynx may develop rapidly (over minutes) or more slowly over several hours. The first symptom that patients experience is usually hoarseness followed by throat tightness and difficulty swallowing. The patient may present with severe stridor, which indicates marked swelling of the laryngeal tissues and warrants immediate therapy (see below). Angioedema may affect the wall of the GI tract and often presents as colicky abdominal pain, sometimes accompanied by nausea, vomiting, and/or diarrhea. Angioedema is usually a benign condition but may be life-threatening if it

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involves the larynx or other upper airway structures in which the patient presents with symptoms of upper airway obstruction or if it is associated with severe anaphylaxis (e.g., asthma and/or hypotension).

Differential Diagnosis Cutaneous or mucosal swelling that can mimic angioedema may result from contact dermatitis, cellulitis, autoimmune disease, superior vena cava syndrome, blepharochalasis (causing eyelid edema), some parasitic infections, hypothyroidism, idiopathic edema, and some rare disorders. Laryngeal edema may be mimicked by infectious laryngitis (usually viral), parapharyngeal abscess or neoplasm, severe tonsillitis, peritonsillar cellulitis or abscess, and pharyngeal/laryngeal foreign bodies. Angioedema of the gut may be mimicked by inflammatory bowel disease (especially Chrohn’s ileitis or colitis), acute bacterial ileitis (Campylobacter, Yersinia infections), mesenteric ischia or infarction, intramural hemorrhage, vasculitis inflammatory disorders near the bowel wall, and many others.21

Management Management of isolated, “benign” allergic angioedema affecting only the skin, lips, or minimal swelling of the tongue with or without acute urticaria is the same as management of acute urticaria, e.g., H1 and H2 antihistamines given orally or IV and glucocorticoids (see management of acute allergic urticaria above). Management of laryngeal edema or severe angioedema of the tongue, uvula, or soft palate requires immediate assessment for signs of airway obstruction. If the patient is only hoarse and there is only minimal swelling of the other upper airway structures, the patient should be placed in a critical care room with emergency airway management equipment immediately available. An IV should be started and the patient placed on high flow oxygen. As soon as possible, the patient should be given 0.3-0.5 mg of epinephrine intramuscularly (in children 0.01 mg/kg to a maximum of 0.5 mg). The patient should also be given diphenhydramine 25 mg IV, ranitidine 50mg IV, as well as intravenous corticosteroids, such as 125 mg of methylprednisolone (Solumedrol). However, none of these agents has been shown to reverse allergic angioedema-induced airway obstruction without the concomitant use of epinephrine.7 Usually the epinephrine will start working within 10-15 minutes, and the patient’s symptoms will improve. However, if there has been no improvement within 15 minutes, and the patient’s airway status has not deteriorated, another dose of 0.3-0.5 mg IM should be administered. This therapy is indicated even if the patient has a history of coronary artery disease. Most patients will respond to this therapy. Should the patient’s airway status deteriorate after epinephrine administration, particularly if the patient becomes stridorous, immediate rapid sequence orotracheal intubation (RSI) should be accomplished. The orotracheal intubation should be attempted by the most experienced operator available. In the rural setting, this may be the

nurse anesthetist or anesthesiologist, if available.6,7 If the tongue and posterior pharynx or the laryngeal structures themselves are markedly swollen, orotracheal intubation may be very difficult, and, if unsuccessful, a surgical airway (emergency cricothyroidotomy) should be attempted. An otolaryngologist or experienced general surgeon should be standing by (if available) to assist with the cricothyroidotomy or, if this is not successful in obtaining an airway, perform a tracheostomy. The surgical airway should be attempted by the most experienced operator available.6,7 If the patient is stridorous on presentation, the epinephrine and, time permitting, the other drugs noted above should be administered, but, unless there is immediate (less than 10-15 minutes) dramatic improvement in the stridor, emergent RSI should be performed with setup for an emergent surgical airway if needed.

Disposition and Follow-up The disposition and follow-up depends upon the severity and location of the angioedema and the response to therapy to epinephrine and antihistamines. If the angioedema does not involve the tongue, uvula, soft palate, larynx, or GI tract, the patient should be observed for a period of one to two hours after resolution of the angioedema. If the angioedema involves the above structures, and the edema has resolved with therapy, the patient should be watched for at least four hours, either in the ED, an observation unit, or in the hospital, admitted under “observational status.” It is unusual for the angioedema to recur following complete resolution of symptoms and signs, but it is possible – similar to patients with biphasic anaphylaxis (see below). If the angioedema involves these structures and has not resolved, it is probably advisable to admit the patient either as an inpatient or under observational status. When the patient is discharged, they should be given two epinephrine auto-injectors and given instructions on their use should the angioedema recur, as well as printed materials regarding angioedema and its treatment. They should have follow-up arranged with their primary care physician or an allergist.22

SEVERE ANAPHYLAXIS (Urticaria and/or Angioedema with Bronchospasm and/or Hypotension) Pathophysiology Severe anaphylaxis may be immunologic, e.g., IgE-mediated reactions or immune complex/ complement-mediated anaphylaxis, versus nonimmunologic anaphylaxis, e.g., caused by agents that cause sudden mast cell or basophil degranulation in the absence of immunoglobins. Mast cells and basophils release mediators such as histamine that act on the H1 and H2 receptors to cause a generalized capillary leak of plasma into the interstitial tissues as well as a reduction in venous tone, leading to hypovolemic shock. Stimulation of these receptors by


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histamine may also lead to depressed myocardial function and bronchospasm, contributing to the shock and resulting in respiratory failure.23 These more severe effects may be combined with urticaria and/or angioedema. Autopsy findings in fatal anaphylaxis may not reveal any specific pathology, since many of these patients die very rapidly (within one hour in 39 of 56 cases of fatal anaphylaxis in one study). Findings at autopsy include mucous plugging and hyperinflation of the lungs, laryngeal and/or pharyngeal edema, petechial hemorrhages in the bronchi and cerebral edema. The “empty ventricle syndrome” resulting from inadequate right ventricular filling may occur in patients who have significant hypotension and may lead to pulseless electrical activity (PEA). Therefore, the patient with severe anaphylaxis should always be placed in the supine position as soon as possible.24

Clinical Presentation Severe anaphylaxis has many clinical presentations, varying from mild-to-moderate asthma to rapid development of profound shock in less than 15 minutes after exposure to the allergen. The patient may exhibit urticaria and/or angioedema, although most cases of fatal anaphylaxis do not have these findings at autopsy. They often appear flushed, with generalized erythema. The patient usually presents with a combination of bronchospasm and hypotension, although they may have only one or the other. Patients with fatal anaphylaxis usually present with profound anaphylactic shock, with either PEA or occasionally bradycardia and/or very severe bronchospasm. Occasionally, the patient may initially have either urticaria or angioedema for one to three hours and then rapidly progress to severe asthma and/or severe hypotension. The most common causes of death are cardiovascular collapse or asphyxia. Ten to twenty percent of patients with severe anaphylaxis may develop biphasic anaphylaxis, a recurrence of symptoms following an asymptomatic period lasting from one to thirty hours. Although the recurrent episode is usually less severe than the initial, the second phase may be even more severe.16

Differential Diagnosis Conditions that may mimic severe anaphylaxis include acute asthma exacerbation, other causes of respiratory distress (choking from foreign body aspiration), vasodepressor (vasovagal) syncope, anxiety disorder, acute isolated severe urticaria, acute isolated angioedema, vocal cord dysfunction, administration of medication causing flushing (e.g., niacin, levodopa, vancomycin, etc.), other causes of shock (myocardial infarction with cardiogenic shock, septic shock, etc.), CVAs, carcinoid tumors, mastocytosis, capillary leak syndrome, scombroid poisoning, and others.26 Unfortunately, severe anaphylaxis does not always present with the classic findings of a combination of flushing or urticaria/ angioedema associated with bronchospasm and hypotension. Thus, treatment may be delayed in some patients, with occasionally fatal consequences.

Management Initial Assessment and Management As with any patient with a potentially fatal disease, patients with suspected severe anaphylaxis should immediately have an assessment of their airway, breathing, and circulation. Occasionally, patients may present with generalized erythema with no pulse and with apnea. These patients should be immediately orotracheally intubated and ventilated and have an ECG monitor and two large bore (16 gauge or larger) IVs started (if not already intubated and resuscitation begun by prehospital personnel). They should have initial CPR, and the ACLS protocols should be followed, depending upon their cardiac rhythm. These patients should all be given epinephrine 1 mg V. However, most patients with severe anaphylaxis are not in such extremis. They usually present with urticaria and/or angioedema with asthma and/or hypotension. These patients also require evaluation of their ABCs as well as an evaluation of their skin and mucous membranes, and a blood pressure should be obtained immediately. The patient should be placed in the supine position, given supplemental oxygen (6-8 liters via face mask) and two large bore IVs started (usually antecubital). The cardiac rhythm and pulse oximetery should be continuously monitored and their BP and respiratory rate monitored at least every five minutes. If these patients have severe asthma but have systolic blood pressures above 90 mmHg, they should be given 0.5 mg of 1:1,000 epinephrine IM into the mid-anterolateral thigh (adults) or .01 mg/kg IM to a maximum of 0.5 mg in infants and children.28 Epinephrine should always be given IM rather than subcutaneously because its absorption is more rapid and predictable.29 These patients should have NS running at 200-300 cc/hr, unless they have a history of congestive heart failure and/or renal failure, in which case the NS infusion rate should be no more than 150cc/hr. If the patient is hypotensive (<90 mmHg in adults), they should be given the NS wide open in both large bore IVs and, if their systolic BP is <70 mm/Hg, blood pressure cuffs should be placed around the NS bags and inflated to >250 mmHg to infuse the NS as rapidly as possible. These patients should receive the epinephrine IV rather than IM. Most authorities recommend giving the epinephrine by intravenous infusion.28 In adults, begin the IV infusion at 2 mcg/min, with titration upward if the patient continues to be hypotensive despite massive infusion of NS after 10-15 minutes. Children should receive 0.2 mcg/kg/min with titration upward if there is no response in blood pressure after 10-15 minutes. Adults should receive 1-2 liters of NS in the first 10-15 minutes, with continued high volume infusion if the blood pressure does not increase above 90 mmHg after the first two liters and continuation of the epinephrine infusion. Although the epinephrine may be given by IV boluses of 0.1 mg, errors in dosing (the patient receiving 1 mg, rather than 0.1 mg IV) have resulted in death. If given in IV boluses, epinephrine should always be given utilizing the 10 cc 1:10,000 syringes, giving no more than 1cc boluses.30

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Some patients may require up to 10 liters of NS on the first one to three hours because of the massive fluid shifts from the intravascular to the extravascular spaces in severe anaphylactic shock. Children should be given NS boluses of 20 cc/kg until they are no longer hypotensive. Again, patients with CHF and/or renal failure should be closely monitored for fluid overload. Although there may be a reluctance to use epinephrine in patients with known CAD, those receiving monoamine oxidase inhibitors, those receiving stimulant medications (cocaine, methamphetamines, etc.), and those with known aortic aneurysms or recent intracranial surgery, the benefits of epinephrine outweigh the risks in severe anaphylaxis, even in these high risk patients.28 If the patient is on beta blocker medications, they may be refractory to epinephrine in severe anaphylaxis. If so, glucagon should be administered in doses of 2-4 mg IV in adults and 20-30 mcg/kg to a maximum of 1 mg in children. The drug should be given by slow IV infusion (over five minutes) because rapid infusion may cause vomiting.31 If the patient is refractory to intravenous epinephrine, vasopressin (100 units in 500 cc D5W, with a dose of 0.04 units per minute or administration of a slow bolus of two units over five minutes, titrating upward to eight units) has been shown to benefit some patients with severe anaphylaxis that is refractory to epinephrine.32 Adjunctive therapy in severe anaphylaxis includes H1 antihistamines, H2 antihistamines, inhaled beta2 agonist bronchodilators, such as albuterol (if the bronchospasm is not relieved with epinephrine), and glucocorticoids. However, there is no good evidence that these adjunctive medications provide any additional benefit to the use of epinephrine in patients with severe anaphylaxis.33, 34, 35 Most patients with severe anaphylaxis will respond relatively quickly (within 15-30 minutes) to aggressive therapy with IM epinephrine in those without hypotension and to IV epinephrine and large quantities of IV NS in those with even severe hypotension.64 However, if the patient persists with hypotension and/or severe bronchospasm, they should be admitted to an ICU, either at the treating hospital or transferred to an ICU at the closest facility via paramedic and/or nurse transport, in order to provide airway support and if continued IV epinephrine is required. Often, they will require aeromedical transport if the treating hospital is in a rural area with few resources. Some authorities recommend transferring the patient from a small hospital with few resources to either an ICU or monitored bed at a major hospital, even if the patient is normotensive and has little bronchospasm because of the concern of the patient developing biphasic anaphylaxis (see below).

Disposition and Follow-up Currently, there is no consensus regarding the optimum observation period following successful treatment for severe anaphylaxis. Because of the possibility of biphasic anaphylaxis, it is probably prudent to admit the patient to the hospital with close

monitoring for at least 24 hours. However, the optimal observation time is unclear. If the patient’s symptoms have resolved promptly and completely, some guidelines recommend observing the patient for at least six hours. At the time of discharge, the patient should receive two epinephrine auto-injectors and be instructed in their use. Early epinephrine use is associated with improved outcomes in severe anaphylaxis.28 Patients should be given information regarding severe anaphylaxis. In addition, they should be told to return immediately if symptoms recur. They should follow up with their primary care physician or an allergist within a few days. The mnemonic “SAFE” has been developed by the American College of Emergency Physicians and the American College of Allergy, Asthma, and Immunology to guide physicians in instructing patients with severe anaphylaxis prior to discharge from the ED or hospital.36 The S.A.F.E. instructions can be incorporated into the patient discharge instructions for severe angioedema and/or anaphylaxis. S – Seek support – the patient should be advised that: 1 - They have experienced a very severe allergic reaction. 2 - Symptoms similar to the acute episode may recur up to 72 hours (biphasic anaphylaxis). 3 - If such symptoms recur, they should immediately selfinject epinephrine with the auto-injector and call 911 to transport them to the nearest ED. 4 - They are at higher risk for repeat episodes of severe anaphylaxis in the future. 5 - They should read about anaphylaxis (give the patient resources on anaphylaxis, especially the recognition of its symptoms by the patient). A – Allergen identification and avoidance – the physician should: 1 - Attempt to identify the patient’s anaphylaxis trigger via a thorough history and in vitro testing (measure specific IgE to the allergen in the serum) prior to discharge. 2 - Recommend allergy testing by an allergist to confirm the trigger, so that the patient can avoid it in the future. F – Follow up with an allergist or a physician well versed in the diagnosis and management of severe anaphylaxis. E – Epinephrine (self-injected) to be used at the first sign of allergic symptoms: 1 - Instruct the patient to carry the epinephrine auto-injector with them at all times 2 - Instruct them on how to use the auto-injector as well as encourage them to instruct their family and close friends on its use (in case the patient is unable to give the injection).


American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

Summary The physician or mid-level practitioner in a rural emergency department should be able to recognize and manage patients with anaphylaxis, whether it is isolated acute urticaria and/or angioedema or severe anaphylaxis with associated bronchospasm and/or hypotension. In the case of severe angioedema or anaphylaxis, epinephrine should be administered as soon as possible. If the patient does not respond to therapy relatively rapidly, they should consider transfer to a facility that has the resources to deliver a higher level of care, especially if the patient has severe anaphylaxis. Patients with angioedema or severe anaphylaxis should always be discharged with two epinephrine auto-injectors and be instructed in their use. They should be told to return if their symptoms recur and to follow up with a medical provider who specializes in the diagnosis and management of anaphylaxis. M. Scott Linscott, MD, is Adjunct Professor of Surgery (Emergency Medicine), University of Utah School of Medicine, Salt Lake City. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

References 1.

2.

12. Dhanya NB, Rai R, Srinivas CR. Histamine H2 blocker potentiates the effects of histamine 1 blocker in suppressing histamine-induced wheal. Indian J Dermatol Venereal Leprol. 2008;74:475-9. 13. Pollack CV Jr, Romano TJ. Outpatient management of acute urticaria: the role of prednisone. Ann Emerg Med. 1995;26:547-51. 14. Kaplan AP, Graves MW. Angioedema. J Am Acad Dermatol. 2005;53:373-88. 15. Romano A, Viola M, Gudeant-Rodriguez RM, et al. Imipenem in patients with immediate hypersensitivity to penicillins. N Engl J Med. 2006;354:2835-7. 16. Bluestein HM, Hoover TA, Banerji AS, et al. Angiotensin-converting enzyme inhibitor-induced angioedema in a community hospital emergency department. Ann Allergy Asthma Immunol. 2009;103:502-7. 17. Zembowicz A, Mastalerz L, Setkowitz M, et al. Safety of cyclooxygenase 2 inhibitors and increased leukotriene synthesis in chronic idiopathic urticaria with sensitivity to nonsteroidal anti-inflammatory drugs. Arch Dermatol. 2003;139:1577-82. 18. ONTARGET investigators, Yusuf S, Teo KK, et al. Telmisartin, ramapril, or both in patients at high risk for vascular events. N Engl J Med. 2008;358:1547-59. 19. Zuraw BL. Clinical practice. Hereditary angioedema. N Engl J Med. 2008;359:1027-36. 20. Zingale LC, Castell R, Zanichelli A, et al. Acquired deficiency of the first complement component: presentation, diagnosis, course, and conventional management. Immune Allergy Clin North AM. 2006;26:669-90. 21. Charlesworth EN. Differential diagnosis of angioedema. Allergy Asthma Proc. 2002;23:337-9. 22. Simons FER. Anaphylaxis, killer aller: long term management in the community. Allergy Clin Immunol. 2006;117:367-77. 23. Kemp SF, Lockey RF. Anaphylaxis: a review of causes and mechanisms. J Allergy Clin Immunol. 2002;110:341-8.

Sampson HA, Munoz-Furlong A, Campbell RL, et al. Second symposium on the definition and management of anaphylaxis: summary reportSecond National Institute of Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network symposium. J Allergy Clin Immunol. 2006;117:391-7.

24. Brown SG. Cardiovascular aspects of anaphylaxis: implications for treatment and diagnosis. Curr Opin Allergy Clin Immunol. 2005;5:359-64.

Ying S, Kikuchi Y, Meng Q, et al. TH1/TH2 cytokines and inflammatory cells in skin biopsy specimens from patients with chronic idiopathic urticaria. Comparison with the allergen-induced late-phase cutaneous reaction. J Allergy Clin Immunol. 2002;109:694-700.

26. Simons F, Estelle R. Differential diagnosis of anaphylaxis in children and adults. UpToDate. December 7, 2010.

3.

Zuberbier T, Bindslev-Jensen C, Canonica W, et al. EAACI/GAZLEN/ EDF guideline: definition, classification and diagnosis of urticaria. Allerg. 2006;61:316-20.

4.

Beltrani VS. Urticaria:reassessed. Allergy Asthma Proc. 2004;25:143-49.

5.

Charlesworth EN. Urticaria and angioedema: a clinical spectrum. Ann Allergy Asthma Immunol. 1996;76:484-95.

6.

Lieberman P, Nicklas RA, Oppenheimer J, et al. The diagnosis and management of anaphylaxis practice parameter: 2010 update. J Allergy and Clin Immunol. 2010;126:477-80.

7.

Simons FER, Ardusso LR, Bilo MB, et al. World Allergy Association anaphylaxis guidelines: summary. J Allergy Clin Immunol. 2011;127:587-93.

8.

Weldo D. When your patients are itching to see you: not all hives are urticaria. Allergy Asthma Proc. 2005;26:1-7.

9.

Van Dellen RG, Maddox DE, Dutta EJ. Masqueraders of angioedema and urticaria. Ann Allergy Asthma Immunol. 2002;88:10-14.

10. Beno SM, Nadel FM, Alessandrini EA. A survey of emergency department management of acute urticaria in children. Pediatr Emerg Care. 2007;23:862-8. 11. Lin RY, Curry A, Pesola GR, et al. Improved outcomes in patients with acute allergic syndromes who are treated with combined H1 and H2 antagonists. Ann Emerg Med. 2000;36:462-8.

25. Ellis AK, Gay JH. Incidence and characteristics of biphasic anaphylaxis: a prospective evaluation of 103 patients. Ann Allergy Asthma Immunol. 2007;98:64-9.

27. Soar J, Pumphrey R, Cant A, et al. Emergency treatment of anaphylactic reactions-guidelines for healthcare providers. Resuscitation. 2008;77:157-69. 28. Simons KJ, Simons FER. Epinephrine and its use in anaphylaxis: current issues. Curr Opin Allery Clin Immunol. 2010;10:354-61. 29. Sheikh A, Shehata, YA, Brown SG et al. Adrenalin for the treatment of anaphylaxis: Cochrane systematic review. Allergy. 2009;64:204-12. 30. Kanwar M, Irvin CB, Frank JJ, et al. Confusion about epinephrine dose leading to iatrogenic overdose: a life-threatening problem with a potential solution. Ann Emerg Med. 2010;55:341-4. 31. Thomas M, Crawford I. Best evidence topic report. Glucagon infusion in refractory anaphylactic shock in patients on beta blockers. Emerg Med J. 2005;22:272-3. 32. Schummer C, Wirsing M, Schummer W. The pivotal role of vasopressin in refractory anaphylactic shock. Aneth Analg. 2008;107:620-4. 33. Sheikh A, Ten Broek V, Brown SG. H1-antihistamines for the treatment of anaphylactic shock. Cochrane systematic review. Allergy. 2007;62:830-7. 34. Simons FER. Pharmacologic treatment of anaphylaxis: can the evidence base be strengthened? Curr Opin Allergy Clin Immunol. 2010;10:384-93. 35. Choo KJ, Simons E, Sheikh A. Glucocorticoids for the treatment of anaphylaxis: Cochrane systematic review. Allergy. 2010;65:1205-11. 36. http://www.acaas.org/allergist/allergies/anaphylaxis/pages/action-guideanaphylaxis.aspx.

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Investigating a Rural Rotation in the Mississippi Delta: A Qualitative Study in Medical Education C. Cory Smith, RN, PhD(c)

Abstract With access to health care coupled with a severe shortage of physicians being on the forefront of much debate nationally and internationally, rural areas often suffer the worst. Innovations in medical education, such as the creation of rural immersion rotations, are attempting to solve this health crisis. The purpose of this study was to investigate and analyze the narrative writings of 36 fourth-year medical students and primary care residents that participated in a rural immersion rotation in the Mississippi Delta. A grounded theory data analysis led to the emergence of three central themes: professional development, contrast from academic medicine, and alteration of prior perceptions/misconceptions. The findings of this research provided rich data from the perspective of the medical student and resident.

Key Words Medical Education, Mississippi Delta, Qualitative Research, Rural Health

Introduction As of August 2011, approximately 60% of Mississippi’s total population lives in Primary Care Health Professional Shortage Area or HPSA’s.1, 2 HPSAs are designated by HRSA “as having shortages of primary medical care, dental or mental health providers and may be geographic, demographic, or institutional (comprehensive health center, federally qualified health center or other public facility).”1 Jeralynn Cossman, a professor from Mississippi State University specializing in medical sociology and health services issues, argues that certain regions of Mis-

sissippi, such as rural areas, are affected more so than others.3 Demographically, more than half (56%) of all Mississippi physicians are located in four urban areas. According to Cossman, physicians in the Mississippi Delta are slightly more likely to be older and nearing retirement.3 Cossman suggests this would indicate that future shortages could be hardest felt in the Mississippi Delta, assuming many doctors are approaching retirement and few doctors are in a position to replace them.3 With educators at the University of Mississippi recognizing the need for programs to address the shortage of future rural physicians in the Mississippi Delta, educators created a rural rotation specifically focusing in the Mississippi Delta region in Mississippi. The program was initially designed for fourth-year medical students but later became eligible to medical residents. This rural immersion program established ten training sites in seven of the 18 Mississippi Delta counties. Initially, preceptors were board-certified internal medicine physicians but later included family medicine physicians. In the inaugural year of the program, a total of nine fourth-year medical students were recruited.4 After the expansion of the program to residents in 2010, a total of 17 medical students and residents participated.4 With more campus-wide awareness growing, the third year of the program recruited a total of 24 medical trainees to participate in this rural immersion program.4

Review of the Literature The Accreditation Council on Graduate Medical Education (ACGME) reported that “one of the greatest barriers to better focused health workforce policy has been a lack of research into the causes and consequences of shortages.”5 With little


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crucial research available, theories about the nature of shortages have been loosely conceived and often not confirmed.6 Therefore, training for rural practice has become critically important in the context of continuing serious shortages of doctors and other health workers in rural areas. Rosenman and Batman recognized that “the overflow effect,” which is producing more health-care providers and expecting the excess to spill over from the cities into the rural areas, is not effective in solving the rural medical workforce shortage.7 Barer and Stoddart maintain that no single strategy is likely to be successful, but multiple strategies are required at different levels of education and training, each of which will have a small effect and contribute to an overall substantial change.8 However, one key series of initiatives that has been shown to be effective involves ruralbased medical education.9 Strasser and Neusy found that to appreciate the requirements of rural training, it is necessary to understand rural communities, their health service needs, and the nature of rural practice.10 The earlier work of Strasser discovered there are a series of key parameters that provide the framework, which determines the structure and function of rural health services and how rural practitioners work and the nature of rural practice.11 Strasser suggested that these parameters or “rural realities” are: geography and demography; the rural culture; rural morbidity and mortality patterns; resource limitations; and workforce shortages.11 While research of rural training programs has been investigated nationally to a limited extent, there has not been any documented investigation of rural training programs within the Mississippi Delta. In addition, there is needed research in some of the newer modes of medical education theory, such as narrative writing, being incorporated to enhance reflective learning practice and rural training programs utilizing immersion learning principles. Strasser and Neusy maintain that, since the mid-1980s, research evidence has been accumulating regarding the specific range of knowledge and skills required by rural physicians.13 Hayes and Gupta suggested that such evidence has led to the inclusion of specific curriculum content on rural health and rural practice in undergraduate medical programs and in rural-based family medicine residency programs.12 These advances in medical school curricula content were brought about when researchers compared rural physicians to their metropolitan counterparts and found that rural physicians carry a higher workload, provide a wider range of services, and carry a higher level of clinical responsibility in relative professional isolation.13 Also, Hogenbirk et al. discovered that these characteristics hold true for all rural health care professionals, such as nurses and pharmacists.13 Medical educators have responded with curricular reform that includes the use of learning communities and community-based education that is responsive to local needs.14 Strasser and Neusy argue that immersion learning has emerged as a strategy that addresses both educational and societal needs.10 Also, rural practitioners often become integral members of the community that they serve; they have a significant public health role that may

range from issues such as clean water and sanitation to community education.10 On the other hand, the community serves an integral role to the learner because the local communities and health care workers become medical educators.15 Furthermore, Crampton et al. suggested that the student is not just the receptacle of experience and teaching, but an active and necessary member of the health care team. The end result is that all team members become teachers and relationships start to develop.15 Irby and Wilkerson discovered that some US medical schools have instituted rural clinical education programs that included an immersion component in rural communities.14 These dramatic trends in health care coupled with new theories of learning have prompted significant changes in medical education.14

Methods The choice of a qualitative research design was determined given benefits related to the realm in medical education. Chretien, Ginsburg, and Hauer suggest that the fit between medical education and qualitative research is a natural one.16 “Qualitative research is particularly well-suited to providing data on needs assessments, program development, and curriculum evaluation, and helps us understand not just what outcomes were achieved, but also the process, impact, and unintended effects.”16 The inclusion criteria for the research population was limited to fourth-year medical students and primary care residents of the University of Mississippi Medical Center, who self-selected to participate in this particular rural immersion program and provide a journal upon completion of their rotation. A total of 36 journals were collected prior to analysis of the data. The type of data collected and analyzed is classified as narrative inquiry. A grounded theory approach was utilized for this particular study. Grounded theory exists at the most abstract level of various forms or pure empirical description, such as found in life histories, simple data presentations, and other phenomenological approaches to exact reports of what is happening.17 Furthermore, the purpose of grounded theory method is to build theory that is faithful to and illuminates the area under study.18 From the data collected, a series of coding was used for data analysis, which consisted of open coding, axial coding (or theoretical coding), and selective coding (or constant comparative coding). Trustworthiness was ensured by the researcher utilizing a research partner. The researcher served as a detached peer and outside coder.17 In order to protect the rights of human participants involved within the research, proper steps were taken to obtain permission from the Institutional Review Board (IRB).

Results Three themes emerged from the data analysis process: professional development, contrast from academic medicine, and alteration of prior perceptions/misconceptions. These three themes are grounded in the data analyzed. Furthermore, the use of direct quotations was largely used to support the findings of this particular research. These quotations provided insight into

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the participant’s live experiences during their rural rotation in the Mississippi Delta. A tabulated overview of the participant demographics was created, which consist of their participation year, educational classification, and corresponding clinical site location (see Table 1).

Professional Development The first theme that emerged was the concept of participants reflecting on their professional development as a result of this particular clinical rotation. Participants often acknowledged becoming more autonomous. One medical student wrote: “I find that this rotation has given me plenty of autonomy. I am free to order whatever lab test I see fit and with each patient I present my treatment plan…” (Participant 1). Participants were also able to reflect on their professional development in regards to gaining confidence in their clinical skills. One resident commented: “Fairly busy morning with high volume of DM [diabetes mellitus] management. I am becoming more comfortable adjusting insulin dosing” (Participant 8). There were also instances when autonomy and gaining confidence merged. For instance, one resident stated: “I made rounds at __________ Hospital and now feel comfortable seeing patients on my own” (Participant 14). Participants also acknowledged areas of emotional growth within this theme of professional development. One medical student reflected:

“It’s so much different when doing a procedure than merely watching. As students we normally watch and rarely get to do!” (Participant 24). One medical student remarked how the academic setting does not prepare future physicians on the business management portion of practicing medicine. “Nowhere else in medical school training we get to work in a private physician’s clinic and learn the ins and outs of running a successful practice” (Participant 32). Another medical student suggested their idea for improving the relationship between training in the academic setting versus real-world medicine: “The way rotations are set up for junior and senior medical students is NOT a reflection of real-life medicine. I also think every student should spend at least one month in a community setting in the specialty they wish to pursue. This is the only way to improve the real-world understanding of what practice will be like if we chose to practice in a non-metropolitan area” (Participant 34). Participants also contrasted how clinical decision-making can be different. One resident noted: “How ironic that in training we are told to limit antibiotics in clinic to prevent resistance and then in the private sector they are so widely distributed to appease patients” (Participant 29).

Alteration of Prior Perceptions/ Misconceptions

“I don’t feel bad anymore about not being able to examine her [patient]. I initially viewed it as a defeat, but I am learning to not take things so personally” (Participant 12).

The final theme that emerged from the data analysis was the concept of participants changing prior perceptions and sometimes misconceptions as a result from this experience.

One medical student noted an area of needed growth in their professional development by the following statement:

An example of a participant changing their perception of practicing in a rural setting is noted when a medical student stated:

“I realized I am not as efficient when it comes to noticing when a disease process is presenting. The signs can be subtle” (Participant 27).

“When I started this rotation, I was not really at all considering doing general practice and didn’t expect that to change. However, I am considering it much more seriously after this rotation. I kind of like the idea of being a small-town doctor. I would like to be able to fill that role” (Participant 5).

Contrast from Academic Medicine The second theme that emerged was noted when participants stated how this rotation was a divergence from their clinical experiences in academic medicine. Participants often felt a sense of belonging. One medical student wrote: “I didn’t feel like just another medical student; I felt I was wanted and needed” (Participant 19). Another medical student commented on how in the academic setting their experiences were more observing than participating:

One resident reflected on how their experience not only altered their view of practicing medicine in an underserved area but could have impacted their previous clinical experiences: “The benefits of working here far outweigh the negatives . . . I wish I had it [rotation] earlier in my first year. The things I’ve learned here would have helped me in a number of my previous rotations . . . It has definitely made me consider working in an underserved community after graduation.


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Table 1: Participant Demographics PARTICIPANT NUMBER

PARTICIPATION YEAR

EDUCATIONAL CLASSIFICATION

SITE LOCATION City – County

1

2009

Medical Student

Greenwood – Leflore Co.

2

2009

Medical Student

Cleveland – Bolivar Co.

3

2009

Medical Student

Belzoni – Humphreys Co.

4

2009

Medical Student

Belzoni – Humphreys Co.

5

2009

Medical Student

Vicksburg – Warren Co.

6

2010

Medical Student

Belzoni – Humphreys Co.

7

2010

Medical Student

Lexington Holmes Co.

8

2010

Resident

Belzoni – Humphreys Co.

9

2010

Medical Student

Cleveland – Bolivar Co.

10

2010

Medical Student

Belzoni – Humphreys Co.

11

2010

Medical Student

Belzoni – Humphreys Co.

12

2010

Medical Student

Lexington –Holmes Co.

13

2010

Resident

Vicksburg – Warren Co.

14

2010

Resident

Vicksburg – Warren Co.

15

2010

Medical Student

Cleveland – Bolivar Co.

16

2010

Resident

Cleveland – Bolivar Co.

17

2010

Medical Student

Greenville – Washington Co.

18

2011

Resident

Greenwood – Leflore Co.

19

2011

Medical Student

Lexington – Holmes Co.

20

2011

Medical Student

Vicksburg – Warren Co.

21

2011

Medical Student

Lexington – Holmes Co.

22

2011

Resident

Mound Bayou – Bolivar Co.

23

2011

Resident

Mound Bayou – Bolivar Co.

24

2011

Medical Student

Belzoni – Humphreys Co.

25

2011

Medical Student

Belzoni – Humphreys Co.

26

2011

Medical Student

Belzoni – Humphreys Co.

27

2011

Medical Student

Cleveland – Bolivar Co.

28

2011

Medical Student

Belzoni – Humphreys Co.

29

2011

Resident

Cleveland – Bolivar Co.

30

2011

Resident

Mound Bayou – Bolivar Co.

31

2011

Resident

Mound Bayou – Bolivar Co.

32

2011

Medical Student

Yazoo – Yazoo Co.

33

2011

Resident

Mound Bayou – Bolivar Co.

34

2011

Medical Student

Vicksburg – Warren Co.

35

2011

Resident

Mound Bayou – Bolivar Co.

36

2011

Resident

Mound Bayou – Bolivar Co.

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Before this rotation I wouldn’t have ever given it a second thought” (Participant 30). Participants also re-evaluated prior misconceptions. For instance, one medical student stated: “Most people think that general internists make less than 200K per year, but the physicians in this office are making 2-3x this amount . . . I have always thought that internists were underpaid . . . This is a huge misconception” (Participant 34). Another medical student commented: “I had been to the Delta before but had never been in the area of medicine. I actually expected the place to be rundown and in a poor condition based on what I heard about the Delta . . . Before this experience I only knew what I heard about the Delta healthcare. I see the Delta in a brighter light. When I finish my training in family medicine, I will definitely consider practicing in the Delta” (Participant 19). An example of a resident expressing this concept was found in the following statement: “I’m actually starting to like doing clinic work, something I’ve disliked in residency and grown to hate . . .” (Participant 29).

Conclusion As referenced in the review of literature there is a lack of research and knowledge of rural training programs as a whole, and this gap in research has been linked to be a fundamental cause of the physician shortage. Therefore, the findings from this qualitative study suggest that the use of narrative inquiry can be an asset in the realm of medical education. For instance, when the Mississippi Delta is often cited in health publications, there are generally negative connotations implicated regarding its poor health outcomes. Because of these connotations, it would be reasonable to assume future physicians form perceptions or biases regarding the health and health care in Mississippi Delta. Pathman, Konrad, Dann, and Koch argue it is primarily perceptions that link the multitude of factors to retention in underserved areas, and perceptions can be inaccurate.19 As evidence of the third theme, participant attitudes, insights, and future career decision-making featured in the journals could dispel some of these incorrect perceptions toward practicing medicine in the rural Mississippi Delta. Additional knowledge of rural training programs better informs medical educators on how to make decisions concerning facilities for practices and rural clinic and hospital partnerships to better accommodate the teaching and training of medical students and residents. Furthermore, promoting educational collaboration between rural physicians of the Mississippi Delta and the University of Mississippi Medical Center could lead to training

consortiums to integrate clinical experiences and teaching commitments. The importance of this promotion could alleviate the load of overburdened staff and resources while improving the health of many rural Mississippi Deltans. Future research should continue to invest in and investigate rural immersion programs, such as this one. While studying quantitative research in the rural physician shortage is useful, perhaps future research can investigate this issue using mixed methodology. Qualitative results, such as the ones focused on in this research article, can be a great source in providing richer data on the social issues behind this phenomenon.

Limitations One limitation of the study is noted geographically. Although the research setting included multiple site locations, which are all considered rural counties by US Census Bureau data, the research only utilized seven of the 18 Mississippi Delta areas. Therefore, the findings of this research may not be applied in broader contextual terms representing all areas of the Mississippi Delta. Since qualitative methodology was utilized, which centered on the lived experiences of the participants, the findings of this research cannot be generalized to the larger population of medical students and residents. C. Cory Smith, RN, PhD(c), is the Director of Performance Improvement at the Mississippi State Department of Health and a doctoral candidate in the School of Graduate Studies in Health Sciences at the University of Mississippi Medical Center in Jackson, Mississippi. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

References 1.

Health Resources and Services Administration. One Department Serving Rural America: HHS Rural Task Force Report to the Secretary, 2007. Available at: http://ruralhealth.hrsa.gov/PublicReport.htm. Accessed November 15, 2011.

2.

US Census Data. State and county quick facts, 2010. Available at: http:// quickfacts.census.gov/qfd/states/28/28163.html.

3.

Cossman JS. Mississippi’s physician labor force: current status and future challenges. Journal of Mississippi State Medical Association. 2004;84:8-31.

4.

Mazurak J. Delta views: health scholars’ insights shed light on rural primary care. Centerview. 2011;4-5.

5.

Accreditation Council on Graduate Medical Education. Tenth report: physician distribution and health care challenges in rural and inner city area, 1998. Washington, DC; Department of Health and Human Services.

6.

Pathman DE, Konrad TR, Dann R,Koch G. Retention of primary care physicians in rural health professional shortage areas. American Journal of Public Health. 2004;94:1723-1729.

7.

Rosenman SJ, Batman GJ. Trends in general practitioner distribution from 1984 to 1989. Australian Journal of Public Health. 1992;16:84-88.


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8.

9.

Barer ML, Stoddart GL. Toward integrated medical resources policies for Canada: Geographic distribution of physicians. Canadian Medical Association Journal. 1992;147:617-623. Rosenblatt RA, Whitcomb ME, Lishner DM, Hart LG. Which medical schools produce rural physicians? JAMA. 1992;268:1559-1565.

10. Strasser R, Neusy AJ. Context counts: training health workers in and for rural and remote areas. Bulletin of World Health Organization. 2010;88:777-782. 11. Strasser R. Training for rural practice: lessons from Australia, 2001. Retrieved from http://fammedmcmaster.ca/faculty/development/carl_ moore_lecturship/lecture_2001.pdf. 12. Hayes R, Gupta T. Ruralising medical curricula: the importance of context in problem design. Australian Journal of Rural Health. 2003;11:15-17. 13. Hogenbirk JC, Wang F, Pong RW, Tesson G, Strasser RP. Nature of rural medical practice in Canada: an analysis of the 2001 national family

physician survey. Laurentian University, Sudbury, Ontario. 14. Irby DM, Wilkerson L. Educational innovations in academic medicine and environmental trends. Journal of General Internal Medicine. 2003;18:370-376. 15. Crampton P, Dowell A, Parkin C, Thompson C. Combating effects of racism through a cultural immersion medical education program. Academic Medicine. 2003;78: 595-598. 16. Chretien KC, Ginsburg S, Hauer K. Qualitative analysis for medical educators. Academic Internal Medicine. 2010;8(2):14-15. 17. Glaser BG. Basics of Grounded Theory Analysis. Mill Valley, California: Sociology Press;1992. 18. Mertens D. Research methods in education and psychology: Integrating diversity with quantitative and qualitative approaches. Thousand Oaks, CA:Sage;1998.

Do you know a qualified physician who is in need of board certification or a Diplomate needing to recertify? The American Board of Physician Specialties (ABPS), the official certifying body of the American Association of Physician Specialists, Inc. (AAPS), provides medical specialty certification and recertification in the following specialties: • • • • • • • • •

Anesthesiology Dermatology Diagnostic Radiology Disaster Medicine Emergency Medicine Family Medicine Obstetrics Family Practice Geriatric Medicine Hospital Medicine

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Internal Medicine Obstetrics and Gynecology Ophthalmology Orthopedic Surgery Psychiatry Radiation Oncology Surgery Urgent Care

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 Eligibility requirements include advanced training, significant experience, good moral character, and successful completion of a specialty written and/or oral examination.  In order to maintain certification, every physician is required to complete re-certification every eight years.  ABPS also provides recertification for eligible Diplomates from Member Boards of ABMS and AOABOS. See Complete Requirements at http://www.abpsus.org/certification/index.html. For additional information, contact the ABPS Certification Department at 813.433.2277

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sounding board

“Doctor, my legs are swelling” Thomas G. Pelz, DO

One of the most frequent reasons a physician is consulted by a patient is for leg swelling. The expected response from the physician is a prescription for a diuretic, such as hydrochlorothiazide or furosemide. Unfortunately, our society expects that a pill will cure their problem. All too often, the desired solution has untoward outcomes. I find it interesting that the person seeking the pill solution to this problem will ignore all of the published side effects of the diuretic but will usually demand to be informed what the complications are for a medication that they need for another problem. My practice is primarily hospital-based; that is, the majority of my patients are inpatients. All too often I find that I am trying to diagnose and then correct the medical complications of patients’ bad habits. Often complications of diuretics are a major source of the patient’s reason for admission. Problems include but are not limited to hyponatremia, hypercalcemia, hypokalemia, hyperlipedemia, muscle cramps, headaches, vertigo, and hyperglycemia. The problems created by diuretics are not minor; otherwise, the patient would not be admitted. During the past year, I have managed a serum calcium of 14, which was almost entirely secondary to a patient’s use of a diuretic to manage her leg edema. Another admission involved a patient with a potassium of 2.1. Not surprisingly, this patient also had numerous arrhythmias. The overlooked aspect of this situation is that determining the etiology of these problems requires time and considerable expense. There is a large and varied list of sources regarding the evaluation of the etiology and the therapy of swollen legs. Google Scholar listed more than 15,000 citations published since 2010, when “swollen legs” is searched. When I searched “swollen legs

and diuretics” since 2010, I found more than 500 citations. The citations deal with swollen legs that occurred secondary to complications of numerous serious co-morbid medical conditions. The patients I am discussing now had no such co-morbidities. They only wanted an easy fix for the swelling that was occurring in their legs. They did not want to change their lifestyle or bad habits. They wanted a quick and easy solution to their problem. My suggested solution requires the patient to change many of his/her current habits. I have found that my suggested changes are greeted with great reluctance on the part of the patient. The first step I suggest is that they wear support hose. Sources for these include the Jobst stockings (jobst-usa.com) and Thera wear (therawear.com). I usually recommend that the patient use the “moderate” compression (20 - 30 mmHg) hose. This is greeted with, “They are too hard to put on.” Or “They make my legs look horrible.” My second recommendation is the reduction in the amount of salt used by the patient. This is likewise greeted with derision. The third recommendation involves limiting the patient’s fluid intake to less than sixty ounces per day. This is the life style change that most patients are reluctant to alter. Most patients tell me that they have been consuming the recommended eight glasses of water a day in addition to their coffee, soda pop, and alcoholic beverages, which they have been using for years. When I discuss my recommendations with the patient, I have found that it is best if I sit down with the patient and another person in the room. I leave my sixteen ounce cup of coffee at the nurse’s station. I then discuss the complications of the diuretics they were using which have resulted in their hospitalization.

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sounding board Unfortunately, in the practice of medicine, it is often easier to acquiesce to the patient’s wishes. It takes considerable time to explain why such an easy solution, such as a simple water pill, is the worst choice. In my experience it is even more difficult, and usually impossible, for the physician to convince the patient to change his/her life style or bad habits. The only positive thought in this whole process is that there is a billing code which permits the physician to bill for the length of time spent with a patient. When billing for this situation, it is important that the physician include in his/her dictation the amount of time spent with the patient and to include the phrase “. . . more than 50% of the time with the patient was spent counseling and coordinating the patient’s health care problems.” It also helps to list the topics covered during the office visit, so that you can review them with the patient when you see them next. How successful is this therapy for swollen legs? I would like to think that my success rate is more than 70%. However, since most of the people I manage in the hospital return to their primary care physician, it is difficult to determine if the patient is continuing to be compliant with the discharge instructions.

Thomas G. Pelz, DO, practices hospital-based medicine at Boscobel Hospital, Boscobel, Wisconsin. He holds board certifications in both Internal Medicine and Hospital Medicine. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

References The following references are but a few of the articles that discuss the complications of diuretics use. Google Scholar (scholar.google.com) was the search engine. Entering complications of diuretics use resulted in more than 60,000 possible references. Messerli FH, Bangalore S, Julius S. Risk Benefit Assessment of ?-Blockers and Diuretics Precludes Their Use for First-Line Therapy in Hypertension. Circulation. 2008;117:2706-2715. Kaplan NM. Problems with the use of diuretics in the treatment of hypertension. Am J Nephrol. 1986;6(1):1-5. Greenberg A. Diuretic Complications. American Journal of the Medical Sciences. January 2000;319 (1):10.

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“Doctor, my legs are swelling”

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American Journal of Clinical MedicineŽ • Winter 2012 • Volume Nine Number One

Preconception Counseling to Prevent the Complications of Obesity during Pregnancy Merri M. Paden, MD Daniel M. Avery Jr., MD

Abstract Obesity is epidemic in proportions in the United States, and obesity rates continue to rise each year. The group with the fastest rising obesity rate is young women in the reproductive years between the ages of 19 and 28. Obstetricians and gynecologists see many women who fall into the overweight and obese categories. Without intervention these numbers will continue to rise, putting patients and offspring at risk for numerous complications during pregnancy. Obstetricians and gynecologists often function as primary care providers because many women see only their OB/GYN for health care needs. Obstetricians and gynecologists have an opportunity to incorporate nutrition and exercise education into the primary care regimen of the women they see, because pregnancy and the postpartum period present a unique opportunity to impact the health of women for the rest of their lives. Lifestyle, nutrition, and weight loss counseling are not traditional components of OB/GYN care but can complement services of other primary care specialties such as internal medicine and family medicine. Pre-pregnancy body mass index, weight gain during pregnancy, and weight loss in the first year postpartum are all independent predictors of body mass index 15 years after pregnancy.5

Introduction Obesity is epidemic in proportion in the United States, and obesity rates continue to rise each year. According to the CDC 34% of adult Americans were obese in 2008, and another 34% were overweight.1 Perhaps even more alarming, 18% of adolescents ages 12-18 were obese, along with 20% of children ages 6-11 and 10% ages 2-5.1 In 2009 there were 33 states with an obesity

percentage above 25%.2 Obstetricians and gynecologists see more and more patients who fall into the overweight and obese categories. This specialty has an opportunity to impact this epidemic, because the fastest growing obesity group in the United States is young women between the ages of 19 and 28 years.3 A 2007 study by Kim et al. analyzed data from the Pregnancy Risk Assessment Monitoring System, which compared the rate of pre-pregnancy obesity in 1993 and 2003 in nine states. This study found a 69% increase in the prevalence of pre-pregnancy obesity in this time from 13% in 1993 to 22% in 2003.4 Without intervention these numbers will probably continue to rise, putting women and their offspring at risk for numerous complications during pregnancy. Obstetrician and gynecologists have an opportunity to incorporate nutrition and exercise education into the primary care regimen of their patients. Pregnancy and postpartum present unique opportunities to impact the health of women for the rest of their lives. Many women retain a significant amount of weight after pregnancy, predisposing them to become overweight or obese as they gain more weight with age. Pre-pregnancy body mass index, weight gain during pregnancy, and weight loss in the first year postpartum are all independent predictors of body mass index 15 years after pregnancy.5 Almost half of women who are in the normal body mass index range before pregnancy who gain more than the recommended amount during pregnancy will be overweight later in life.6 Due to these factors the time period before pregnancy is an excellent opportunity for weight loss and management counseling. Obesity and excessive weight gain during pregnancy put both mother and baby at risk for many complications. Maternal risks include increased risk of gestational diabetes, type 2 dia-

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betes mellitus, and pregnancy-related hypertensive disorders. Parturition risks include an increase rate of cesarean delivery, decreased success of vaginal delivery after cesarean delivery, and an increase in operative morbidity and mortality including anesthesia complications, excessive loss of blood, postoperative infections, postpartum wound breakdown, and postpartum thrombophlebitis. Obese women are also more likely to suffer from infertility, spontaneous miscarriage, stillbirth, and preterm birth.7 Babies of obese mothers are more often macrosomic, >4500g, which puts them at risk for shoulder dystocia which can lead to cervical plexus injuries, clavicle fracture, fetal hypoxia with or without neurological damage, and even fetal death.8 Maternal complications of shoulder dystocia include postpartum hemorrhage, rectovaginal fistula, third or fourth degree episiotomy tear, and uterine rupture.8 Fetal anomalies are more common in obese patients, with the risk of neural tube defects, ventral wall defects, heart defects, and facial defects increased.9 Unfortunately, ultrasound is more difficult in women who are obese. Specifically, it is more difficult to visualize the fetal heart, diaphragm, kidneys, spine, and umbilical cord.10 Further complicating our issue of obesity, babies born to obese mothers are more likely to suffer from childhood obesity.7 Obstetricians and gynecologists have the opportunity to care for women throughout their lives, long after their pregnancies are over. Coordinating care among providers improves the overall safety and outcomes for health of women. While lifestyle, nutrition, and weight loss counseling are not traditional components of the scope of practice for OB/GYN, they serve an expanded role in primary care. Obstetricians and gynecologists can complement and even multiply the care of internal medicine and family medicine physicians who are trained in comprehensive care. Such counseling is even more important in women who only see obstetricians and gynecologists for health care. After pregnancy we must continue to counsel and educate our patients on weight control because the health consequences of obesity, especially chronic obesity, are numerous. Patients who are overweight or obese are more likely to suffer from high blood pressure, type 2 diabetes, high cholesterol, heart disease, stroke, gallstones, sleep apnea, infertility, depression, and arthritis. Obesity increases the risk of certain cancers including breast and colon cancer.11 The risk of endometrial cancer is about five times higher in obese women than women of normal weight.12

Pre-conception Counseling The goal of preconception counseling is to improve pregnancyrelated outcomes by identifying and modifying any health risks of the potential mother before conception and educating her on methods to reduce these risks.13 Obesity is a modifiable risk factor for gestational complications. Weight loss is inadvisable during pregnancy, so the attainment of a healthy weight must occur prior to pregnancy. Counseling should begin before pregnancy to allow the patient to conceive at a healthy weight. Entering pregnancy at an ideal weight provides the patient with the best opportunity to have a healthy pregnancy and to gain weight appropriately during gestation. Pre-pregnancy body

mass index is the most important predictive factor for weight gain during pregnancy and future obesity.14 Furthermore, weight loss prior to pregnancy reduces the risk of developing diabetes, hypertensive disorders, thromboembolic disease, preterm labor, and cesarean section.15 Reduction of these risks can be a coordinated effort of obstetricians/gynecologists with family medicine physicians, family medicine/obstetric physicians, and internal medicine physicians. A healthy pre-pregnancy weight also helps reduce the risk to the future child. Organ development occurs in the first three to seven weeks of pregnancy so maternal nutrition and weight status during the early stages when a woman may not even know she is pregnant are very important.16 This is especially true considering the first prenatal visit is often at eight weeks into the pregnancy. An important goal for obstetricians and gynecologists may be to provide a higher rate of preconception counseling. Although it provides many benefits to the mother and child, many physicians still do not routinely provide preconception counseling. The Center for Disease Control’s Select Panel on preconception health recommends that all women should receive preconception counseling to allow them to conceive at their healthiest.17 One study surveying obstetricians and gynecologists all over the United States revealed that 87.3% believed preconception counseling is an important issue and 83.5% agreed it has a positive influence on pregnancy, but only 20% say it is a high priority in their practice.18 When asked how often they recommend preconception counseling to obese women, 61.5% said always, 27% said usually, 9.5% said sometimes, and 2% said never.18 When asked how important they felt discussing obesity, physical activity, and nutrition during preconception counseling, 82.7%, 68.3%, and 82.2% ranked them as very important respectively.18 When asked why they thought preconception counseling was very important but not a high priority in their practice, the majority answered that they did not have enough time to provide it, and they were not compensated for the time.18 Obstetricians, gynecologists, and primary care physicians who treat reproductive age women could consider every encounter as an opportunity to discuss reproductive plans. A 2004 survey indicated that 84% of women ages 18-44 had sought medical care in the previous year, and more than one-third of women had more than one primary care health provider.19,20 The interconception period is also a great time for counseling to avoid any risks of complications in a subsequent pregnancy, especially for women who experienced an adverse outcome in a prior pregnancy.15 Approximately 50% of pregnancies in the United States are unplanned, so simply establishing patient care when a woman decides to begin attempting to conceive is not enough.21 In women during the reproductive age, who do not practice contraception, assessment of the risks for pregnancy is important because pregnancy can occur unexpectedly. This is also an opportunity to prescribe contraception to avoid unplanned pregnancies.13 Risk assessment and initial management should begin with the patient’s plan for reproduction including her reproductive,

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medical, surgical, allergy, genetic, family, psychological and social histories, and medications use. Follow-up visits can be scheduled to discuss and treat any identified conditions or risk factors.22 Conditions may fall outside the physician’s scope, experience, and resources, and coordination of care with other physicians may be necessary. It is important to counsel patients on health risks that may affect a future pregnancy and impact long-term health and well-being.

Strategies for Weight Control, Diet, and Exercise Counseling before Conception Obstetricians and gynecologists are the primary care provider for many women and usually see them each year for annual well-woman examinations. Just as the pap smear is viewed as a routine screening test, so might the calculation of the patient’s body mass index. Many electronic medical records automatically compute body mass index (BMI) at each visit along with vital signs, so that it could be readily available to the physician. All women should have their body mass index calculated at least annually and be counseled on its meaning.23 Table 1 presents the meaning of the body mass index ranges. There are three classes of obesity for patients with body mass indices above 30 (Table 2). It is also important to measure waist circumference in all patients, but especially obese patients because a waist circumference greater than thirty-five inches in females further increases the risk of developing diabetes, hypertension, dyslipidemia, and metabolic syndrome.24 Since this recommendation falls outside the scope, training, and experience of most obstetricians and gynecologists, coordination and management among primary care physicians and other specialists would be important. Patients at risk of being overweight or obese patients should have their blood pressure taken, their blood glucose screened, and a lipid panel drawn.25 There should also be a discussion about future plans for pregnancy and the risks of being overweight or obese during pregnancy.17 Table 126 Body Mass Index Ranges

BMI

Weight Category

Less than 18.5

Underweight

18.5-24.9

Normal weight

25-29.9

Overweight

30 and higher

Obese

Table 226 Classes of Obesity

BMI

Obesity Class

30-34.9

Class I

35-39.9

Class II

40.0+

Class III

Diet, Exercise, and Weight Loss Counseling at Preconception Visits Physicians should gauge the patient’s readiness to change before counseling in order to best individualize their counseling efforts. The American College of Obstetrics and Gynecology recommends using the stages of change model to do so. There are five stages we experience when making an important change in our lives. The first is the pre-contemplation phase. During this phase the patient is not aware that there is a problem and is not interested in change. At this time it is appropriate for the physician to educate the patient on her weight status and reasons why it would be beneficial to lose weight. The second phase is the contemplation phase where the patient is aware a problem exists and is thinking about making changes. This is a good time for the physician to discuss anything that is hindering the patient from changing and help her work through these problems and gain support. The preparation phase occurs when the patient realizes a change would be beneficial to her, and she is making small changes or deciding how to change. The physician should now educate the patient on small changes to lose weight slowly. The action phase is in place when the patient has enacted some changes in her life. The physician is now an important source of support for the patient and can also help her make further changes that she may not have been ready for previously. When the patient’s initial goal has been met, the patient enters the maintenance phase. At this point the physician should assess the progress and help the patient to maintain her changes or move forward by setting new goals and increasing change.23 When counseling begins, all patients should have a dietary assessment and be counseled on the components of a healthy diet. Patients should be screened on what a typical day is like for them nutritionally. Because most physicians lack time to fully assess this topic with each patient, a questionnaire could be used, and patients could fill it out while waiting to see their physician. The questionnaire should assess how many servings are consumed each day of the major food groups. The components of a healthy diet include fresh fruits and vegetables of all color selections; lean protein such as chicken, fish, turkey, and lean cuts of pork and beef; nuts; low and no fat dairy; and whole grain breads. Meats and vegetables should be grilled, broiled, or baked instead of fried.27 Overweight and obese women should be encouraged to reduce their caloric intake. Resources such as mypyramid.gov can help give patients a good idea of how many calories they need each day and how many servings of the different food groups they need. Exercise is crucial to optimal health, and, because most Americans do not meet the minimum requirements for physical activity, physicians must address this issue. Exercise provides health benefits for all women and is an important component of weight loss. Women who exercise on a regular basis are less likely to develop coronary artery disease, high blood pressure, diabetes, breast and colon cancer, osteoporosis, or suffer a stroke. Exercise also helps to stabilize mood and reduce symp-

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toms of depression and anxiety.28 Exercise prior to pregnancy benefits the patient during pregnancy by helping her to control her weight and helping with her mood, possibly reducing her risk of postpartum depression.17 As seen in the section above, overweight and obesity present the patient with numerous complications during pregnancy, and postpartum depression can be severe and life threatening so these are very important factors. Current physical activity recommendations from the Centers for Disease Control are 150 minutes of moderate aerobic activity, such as brisk walking, each week and two days of strength training for all the major muscle groups of the body: arms, legs, back, abdomen, chest, shoulders, and hips. An alternative to this regimen is 75 minutes of vigorous aerobic activity, such as jogging, per week and strength training of all the major muscle groups two times per week. This may sound like a lot to patients who are not currently exercising, but they should be encouraged to start out slowly and work their way up. They can begin with 10-minute walking sessions and build their way up. Any exercise is preferable to being sedentary. As patients builds up to the current minimum recommendations, they should be further encouraged to build up their exercise session times. The longer they exercise each week, the greater their health benefits will be. For even greater health benefits the Center for Disease Control recommends 300 minutes of moderate aerobic activity or 150 minutes of vigorous physical activity each week combined with two sessions of muscle strengthening exercises.29 There are a variety of aerobic exercises that the patient can do to fulfill the weekly requirements. Table 3 lists several options. Table 330 Aerobic Exercises

Moderate Aerobic Exercise: Walking briskly, doubles tennis, biking on level ground or at 5-9 miles per hour, pushing a lawn mower, elliptical trainer, ballroom or line dancing, gardening, roller skating, hiking small hills, golf, softball, baseball. Vigorous Aerobic Exercise: Swimming, jogging or running, riding a bike on hills or at a speed greater than 10 miles per hour, playing basketball, playing singles tennis, mountain climbing, backpacking, jumping rope, hiking uphill. Patients with a body mass index of 25 and above should be counseled on the basics of weight loss and referred to outside services for help with weight loss as necessary. The ultimate goal is for patients to be of a normal weight and body mass index prior to conception with further goals of maintaining the lower body weight and controlling any complications from overweight or obesity the patient may have. The loss of 10% of body weight should be the initial goal, because even this small amount of loss reduces the patient’s risk of hypertension, diabetes, and hyperlipidemia.31 Further weight loss should be at a rate of one to two pounds per week to prevent excessive loss of lean body mass, dehydration and electrolyte disturbances, gallbladder disease, and psychological problems.31 Patients should understand that they are making healthy lifestyle chang-

es, which should be long-term, rather than a temporary change to lose weight. Caloric balance is the basic concept all patients should know in order to help them lose weight. The patient must burn 3500 calories in excess of the calories they consume in order to lose one pound. It is recommended that patients aim for a 500-calorie deficit each day.32 The most effective approach to achieve this calorie deficit is by reducing caloric intake through diet and increasing caloric expenditure by increasing exercise. The resting energy expenditure equation is a good method to calculate how many calories a patient burns in a day and needs to consume to maintain their current weight. Using this equation, and subtracting 500 calories from the result, will give the patient a good idea of how many calories to consume for weight loss. Equation 1 shows the resting energy expenditure equation. Equation 131 Resting Energy Expenditure

Step 1 For adult men REE= 10 x weight (kg) + 6.25 x height (cm) – 5 x age (years) + 5 For adult women: REE= 10 x weight (kg) + 6.25 x height (cm) – 5 x age (years) -161

Step 2: Multiple the REE by an activity factor Activity Factor: 1.5 for women, 1.6 for men who perform light activity most days, and 1.6 for women, and 1.7 for men with higher activity levels REE x Activity Factor= Daily Caloric Need to Maintain Weight Reducing caloric intake is accomplished through dietary changes or a reduction in absorption of food consumed. There are many diet approaches patients can take, including low fat, low carbohydrate, and high protein diets. All diets provide roughly equal amounts of weight loss, provided the patient achieves the same calorie deficit.33 Low carbohydrate diets may provide greater weight loss in the first three to six months, but loss at one year is similar between all diets.34 The concept of reducing the energy density of the foods consumed is an important one. The energy density of a food is the amount of calories provided in a given weight of the food. Fruits and vegetables are low energy density foods so they keep you full longer by allowing you to consume more for a given calorie allowance.32 Eating less energy-dense foods helps by allowing you to eat more under the same calorie allowance. The most important concept for diet change is that the patient incorporates healthy foods and reduces their intake for calorie deficit. Food journals, daily weighing at home, and diet programs may be beneficial to some

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patients. In the right circumstances pharmacologic agents and even surgical procedures can be explored for patients to reduce the amount of energy absorbed through calories consumed. Weight loss is achieved when a calorie deficit is created, and physical activity helps burn additional calories above resting metabolic rate to create this deficit.35 For weight loss, experts recommend an hour to an hour and a half of daily exercise.36 The patient should acknowledge that their diet is a very important component of weight loss even if they are exercising, because an increase in caloric intake can offset the calories burned through exercising, causing the patient to maintain their weight or even gain weight. Exercise is difficult, and it takes a significant time commitment each day to burn calories, so patients should keep a strict diet to maintain a calorie deficit.35 Losing weight through diet will also make exercise easier because it will then put less stress on the joints, and the patient will be able to move around more easily. Studies have revealed that more weight is lost through diet or diet and exercise combined than through exercise alone.37 Lifestyle changes are very difficult for most patients to make. Most people require repeated motivation to make small changes in their diet and exercise regimen. This is not a reason for physicians to avoid the issues but a reason to continue to reinforce the importance of these changes. Recommendations should be made in the office, educational pamphlets should be sent home, and counseling should continue at every followup visit. These repeated discussions help keep the issues in the forefront of patients’ minds and help them to realize it is important to their physician. Creating an action plan and assessing progress made through the plan can help patients and physicians assess change. A plan is made as a joint effort by the patient and physician, including a goal for weight loss and a plan of how to achieve it. The physician should ask their patients what changes they have made since their previous visit, revisit the action plan, and make changes to it as necessary.38 Physician support is key throughout this process. The physician should always counsel their patients about weight loss in a non-judgmental, sensitive way. The physician and patient are a team in this process, and the patient should always know their physician will care for them even if goals are not met quickly or at all. Support at home is crucial as well. Patients should be encouraged to find a partner at home to help them with their health goals. If the rest of the family is sedentary and snacking on junk foods, it will be difficult for the patient to change, but women with support at home are more likely to be motivated to make healthy changes.39 Merri M. Paden, MD, graduated from the University of Alabama School of Medicine. She now serves as a resident in OB/ GYN, University of Alabama School of Medicine. Daniel M. Avery, Jr., MD, is Professor and Chair of OB/GYN, University of Alabama School of Medicine, Tuscaloosa. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might ®

create any potential conflict of interest. The author has stated that no such relationships exist.

Conclusion Primary care physicians for women have the opportunity to address the current obesity crisis in America. Obstetricians and gynecologists can address nutrition, physical activity, and weight management in the preconception period. Physicians can be effective in reducing the rate of overweight and obesity in our patients. By addressing this sensitive subject, physicians can partner with patients to achieve a healthier life.

References 1.

Obesity and Overweight. Centers for Disease Control and Prevention. 2010. 10 May 2011. http://www.cdc.gov/nchs/fastats/overwt.htm.

2.

U.S. Obesity Trends: Trends State by State 1985-2009. Centers for Disease Control and Prevention. 2011. 10 May 2011. http://www.cdc.gov/ obesity/data/trends.html.

3.

Mokdad AH, Serdula MK et al. The spread of the obesity epidemic in the United States. 1991-1998. JAMA. 1999;282:1519-1522.

4.

Shin K, Dietz P, England L, Morrow B, Callaghan W. Trends in Prepregnancy Obesity in Nine States. Obesity. 15.4 (2007): 986-993.

5.

Rooney B, Schauberger CW, et al. Impact of perinatal weight change on long-term obesity and obesity-related illnesses. Obstet Gynecol. 2005;106:1349-1356.

6.

Linne Y, Dye L, et al. Long-term weight development in women: A 15year follow-up of the effects of pregnancy. Obes Res. 2004;12:1166-1178.

7.

Leddy M, Power M, Schulkin J. The Impact of Maternal Obesity on Maternal and Fetal Health. Reviews in Obstetrics and Gynecology. 1.4 (2008): 170-178.

8.

Baxley E, Gobbo R. “Shoulder Dystocia.” American Family Physicians. 69.7(2004);1707-1714.

9.

Nuthalapaty FS, Rouse DJ. The impact of obesity on obstetrical practice and outcome. Clin Obstet Gynecol. 2004;47:898-913.

10. Hendler I, Blackwell SC, et al. Suboptimal second-trimester ultrasonographic visualization of the fetal heart in obese women: should we repeat the examination? J Ultrasound Med. 2005;24:1205-1209. 11. Obesity: Complications. Mayo Clinic. 2011. 14 May 2011. http://www. mayoclinic.com/health/obesity/DS00314/DSECTION=complications. 12. Schouten LJ, Goldbohm RA, van der Brandt PA. Anthropometry, physical activity, and endometrial cancer risk: results from the Netherlands Cohort Study. J Natl Cancer Inst. 2004;96:1635-1638. 13. ACOG Committee Opinion number 313, September 2005. The importance of preconception care in the continuum of women’s health care. Obstet Gynecol. 2005;106: 665-666. 14. Krummel DA. Postpartum weight control: a vicious cycle. J Am Diet Assoc. 2007;107:37-40. 15. Dunlop A, Jack B, Frey K. National Recommendations for Preconception Care: The Essential Role of the Family Physician. JABFM. 2007;20.1:81-84. 16. Williamson CS. Nutrition in pregnancy. Nutrition bulletin. 2006;31:28-59. 17. Moos M, Dunlop A, et al. Healthier Women, healthier reproductive outcomes: recommendations for the routine care of all women of reproductive age. Am J Obstet Gynecol. 2008;199:S280-S289. 18. Morgan M, Hawks D, et al. What Obstetrician-Gynecologists Think of Preconception Care. Matern Child Health J. 2006;10:S59-S65. 19. Salganicoff A, Ranji U, Wyn R. Women and health care: a national profile

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- key findings from the Kaiser Women’s Health Survey. Menlo Park, CA: Kaiser Family Foundation; 2005. http://www.kff.org/womenshealth/ upload/Women-and-Health-Care-A-National-Profile-Key-Findingsfrom-the-Kaiser-Women-s-Health-Survey.pdf. 20. Weisman CS. Women’s use of health care. In: Falik M, Collins K, eds. Women’s Health: the Commonwealth Fund Survey of Women’s Health. Baltimore, MD: Johns Hopkins University Press: 1996. 21. Henshaw S. Unintended pregnancy in the United States. Fam Plann Perspect. 1998;30:24-9,49. 22. Lu M. Recommendations for Preconception Care. Am Fam Physician. 2007;76.3:397-400 23. ACOG Committee Opinion number 319, October 2005. The role of the Obstetrician-Gynecologist in the Assessment and Management of Obesity. Obstet Gynecol. 2005;106:895-899. 24. Janssen I, Katzmarzyk P, et al. Body Mass Index, Waist Circumference, and Health Risk, Evidence in Support of Current National Institutes of Health Guidelines. Arch Int Med. 2002;162: 2074-2079. 25. Kushner RF. Roadmaps for Clinical Practice Case Studies in Disease Prevention and Health Promotion- Assessment and Management of Adult Obesity: A Primer for Physicians. Chicago, Ill: American Medical Associations; 2003. 26. Classification of Overweight and Obesity by BMI, Waist Circumference, and Associated Disease Risk. National Heart, Lung, and Blood Institute 2011. 11 May 2011. http://www.nhlbi.nih.gov/health/public/heart/obesity/ lose_wt/bmi_dis.htm. 27. Healthy Eating for a Healthy Weight. Centers for Disease Control and Prevention 2011. 13 May 2011. http://www.cdc.gov/healthyweight/healthy_ eating/index.html. 28. Physical Activity and Health: The Benefits of Physical Activity. Centers for Disease Control and Prevention 2011. 13 May 2011. http://www.cdc. gov/physicalactivity /everyone/health/index.html

29. Physical Activity for Everyone: How much physical activity do adults need? Centers for Disease Control and Prevention. 2011. 13 May 2011. http://www.cdc.gov/physicalactivity/everyone/guidelines/adults.html. 30. Physical Activity for Everyone: Measuring Physical Activity Intensity. Centers for Disease Control and Prevention 2011. 13 May 2011. http:// www.cdc.gov/physicalactivity/everyone/measuring/index.html. 31. Lyznicki J, Young D, et al. Obesity: Assessment and Management in Primary Care. Am Fam Physician. 2001;63:2185-2196. 32. Thompson W, Cook D, et al. Treatment of Obesity. Mayo Clin Proc. 2007;82.1:93-102. 33. Dansinger ML, Gleason JA, et al. Comparison of the Atkins, Ornish, Weight Watchers, and Zone diets for weight loss and heart disease risk reduction: a randomized trial. JAMA. 2055;293:43-53. 34. Foster GD, Wyatt HR, et al. A randomized trial of a low-carbohydrate diet for obesity. N Engl J Med. 2003;348:2082-2090. 35. Jakicic J, Otoo A. Treatment and Prevention of Obesity: What is the Role of Exercise? Nutrition Reviews. 2006;64.2:S57-S61. 36. Physical Activity and Weight Control. Weight-Control Information Network, National Institute of Diabetes and Digestive and Kidney Diseases 2011. 13 May 2011. http://win.niddk.nih.gov/publications/ physical.htm. 37. Hagan RD, Upton SJ, Wong L, Whittam J. The effects of aerobic conditioning and/or calorie restriction in overweight men and women. Med Sci Sports Exerc. 1986;18:87-94. 38. Handley M, MacGregor K, et al. Using Action Plans to Help Primary Care Patients Adopt Healthy Behaviors: A Descriptive Study. J Am Board Fam Physicians. 2006;19.3:224-231. 39. Evenson K, Aytur S, Borodulin K. Physical Activity Barriers, and Enablers among Postpartum Women. Journal of Women’s Health. 2009;18:12.1925-1934.

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Heterotaxy Syndrome in a Young Adult Andres Abreu, DO, MS Maria Luisa Nieto-Morales, MD, Ph.D Margarita Fuentes, MD

Abstract

Terminology

Heterotaxy syndrome is found infrequently in the adult population. The syndrome is characterized by the presence of abnormal position of the viscera. If the patient does not present with cardiac anomalies incompatible with life, it can be asymptomatic, being diagnosed only incidentally in adulthood (5-10%). We present the case of a 36-year-old with heterotaxy syndrome who was diagnosed after presenting to the emergency department with symptoms of pulmonary thromboembolism. After several radiology diagnostic studies, we observed interruption of the inferior vena cava along its course to the level of the renal venin with subsequent dilatation of the azygous vein to the level of the superior vena cava. Polysplenia and drainage of suprahepatic veins directly into the right atrium was also noticed.

The term situs refers to the position of the atria and viscera relative to the midline. The atrium whose appendage is broadbased and receives blood from the inferior vena cava may best be called the systemic or right atrium. The atrium with the smaller, narrower appendage and that receives blood from the pulmonary veins is called the pulmonary or left atrium.

Introduction

Situs solitus is the usual arrangement of organs and vessels within the body. The systemic atrium is on the right with a right-sided trilobed lung, liver, gallbladder, and inferior vena cava. The pulmonary atrium is on the left with a left-sided bilobed lung, stomach, single spleen, and aorta. The cardiac apex is on the left. The incidence of congenital heart disease in patients with situs solitus and levocardia is only 0.6%–0.8%.2 Figure 1: Polysplenia and Situs Inversus

Situs anomalies are rare, complex, and confusing. Situs ambiguous, or heterotaxy, implies a disordered organ arrangement in the chest or abdomen. This unpredictable anatomy differs from the orderly arrangement of the truncal organs in either the typical anatomy (situs solitus) or the mirror image of it (situs inversus). Situs anomalies present a diagnostic challenge to the radiologist. Attempts at classification are difficult and usually oversimplify cases, since many patients do not demonstrate the expected, classical findings. We believe it is essential to understand situs abnormalities and their appearances on radiologic studies in order to recognize which children are at increased risk of congenital heart disease, immune deficiency (due to splenic absence), and catastrophic volvulus with malrotation. Heterotaxy Syndrome in a Young Adult


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Situs inversus (Fig 1) refers to an anatomic arrangement that is the mirror image of situs solitus. The systemic atrium is on the left with a left-sided trilobed lung, liver, gallbladder, and inferior vena cava. The pulmonary atrium is on the right with a right-sided bilobed lung, stomach, single spleen, and aorta. The cardiac apex is on the right. Situs inversus is seen in 0.01% of the population,2 and the incidence of congenital heart disease in patients with situs inversus is 3%–5%.26 Figure 2: Asplenia – Right Sided Isomerism

Situs ambiguous, or heterotaxy, refers to visceral malposition and dysmorphism associated with indeterminate atrial arrangement. This abnormal arrangement of body organs is different from the orderly arrangement seen in situs solitus or situs inversus. The incidence of congenital heart disease in patients with heterotaxy is very high, ranging from 50% to nearly 100%. In asplenia (right isomerism or bilateral right-sidedness), both lungs have three lobes and eparterial bronchi (Fig 2, 5).23 The main bronchus is located superior to the ipsilateral main pulmonary artery on each side. In polysplenia (i.e., left isomerism or bilateral left-sidedness), both lungs have two lobes and hyparterial bronchi (Fig 3).23 In this situation, the reverse is seen: The main bronchus passes inferior to the ipsilateral main pulmonary artery on each side (Fig 4, 6). Figure 3: Polysplenia - Left Isomerism

Figure 5:Asplenia with bilateral Minor Fissure and Trilobed lung

Figure 4: Polysplenia with hyparterial bronchi and bilobed lung

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Figure 6: Polysplenia with hyparterial bronchia and bilobed lung

Figure 7: Interruption of Inferior Vena Cava with Azygous Continuation

Heterotaxy Syndrome with Asplenia The designation of classic right isomerism or bilateral rightsidedness (Fig 2)23 implies that the patient has bilateral trilobed lungs with bilateral minor fissures and eparterial bronchi, bilateral systemic atria, a centrally located liver, and a stomach in indeterminate position. The abdominal aorta and inferior vena cava would classically be located on the same side of the spine (Fig 9).

Figure 8: Polysplenia with interrupted IVC and azygous continuation.

Figure 9: Heterotaxy Syndrome with Asplenia

This syndrome occurs more frequently in males, and patients often present with cyanosis and severe respiratory distress. At chest radiography, the cardiac apex typically appears discordant from that of the stomach and liver. The stomach may also be midline and small (microgastria). Common cardiac malformaHeterotaxy Syndrome in a Young Adult


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tions include a common atrioventricular canal, univentricular heart, transposition of the great arteries, and anomalous venous connections including total anomalous pulmonary venous return (seen in the majority of cases). The complex cardiac anomalies and abnormal immune status are closely linked to the understandably poor prognosis of this group of patients. Death occurs in the first year of life in up to 80% of cases.

cava with azygous or hemiazygous continuation (Fig 7, 8, 10, 11, 12, 13)26 may be suggested from the chest radiograph and is the most consistent finding seen in heterotaxy with polysplenia. Figure 12: Polysplenia with interrupted IVC and azygous continuation

Figure 10: Polysplenia with interrupted IVC and azygous continuation.

Figure 13: Polysplenia with interrupted IVC and azygous continuation

Figure 11: Polysplenia with interrupted IVC and azygous continuation

Heterotaxy Syndrome with Polysplenia Classic left isomerism or bilateral left-sidedness (Fig 3)23 implies that patients have bilateral bilobed lungs, bilateral pulmonary atria, a centrally located liver, a stomach in indeterminate position, and multiple spleens. Interruption of the inferior vena

Polysplenia (Fig 14) is more common in females and has more variable clinical manifestations and prognosis. Fewer polysplenic patients (vs asplenic patients) present with cyanosis and more present with symptoms of congestive heart failure from left-to-right shunts. Absence of the inferior vena cava on the lateral chest radiograph and the azygous continuation on the frontal chest radiograph with discordance of the apex and abdominal viscera help suggest the diagnosis (Fig 10, 11, 12).26 In general, cardiac anomalies are less common in polysplenic pa-

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tients and not as complex as those in asplenic patients (the most common cardiac anomalies in this group are partial anomalous pulmonary venous return, atrial septal defect, and atrioventricular canal). Figure 14: Polysplenia

nosis. With chest radiography, one can determine the presence or absence of normal situs (i.e., situs solitus). Normal situs is inferred when the aortic arch, cardiac apex, and stomach bubble are all located on the left. When these structures are positioned on the right or reversed, situs inversus is present (Fig 15). When any other situation is seen, an indeterminate situs or heterotaxy is inferred (Fig 16). The position of the atria may be inferred from the venous drainage below the diaphragm in a patient with situs solitus or situs inversus. However, in the presence of heterotaxy, even the echocardiographer may not be able to identify the appendages. The stomach may be displaced from its normal location directly under the hemidiaphragm by a bridging (midline) liver in either asplenic or polysplenic patients (Fig 17). Figure 16: Indeterminate situs – stomach on left side

Figure 15: Dextrocardia, right-sided stomach, left-sided liver

Figure 17: Levocardia, right-sided stomach bubble, and polysplenia side

Radiologic Evaluation The critical structures to be evaluated with imaging in determining situs are (a) position of the atria; (b) position of venous drainage below the diaphragm relative to midline; (c) position of the aorta relative to midline; (d) position of the stomach and presence of malrotation; (e) position of the liver and gallbladder; (f) position of the cardiac apex; (g) presence, appearance, and number of spleens; and (h) presence of tri- or bilobed lungs, including presence or absence of bilateral minor fissures. These anatomic structures may be evaluated with chest radiography, ultrasonography (US), CT, MR imaging, and angiocardiography, although the last study is now rarely necessary for diagHeterotaxy Syndrome in a Young Adult


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Case Presentation Modes of inheritance proposed for heterotaxy include autosomal dominant, autosomal recessive, and X-linked recessive. The last mode may help in part to explain the male preponderance of this syndrome. Overall, though, careful genetic study supports a multifactorial inheritance.2,3, 4 The wide spectrum and variability of presentation of this syndrome is quite broad, and there is no single pathognomonic anomaly that can be cited.5,6 The wide spectrum of anomalies in the heterotaxy syndrome is explained by the variable duration of embryological causative factors during fetus development. A small portion of these patients present with cyanosis, while others present with symptoms of congestive heart failure with left to right shunt. The exact moment of embryological development of these anomalies is not known, but it is believed that it occurs at approximately 28 days of embryological development.7 It is during this time period that the primitive heart and venous connections form. Disruption of this early embryologic event, when the cardiac chambers are incompletely septated, helps explain the preponderance of common atria, single ventricles, abnormal pulmonary venous connections, and conotruncal anomalies observed in heterotaxy syndrome (Fig 18).

similar clinical presentation. The presence of deep venous thrombosis in patients with heterotaxy is a more frequent finding than in the general population. It is for this reason that this entity must be considered in the differential diagnosis of deep venous thrombosis in patients younger than 30 years of age. Interruption of the inferior vena cava (Fig 8, 10, 11, 12, 13)26 seen in patients with heterotaxy gives rise to an abnormal venous drainage of the lower extremity venous system that increases the risk of deep venous thrombosis,10,11 and therefore pulmonary thromboembolism.26 Similarly, an anomalous drainage of the splenic venous system into the azygous system predisposes to an increase in platelet aggregation, which also augments the risk of pulmonary thromboembolism. Figure 19: Pulmonary embolism in left Main Pulmonary Artery

Figure 18: Common Atrioventricular Canal with Common Atrium

Our patient presented with a filling defect in the right main renal artery, which may have been the source of her pulmonary thromboembolism. Polysplenia (Fig 1, 13, 14)26 is another anomaly found in patients with heterotaxy syndrome. Multiple nonfunctining splenules, ranging from five to 16, are seen, which are mostly located in the region of the greater curvature of the stomach. This latter finding is explained due to the embryological development of the spleen in the dorsal mesogastric region. In many cases, the abnormal position of the stomach is the only evidence of intestinal malrotation. Only 40% of the affected population reaches two years of age, and most die before the age of five. Only 5-10% of these patients present with normal heart size or with minor cardiac anomalies which allowed the individual to reach adulthood without demonstrating any significant symptoms.8,9 Our 26 year old patient in this case study did not present with any significant clinical manifestations of heterotaxy, except for patent foramen ovale. In this case, the diagnosis of heterotaxy was an incidental one obtained when imaging studies were performed for other causes, in this case pulmonary embolism. Our patient was admitted to the hospital due to symptoms of pulmonary thromboembolism (Fig 19). Prior to this case, there has only been one other case published in the literature with

Bowel malrotation (Fig 20) is also found in heterotaxy syndrome. In a study performed by Ditchfield and Hutson on six pediatric patients with polysplenia,12 all patients also had abnormalities related to bowel malrotation and midgut volvulus. Our patient did not present with any abdominal complaints. However, due to the known bowel abnormalities described in the literature, we recommended performing an upper gastrointestinal examination to exclude the possibility of bowel malrotation or other bowel anomalies. The normal mesentery has a broad base of attachment, which extends from the Ligament of Treitz in the left upper abdomen to the ileocecal junction in the right lower abdomen. Patients presenting with complete or partial bowel malrotation often

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have a thinner mesenteric base, which makes it more prone to the development of volvulus. Figure 20: Malrotation with duodenum and Jejunum on patient’s right side

Our patient was diagnosed with inversion of the mesenteric vessels16 (Fig 21). Although this finding is normally sufficient to diagnose bowel malrotation,8 a recent study demonstrated bowel malroation in only two out of eight patients with mesenteric vasculature inversion.9 Vascular inversion, however, can also be seen in cases of abdominal masses or duodenal atresia. Zerin et al. documented a case of bowel malrotation with midgut volvulus and normal position of the mesenteric vessels. This latter finding demonstrates that inversion of the mesenteric arteries and veins are not pathognomonic of bowel malrotation, because in some of these patients there may be a normal position of the mesenteric vessels.14 An inverse relationship has also been described in which there is inversion in the position of mesenteric arteries and veins without malroation. Because of the reasons stated above, mesenteric inversion is an important finding, which dictates a thorough evaluation of the bowel. Figure 21: Midline Aorta and Left sided IVC

As fluoroscopic upper gastrointestinal barium examination does not provide specific information to suggest which patients with malrotation are at risk for developing midgut volvulus, some institutions recommend performing preventive Ladd’s surgical technique in these patients.13 It is important to be aware of alteration in the position of vascular structures if a surgical procedure is to be performed in these patients. It is estimated that intestinal and duodenal malrotation is diagnosed incidentally in approximately 0.2% of the population and only some cause symptoms in adulthood.12,13,14 Barium enema studies on these patients often shows a malrotation of the ascending colon, but the cecum may demonstrate a normal position in more than 20% of these patients, which must be taken into consideration in order not to miss the diagnosis of malrotation.

Bowel malrotation can be studied with fluoroscopic upper gastrointestinal examination and oral contrast CT examination, paying particular attention to the normal horizontal orientation of the third portion of the duodenum, the position of the ligament of Treitz, and the position of the small and large bowel with the small bowel on the right and large bowel on the left side of the abdomen in malrotation17 (Fig 20).

Applegate et al. performed a study in ten patients with heterotaxy with polysplenia and noticed that the position of the aorta and stomach was variable, but the bowel malrotation was a constant finding.15 In a study performed by Fulcher et al. on eight patients with heterotaxy, they remarked that in five out of seven patients with bowel malrotation the small bowel was localized primarily to the right and the colon to the left side of the abdomen. The stomach was located on the right side of the abdomen in three patients and to the left side in five patients.1

In a study performed by C.K. Chon et al. to diagnose bowel malrotation by CT,18, 19 it concluded that only if there is inversion in position of the superior mesenteric vein and artery along with right-sided first and second jejunal branches can bowel malrotation be diagnosed with certainty. If bowel malrotation can be diagnosed with oral contrast CT, it is not necessary to perform upper GI examination. This can be done in cases in which the diagnosis is still in question after oral contrast CT.

In a study performed by Galler et al. on 15 patients with polysplenia diagnosed in infancy, ten of these patients had either complete or incomplete bowel malrotation.

Given that the development of the pancreas during embryogenesis is closely associated with duodenal jejunal rotation, it is not surprising that bowel malrotation is directly related with abnormal pancreatic morphology.13, 20 There is little reference

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in the literature regarding the morphology of the pancreas in patients with polysplenia. In a study performed by Hatayama and Wells on six pediatric patients with polysplenia and other congenital anomalies, they described a short, thick pancreas. The presence of a short pancreas was mostly due to abnormal embryological development of the dorsal bud. In spite of the short size of the pancreas, the patients did not demonstrate any evidence of diabetes. The presence of abnormal morphology of both pancreas and spleen is due to the fact that both organs develop in the dorsal mesogastrium.21 If, after performing an endoscopic retrograde pancreatograph (ERCP) on a patient with polysplenia, only a short segment of the pancreatic duct opacifes, it is reasonable to conclude that this is due to the presence of a truncated pancreas rather than pancreatic divisum or pancreatic mass obstructing the pancreatic duct. Hader et al. demonstrated the presence of a short pancreas in adult patients with polysplenia. Oral contrast CT in our patient demonstrated the presence of a pancreas with absent body and tail. It is worth noting the presence of a preduodenal portal vein in these patients, which can interfere with the normal pancreatic development, thereby increasing the risks of pancreatic anomalies such as anular pancreas.8,22 Knowing the existence of a preduodenal portal vein is important because of the risk of inadvertent injury during surgical procedures. A bilobed lung is another type of malformation found in patients with polysplenia.

Margarita Fuentes, MD, is Staff Radiologist, Hospital Universitario de Canarias, La Laguna, Spain. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

References 1.

Winer-Muram HT, Tonkin ILD. The spectrum of heterotaxic syndromes. Radiol Clin North Am. 1989;27:1147-1170.

2.

Ito K, Matsunaga N, Mitchell DG, et al. Imaging of congenital abnormalities of the portal venous system. AJR. 1997;168:233-237.

3.

Puvaneswary M, Rajaratnam S. Midgut volvulus in an adult. Australasian Radiology. 2003;47:83-84.

4.

Zissin R, Rathaus V, Oscadchy A, et al. Intestinal malrotation as an incidental finding on CT in adults. Abdomin Imaging. 1999;24:550-555.

5.

Gayer G, Apter S, Jonas T, et al. Polysplenia syndrome detected in adulthood: report of eight cases and review of the literature. Abdomin Imaging. 1999;24:178-184.

6.

Plata-Muñoz JJ, Hernández-Ramírez D, Antón FJ, et al. Polysplenia syndrome in the adult patient. Case report with review of the literature. Ann Hepatology. 2004;3(3):114-117.

7.

Herman TE, Siegel MJ. Polysplenia syndrome with congenital short pancreas. AJR. 1991;156:799-800.

8.

Nichols DM, Li DK. Superior mesenteric vein rotation: a CT sign of midgut malrotation. AJR. 1983;141:707-708.

9.

Zerin JM, DiPietro MA. Mesenteric vascular anatomy at CT: normal an abnormal appearances. Radiology. 1991;179:739.

10. Chou CK, Mak CW, Hou CC, et al. CT of the mesenteric vascular anatomy. Abdomin Imaging. 1997;22:477-482.

Conclusion Review of the literature, recent breakthroughs in embryologic understanding, and our own experience illustrate the complexity and spectrum of abnormalities seen in patients with the heterotaxic syndromes. We propose that the term heterotaxy syndrome be used to described these patients and that the patient’s individual anatomy be described, rather than using the classic but imprecise terms isomerism, asplenia, or polysplenia. In our case, CT examination confirmed the findings of hypoplasia of the inferior vena cava with absence of the intrahepatic segment and direct continuation with the azygous venous systems, which, following polysplenia, is the most frequent finding found in heteroxtaxy. We believe it is essential to understand situs abnormalities and their wide variety of appearances on radiologic studies. A more accurate classification approach to each patient with heterotaxy is the most practical way to recognize those children who are at increased risk of congenital heart disease, immune deficiency (splenic absence), and catastrophic volvulus with malrotation. Andres Abreu, DO, MS, is Radiology Medical Director, Dimensional Imaging Inc., Sarasota, Florida. María Luisa Nieto-Morales, MD, PhD, is Staff Radiologist, Hospital Universitario Nuestra Señora de Candelaria, Santa Cruz de Tenerife, Spain.

11. Hoeper MM, Niedermeyer J, Hoffmeyer F, et al. Pulmonary hypertension after splenectomy? Ann Intern Med. 1999;130(6):506-509. 12. Deveci MS, Ankara GD. Biliary atresia splenic malformation syndrome Is it a result of embryonically midline rotational defects? A case report. J Pediatric Surg. 2000;35(9):1377-1380. 13. Pickhardt PJ, Bhalla S. Intestinal malrotation in adolescents and adults: spectrum of clinical and imaging features. AJR. 2002;179:1429-1435. 14. Vázquez J, López GJC, Gámez M, et al. Biliary atresia and polysplenia syndrome: its impact on final outcome. J Pediatr Surg. 1995;30(3):485487. 15. Castro FJ, Pérez C, Narváez FJ, et al. Agenesia de vena cava inferior como factor de riesgo de tromboembolismo pulmonar. Ann Med Interna. 2003;20(6):34-36. 16. Vincent M, Brandenburg MD, Stefan Krueger MD, et al. Heterotaxy syndrome with severe pulmonary hypertension in an adult. South Med J. 2002;95(5):536-538. 17. Fisher JK. Computed Tomographic diagnosis of volvulus in intestinal malrotation. Radiology. 1981;140:145-146. 18. Inoue Y, Nakamura H. Aplasia or hypoplasia of the pancreatic uncinate process: comparison in patients with and patients without intestinal malrotation. Radiology. 1997;205:531-533. 19. Ruben GD, Templeton JM, Jr, Ziegler MM. Situs inversus: the complex inducing neonatal intestinal obstruction.J Pediatr Surg1983; 18:751-756. 20. Hadengue A, Benhayoun MK, Lebrec D, et al. Pulmonary hypertension complicating portal hypertension: prevalence and relation to splacnic hemodynamics. Gastroenterology. 1991;100:520-528. 21. Soler R, Rodríguez E, Comesaña ML, et al. Agenesis of the dorsal pancreas with polysplenia syndrome: CT features. J Comput Assist

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Tomogr. 1992;16(6):921-923. 22. Fulcher AS, Turner MA. Abdominal manifestations of situs anomalies in adults. Radiographics. 2002;22:1439-1456. 23. Applegate KE, Goske MJ, Pierce G, et al. Situs revisited: Imaging of the heterotaxy syndrome. Radiographics. 1999;19:837-852.

24. Ditchfield MR, Hutson JM. Intestinal rotational abnormalities in polysplenia and asplenia syndromes. Pediatr Radiol. 1998;28:303-306. 25. Ito K, Matsunaga N, Mitchell DG, et al. Imaging of congenital abnormalities of the portal venous system. AJR. 1997;168:233-237. 26. Demos TC, Posniak HV, Pierce K, Olson M, Muscato M, Venous Anomalies of the Thorax. AJR. May 2004 Vol. 182 No 5 1139-1150.

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American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

Case Report Cellulitis Following Newborn Circumcision Daniel M. Avery Jr., MD Daniel M. Avery, III, MD John T. McDonald, Jr., MD Joseph C. Wallace, MD

An obstetrics fellow performed a circumcision using a Plastibell® Circumcision Device on a healthy, term newborn male without incident several hours before discharge. The parents returned with the infant to the hospital emergency department because of a “bubble at the end of his penis the size of a grape.” The child was visibly distressed and crying, but this stopped when the “bubble” burst. An emergency medicine attending and a family medicine resident covering OB/GYN and pediatrics examined the newborn. Several diagnoses were entertained including an allergic reaction. Both physicians thought that the findings were not serious, and the child was discharged home with follow up the next day in the pediatric clinic. The child was seen in the pediatric clinic the following afternoon for a routine bilirubin check. Although the parents thought that the baby was improving, the penis was erythematous and swollen. The examining pediatrician diagnosed cellulitis of the penis, and the child was taken to The Children’s Hospital for further assessment and consultation with a pediatric urologist. Admitting diagnosis was cellulitis of the penis and scrotum associated with circumcision. The child was started on triple antibiotic therapy following a septic workup. The only positive culture was the cerebrospinal fluid culture which grew alpha hemolytic streptococcus on day three and was considered contamination. The erythema and edema resolved by hospital day number two. Of interest, the attending pediatrician reported that he had never seen a penis become infected following circumcision. The child was discharged after six days of intravenous antibiotics on Augmentin® orally at home. The cellulitis resolved completely without sequellae.

Introduction Circumcision is the excision of the foreskin of the penis.1,2 It is one of the oldest known surgical procedures dating back to the Bible.3,4 Newborn circumcision is one of the most common surgical procedures performed.3,5-9 Most males in the United States have been circumcised.2,3,10 The Jewish faith dictates newborn circumcision; medical indications for performing circumcisions are debatable.10-16 Circumcision is thought to reduce the transmission of the HIV virus and reduce the incidence of carcinoma of the penis.4,17,18 Female circumcision is not considered in this paper; however, it not considered part of modern medicine.19 There are social reasons for performing circumcisions, such as circumcising a newborn male when there are older brothers who have already been circumcised. Interest in circumcision seems to wax and wane over time.

Who Performs Circumcisions? There is significant variation in exactly who performs circumcisions. Obstetricians have traditionally performed circumcisions as part of global obstetrical care. Today, many pediatricians have learned the technique and offer the procedure to parents of male newborns. In many large cities, pediatricians do not even go to the hospital any longer, and newborn care is a service of neonatologists who perform circumcisions. In teaching institutions, medical students and residents may perform circumcisions under the supervision of attending physicians and fellows. Pediatricians usually follow up circumcisions as part of outpatient newborn care and, along with pediatric urologists, manage complications of circumcisions. Many OB/GYN train-

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ing programs no longer teach circumcision and have delegated that responsibility to the pediatricians and urologists. Circumcisions in the Jewish faith are performed by mohels who are usually not physicians.11 In other countries, circumcisions may be performed by nurses, midwives, surgical aides, low skilled medical personnel, or traditional circumcisers.4

Complications of Circumcision There are complications of any surgical procedure, and circumcision is no exception. Fortunately, most are rare and almost always self-limited.2, 4- 6,11,12,14,17,18,20,21 Bleeding, infection, and surgical trauma are the most common complications of circumcision.1,3,4,6,9,10,11,16,18,21 Bleeding is the most common complication encountered in hospital-performed circumcisions today. The most serious complications of circumcision are infectious in origin. Trainees learning the procedure will occasionally remove too much foreskin. The risk of complication following circumcision ranges from 0 to 50.1%.2,4,6,17,22 The risk of serious complication following circumcision is about 1 in 500.9

Postoperative Infections An infected circumcision is usually diagnosed by redness and swelling of the penis and scrotum and/or pus in the wound.22 Infections following circumcision have been reported with any type of practitioner performing the procedure. The raw surface of a circumcision may serve as a site of entry for infectious organisms, even when the site appears uninfected.5 While an attempt is usually made for sterile technique in hospital and physician offices, ritual circumcisions suggest that aseptic technique may not be necessary.7 The most serious infectious complications are necrotizing fasciitis, Fournier’s Syndrome, sepsis, osteomyelitis, meningitis, and even death.8,11,20,23-25 Necrotizing fasciitis and Fournier’s Syndrome (penile gangrene) may have devastating results and require extensive surgery and debridement.26 The mortality for Fournier’s Syndrome is high.26 Several organisms have been reported to infect the circumcised penis including aerobic and anaerobic bacteria, herpes, tuberculosis, syphilis and diphtheria.5,6,9,16,22,23,27-29 Methicillin-resistant Staphylococcus Aureus is the most commonly isolated organism.21 Infectious complications may be increased in preterm or sick infants.8 Kirkpatrick and Eitzman reported cases of septicemia due to Proteus and Staphylococcus aureus in preterm infants following circumcision.8 It is a general practice in most hospitals to forego circumcision in sick and/or premature infants until they are ready for discharge. Infection following circumcision is more common with the Plastibell® device than other devices.9 Gesundheit et al reported several cases of Herpes Simplex Virus-1 infection following the traditional practice of circumcision in which the circumciser orally suctions the blood from the incised foreskin with subsequent oral-genital contact.28 Tuberculosis, syphilis, and diphtheria have also been reported to complicate circumcision by oral-genital contact of the mohel.16

Annobil et al reported a case of tuberculosis complicating circumcision following a circumcision by a “local barber” who presumably had pulmonary tuberculosis.16 According to Woodside, infected circumcisions are more serious than they appear on the surface and are usually the result of multiple aerobic and anaerobic organisms requiring broad spectrum antibiotic coverage and early debridement.27 Plastibell® circumcision effects circumcision by strangulation of tissue with subsequent necrosis and sloughing of the foreskin.25 This crush injury may increase the risk of wound infection.25 While some swelling and redness at the incision line is common, erythema should not involve the entire penis and scrotum. Erythema of the shaft of the penis should raise suspicion of infection.26 While topical antibiotic cream is routinely used after circumcision, prophylactic antibiotics have not been recommended.30

Summary Circumcision is one of the most common surgical procedures performed today. Although the risk is low, complications do occur. Most complications are minimal and self-limiting, but they can be devastating and even fatal. Because circumcisions are very common, fairly quick to perform with experience, and often done in bulk, they are often taken for granted. They are nevertheless a surgical procedure with risks requiring consent. Informed consent must be obtained from the parents by the person performing the procedure including a discussion of the risks. Daniel M. Avery, Jr., MD, is Professor and Chair of OB/GYN, University of Alabama School of Medicine, Tuscaloosa. Daniel M. Avery, III, MD, is Resident in Orthopedic Surgery, St. Luke’s Hospital and Health Network, Bethlehem, PA. John T. McDonald, Jr., MD, is Assistant Professor of OB/GYN, University of Alabama School of Medicine, Tuscaloosa. Joseph C. Wallace, MD, Associate Professor and Chair of Surgery, University of Alabama School of Medicine, Tuscaloosa. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

References 1.

Berk B, Ozgu A, Semih T, Tarkan S. Circumcision: Pros and Cons. Indian J Urol. 2010;26:12-15.

2.

Centers for Disease Control and Prevention: Male Circumcision and Risk for HIV Transmission and Other Health Conditions: Implications for the United States. 2008.

3.

Nasrallah PF. Circumcision: Pros and Cons. Primary Care. 1985; 12(4):593-605.

4.

Ahmed A. Childhood Circumcision: A Planned Approach. Tropical Doctor. 2007;37(4):239-241.

5.

Cleary TG, Kohl S. Overwhelming Infection with Group B BetaHemoltyic Streptococcus Associated with Circumcision. Pediatrics.

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1979;64(3):301-303.

19. Asuen MI. Maternal Septicaemia and Death After Circumcision. Tropical Doctor. 1977;7(4):177-178.

6.

Wiswell TE, Geschke DW. Risks From Circumcision During the First Month of Life Compared With Those for Uncircumcised Boys. Pediatrics. 1989;83(6):1011-1015.

7.

Naimer SA, Trattner A. Are Sterile Conditions Essential for All Forms of Cutaneous Surgery? The Case of Ritual Neonatal Circumcision. J Cutan Med Surg. 2000;4(4):177-180.

21. Wiswell TE, Curtis J, Dobek AS, Zierdt CH. Staphylococcus Aureus Colonization After Neonatal Circumcision in Relation to Device Used. J Pediatr. 1991;119(2):302-304.

8.

Kirkpatrick BV, Eitzman DV. Neonatal Septicemia After Circumcision. Clin Pediatr. 1974;13(9): 767-768.

22. Ahmed A, Mbibi NH, Dawam D, Kalayi GD. Complications of Traditional male Circumcision. Annals of Tropical Paediatrics. 1999;19:113-117.

9.

Gee WF, Ansell JS. Neonatal Circumcision: A Ten-Year Overview: With Comparison of the Gomco Clamp and the Plastibell Device. Pediatrics. 1976;58:824-827.

23. Stranko J, Ryan ME, Bowman AM. Impetigo in Newborn Infants Associated with a Plastic Bell Clamp Circumcision. Pediatric Infectious Disease. 1986;5(5): 597-599.

10. Wiswell TE. Circumcision Circumspection. N Engl J Med. 2010;336:12441245.

24. Uwyyed K, Vromen A. Scrotal Abscess with Bacteremia Caused by Salmonella Group D after Ritual Circumcision. Pediatric Infectious Disease Journal. 1990;9(1):65-66.

11. Menahem S. Complications Arising From Ritual Circumcision: Pathogenesis and Possible Prevention. Isr J Med Sci. 1981;17(1):45-48.

20. Preston EN. Whither the Foreskin—A Consideration of Routine Neonatal Circumcision. JAMA. 1970;213(11):1853-1858.

25. Woodside JR. Necrotizing Fasciitis After Neonatal Circumcision. Am J Dis Child. 1980;134(3):301-302.

12. Fergusson DM, Lawton JM, Shannon FT. Neonatal Circumcision and Penile Problems: An 8-Year Longitudinal Survey. Pediatrics. 1988;81(4):537-541.

26. Ngan JH, Waldhausen J, Santucci R. “I Think This Child Has an Infected Penis After Neonatal Circumcision…” Online Pediatric Urology. 1996;1-5.

13. Kinkade S, Meadows S. Does Neonatal Circumcision Decrease Morbidity? J Fam Prac. 2005;54(1):1-3.

27. Woodside JR. Circumcision Disasters. Letter to the Editor. Pediatrics. 1980;65(5):1053.

14. Christakis DA, Harvey E, Zerr DM, Feudtner C, Wright JA, Connell FA. A Trade-Off Analysis of Routine Newborn Circumcision. Abstract. Obstet Gynecol Surv. 2000;55(9):546-548.

28. Gesundheit B, Grisaru-Soen G, Greenberg D, Levtzion-Korach O, Malkin D, et al. Neonatal Genital Herpes Simplex Virus Type 1 Infection After Jewish Ritual Circumcision: Modern Medicine and Religious Tradition. Abstract. Pediatrics. 2004;114(2):e259-e263.

15. Van Howe RS. Is Neonatal Circumcision Clinically Beneficial? Argument Against Nature. Clinical Practice Urology. 2009; 6:74-75. 16. Annobil SH, al-Hilfi A, Kazi T. Primary Tuberculosis of the Penis in an Infant. Tubercle. 1990;71(3):229-230. 17. Muula AS, Prozeky HW, Mataya RH, Ikechebelu JI. Prevalence of Complications of male Circumcision in Anglophone Africa: A Systemic Review. BMC Urology. 2007;7(4):1-6. 18. American College of Obstetricians and Gynecologists: Newborn Circumcision. 2008.

29. Van Howe RS, Robson WL. The Possible Role of Circumcision in Newborn Outbreaks of Community-Associated Methicillin-Resistant Staphylococcus Aureus. Clin Pediatr. 2007;46(4):356-358. 30. WebMD: Procedures That May Require Antibiotics to Prevent Endocarditis-Topic Overview. 2010. Available at http://www.webmd. com/heart-disease/tc/procedues-that-may-require-antibiotics-to-preventendocarditis-topic. 9/11/11.

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M e d i c a l E t h i c s W i t h o u t t h e R h e t o r i c Cases presented here involve real physicians and patients. Unlike the cases in medical ethics textbooks, these cases seldom involve human cloning, bizarre treatments, or stem cell research. We emphasize cases more common to the practice of medicine. Most cases are circumstantially unique and require the viewpoints of the practitioners and patients involved. For this reason, I solicit your input on the cases discussed here at councile@aol.com. Reader perspectives along with my own viewpoint are published in the issue following each case presentation. We are also interested in cases submitted by readers. The following case addresses a potential conflict between the physician’s role as caregiver and the requirements of law.

Mark Pastin, PhD Mark Pastin, PhD, is president and CEO of the Council of Ethical Organizations, Alexandria, VA. The Council, a non-profit, non-partisan organization, is dedicated to promoting ethical and legal conduct in business, government, and the professions.

cas e eleven H a n d s O f f , D o c !

You are on duty in an area of the hospital where there are many elderly patients. While you are caring for one patient in a shared room, you hear warning beeps from a monitoring device for the other patient in the room. While the other patient has a different attending physician, you look to see if the situation is serious and conclude that an immediate intervention is needed. When you approach the patient, the patient mutters, “I don’t want one of you touching me.” You are from the Middle East and assume that the patient is referring to this fact in saying “one of you.” In the state in which you are practicing, it is considered assault to touch a patient against the patient’s wishes. You doubt that you can get another physician to the scene in time to save the patient’s life. Should you intervene? This is an actual case. Of course, there are any number of complicating circumstances and additional details; but please address the case on the basis of the information provided. There will be an analysis of this case and a new case in the next issue.

Your input is requested. Email your responses to: councile@aol.com. © Copyright Council of Ethical Organizations 2012

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M e d i c a l E t h i c s W i t h o u t t h e R h e t o r i c CASE TEN ANALY SIS T i mmy t h e T o r c h Our case from the last issue involves Timmy, a teenager with a growing propensity to light things, including living things, on fire. Timmy is a “torch.” Timmy’s parents have asked their physician to admit Timmy for a couple of days of observation, mainly to relieve the strain his behavior has put on the family. The physician would not admit Timmy otherwise but can see that the parents are at their limit. The majority of readers felt that Timmy should not be admitted since the admission is not medically necessary for Timmy. A minority felt that giving the parents a break would enable them to better work with Timmy’s problems. In this case, I agree with the minority. When treating behavioral issues, the family system needs to be considered, and that system is under immense pressure in this case. However, even if you agree with the minority, as I do, the problem of who will pay for the admission is problematic. Since the admission is not medically necessary for Timmy, few health plans would cover it. If the parents pay out of pocket, this too could become a source of strain on the family.

Reflections r e f l e c t i o n s This is the eleventh case discussed in this space. My hope that the cases would provoke lively discussion has been more than satisfied. In the process, I have learned some lessons about the physician members of the AAPS. The most important lesson is that these physicians are strongly inclined to judge even the most difficult cases in terms of the best interests of patients. I have also learned that a good medical education does not make difficult ethical situations any easier to navigate. Perhaps these observations are predictable. Less predicable is the inclination of some readers to take the cost of care and even payment considerations into account. The idea that medical ethics means taking the patient’s interests as paramount is losing some of its grip on the profession. Some readers are considering not only the patient’s interests but a more general public good consisting of the perceived best use of medical resources. While this is exactly what today’s policy makers want physicians to do, I am concerned that we have not reflected sufficiently on just what this public good consists in - and if we are in a position to assess it. When I tell outsiders about writing these cases, they often doubt that physicians care much about medical ethics. I can certainly tell these folks how wrong they are – and how much effort today’s physicians put into understanding and doing the right thing.

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Adenocarcinoma Arising in a Three Millimeter-Thick Endometrium Daniel M. Avery, Jr., MD Daniel M. Avery, III, MD Marion D. Reed, MD Joseph C. Wallace, MD

Numerous papers have recommended nonintervention for postmenopausal bleeding when the endometrial thickness on transvaginal ultrasound is four millimeters or less because the reported risk of endometrial cancer is very low. A case of adenocarcinoma arising in a three millimeter thick endometrium is presented. Occasionally, clinical judgment and suspicion are important in caring for the patient despite recommended guidelines and protocols.

Case Report A 60-year-old white female gravida 4 para 4004 postmenopausal for five years presented with bleeding of 10 months duration. Past medical history was significant for chronic hypertension and obesity. Physical examination revealed an obese, wellnourished white female in no apparent distress, measuring 5’ 7” in height and weighing 210 pounds. The vagina was lined with old blood. The cervix was normal. The uterus was normal size and mobile without any adnexal masses. Pelvic ultrasound showed a uterus measuring 7.6 by 4.7 by 3.8 cm with an endometrial thickness of 3 mm. Office endometrial biopsy was grossly bloody and insufficient. Dilatation and curettage under hysteroscopic guidance was performed in the operating room with pathological findings, “well to moderately differentiated endometrial adenocarcinoma.” She was referred to a gynecologic oncologist for staging and further treatment. A robotic total laparoscopic hysterectomy with bilateral salpingo-oophorectomy and pelvic and periaortic lymph node dissection were performed without complication.

Discussion Transvaginal sonography is a non-invasive tool used to evaluate the endometrium in women with postmenopausal bleeding to assess the risk of malignancy.1-22 Adenocarcinoma of the endometrium is the most common gynecologic malignancy1,3,16,19 and the fourth most common malignancy overall in women.3 There are more than 41,000 cases of endometrial adenocarcinoma annually resulting in more than 8,100 deaths.1 Postmenopausal bleeding is the most common presenting symptom and is “endometrial cancer until proven otherwise.”1,19 Most women with adenocarcinoma of the endometrium present with vaginal bleeding.1,9,16 Postmenopausal bleeding is defined as resumption of bleeding after 12 months of amenorrhea. Postmenopausal bleeding necessitates a workup including evaluation of the endometrium.1 Most cases of postmenopausal bleeding are from endometrial atrophy,1 and up to 14% of these women with bleeding have cancer.1,2,13,19 Transvaginal sonography has been used to evaluate the endometrium for about 20 years and has become an acceptable means of assessing the risk of malignancy.1-5,9,13-22 The endometrium may act as a biomarker for estrogen stimulation, since estrogen makes the endometrium thicker.14 Studies have shown that endometrial thickness 4 mm or less on transvaginal sonography in women with postmenopausal bleeding have a low risk of endometrial cancer of about 1 in 1000.1,3,11,15,22 Therefore, it has been suggested that these women do not need an endometrial biopsy or dilatation and curettage.1 The normal postmenopausal endometrium is usually 0.5 mm in thickness and rarely more than 3 mm.6 As the thickness of the endometrium

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increases, the risk of developing adenocarcinoma increases.2,16 Reports of mean thicknesses of endometrial carcinomas range from 15.2 mm to 20 mm.1,3,4 In endometria in which measurement of thickness cannot be obtained, there is a high risk of cancer.9 In postmenopausal patients without bleeding, endometrial thickness by transvaginal sonography greater than 11 mm should prompt tissue sampling, but not in less than 11 mm thickness without bleeding.10 There are reports of endometrial cancer arising in endometria that are less than 5 mm in thickness.5 In an Italian study, there were two cases of endometrial cancer in women who had an endometrial thickness less than 4 mm.5 Gull et al reported a single case of endometrial cancer in a woman with postmenopausal bleeding whose endometrium was less than 4 mm.13 In a select group of Jamaican females, 50% of the cases of endometrial cancer was present in endometria 3-4 mm in thickness.15

Summary Postmenopausal bleeding needs some type of assessment of the endometrium.1 Transvaginal sonography is a non-invasive tool used to evaluate the endometria in women with postmenopausal bleeding to assess the risk of malignancy.1 Most cases of endometrial cancer arise in endometria that are greater than 5 mm in thickness. There are isolated reports of endometrial cancer arising in endometria that are less than 5 mm in thickness. This is an important case that fell outside the recommended guidelines. Sometimes clinical judgment trumps guidelines and protocols for effective patient care and follow up. Daniel M. Avery, Jr., MD, is Professor and Chair of OB/GYN, University of Alabama School of Medicine, Tuscaloosa. Daniel M. Avery, III, MD, is a Resident in Orthopedic Surgery, St. Luke’s Hospital and Health Network, Bethlehem, PA. Marion D. Reed, MD, is Assistant Professor of OB/GYN, University of Alabama School of Medicine, Tuscaloosa. Joseph C. Wallace, MD, is Associate Professor and Chair of Surgery, University of Alabama School of Medicine, Tuscaloosa. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

References 1.

Goldstein SR. The Role of Transvaginal Ultrasound or Endometrial Biopsy in the Evaluation of the Menopausal Endometrium. Am J Obstet Gynecol. 2009;July:5-11.

2.

Williams SC, Lopez C, Yoong A, McHugo JM. Developing a Robust and Efficient Pathway for the Referral and Investigation of Women with PostMenopausal Bleeding Using a Cut-Off of <4 mm for a Normal Thickness. British Journal of Radiology. 2007;80:719-723.

3.

Canavan TP, Doshi NR. Endometrial Cancer. American Family Physician. 1999;59(11):1-10.

4.

Symonds I. Ultrasound, Hysteroscopy and Endometrial Biopsy in the

Investigation of Endometrial Cancer. Best Practice & Research Clinical Obstetrics and Gynaecology. 2001;15(3):381-391. 5.

Ferrazzi E, Trio V, Zannoni E, Filiberto S, Dordoni D et al. Sonographic Endometrial Thickness: A Useful Test to Predict Atrophy in Patients with Postmenopausal Bleeding. An Italian Multicenter Study. Ultrasound Obstet Gynecol. 1996;7:315-321.

6.

Osmers RGW, Kuhn W. Endometrial Cancer Screening. Current Opinion in Obstetrics and Gynecology. 1994;6:75-79.

7.

Brockbank EC, Ghaem-Maghami S, Bridges JE. The Gynecological Management of Women on Tamoxifen: A National Questionnaire Study. Journal of Obstetrics and Gynaecology. 2004;24(6):675-679.

8.

Ayodele OE, Bello TO, Odewale MA, Efuntoye AT. Endometrial Thickness in Asymptomatic Postmenopausal Nigerian Women with and Without Hypertension. Int J Gynecol and Obstet. 2006;92:165-166.

9.

Epstein E, Valentin L. Managing Women with Post-Menopausal Bleeding. Best Practices & Research Clinical Obstetrics and Gynaecology. 2004;18(1):125-143.

10. Smith-Bindman R, Weiss E, Feldstein V. How Thick is Too Thick? When Endometrial Thickness Should Prompt Biopsy in Postmenopausal Women Without Vaginal Bleeding. Ultrasound Obstet Gynecol. 2004;24:558-565. 11. Rozenberg S, Auvertin S, Ham H. A Survey of Physicians’ Attitude Towards Women with Postmenopausal Bleeding. Maturitas. 2001;39:189-193. 12. Van den Bosch T, Vandendael A, Van Schoubroeck D, Lombard CJ, Wranz PAB. Age, Weight, Body Mass Index and Endometrial Thickness in Postmenopausal Women. Acta Obstet Gynecol Scand. 1996;75:181-182. 13. Gull B, Karisson B, Milsom I, Granberg S. Can Ultrasound Replace Dilation and Curettage? A Longitudinal Evaluation of Postmenopausal Bleeding and Transvaginal Sonographic Measurement of the Endometrium as Predictors of Endometrial Cancer. Am J Obstet Gynecol. 2003;188(2):401-8. 14. Sit ASY, Modugno F, Hill LM, Martin J, Weissfeld JL. Transvaginal Ultrasound Measurement of Endometrial Thickness as a Biomarker for Estrogen Exposure. Cancer Epidemiol Biomarkers Prev. 2004:13(9):145965. 15. Phillip H, Dacosta V, Fletcher H, Kulkarni S, Reid M. Correlation Between Transvaginal Ultrasound Measured Endometrial Thickness and Histopathological Findings in Afro-Caribbean Jamaican Women with Postmenopausal Bleeding. J Obstet and Gynaecology. 2004;24(5):568-72. 16. Schmidt T, Breidenbach M, Nawroth F, Mallmann P, Beyer IM, Fleish MC, Rein DT. Hysteroscopy for Asymptomatic Postmenopausal Women with Sonographically Thickened Endometrium. Maturitas. 2009;62:176-178. 17. Domingues AP, Lopes H, Dias I, De Oliveira CF. Endometrial Polyps in Postmenopausal Women. Acta Obstetricia et Gynecologica. 2009;88(5):618-20. 18. Ozsener S, Ozsaran A, Itil I, Dikmen Y. Endometrial Pathology of 104 Postmenopausal Breast Cancer Patients Treated with Tamoxifen. Eur J Gynaec Oncol. 1998;19(6):580-3. 19. Nutis M, Garcia KM, Nuwayhid B, Mulla Z, ElMasri W. Use of Ultrasonographic Cut point for Diagnosing Endometrial Pathology in Postmenopausal Women with Multiple Risk Factors for Endometrial Cancer. J Reprod Med. 2008;53:755-59. 20. Pansini F, De Paoli D, Serra MM, Campobasso C, Levato F, Giulini D. Combined Use of Progesterone Challenge Test and Endometrium Thickness Evaluated by Transvaginal Ultrasonography in the Preventive Management of Postmenopausal Women. Gynecol Obstet Invest. 1992;34:237-239. 21. Al-Kadri HM, Al-Awami, Madkhali AM. Assessment of Risk Factors of Uterine Cancer in Saudi Patients with Postmenopausal Bleeding. Saudi Med J. 2004;25(7):857-861. 22. Opolskiene G, Sladkevicius P, Valentin L. Ultrasound Assessment of Endometrial Morphology and Vascularity to Predict Endometrial Malignancy in Women with Postmenopausal Bleeding and Sonographic Endometrial Thickness > 4.5mm. Ultrasound Obstet Gynecol. 2007; 30:332-340.

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Case Report Fetal Heart Rate Misrepresented by Maternal Heart Rate: A Case of Signal Ambiguity Ikechukwu Emereuwaonu, MD

Abstract Interpretation of Electronic Fetal Heart Rate (EFHR) patterns, as the major component of the non stress test (NST) and contraction stress test(CST), is a required skill for prenatal and delivery care. However, there are situations where the fetal heart rate tracing may be misleading.1 Because the decision to perform emergency cesarean section frequently hinges on (EFHR) monitoring, it is important to recognize situations where EFHR monitoring could be misleading. This report describes a case in which the maternal heart rate was detected by the EFHR monitor and mistaken for a reas-

suring (category I)6 fetal heart rate tracing during second stage of labor. The mistake was discovered immediately postpartum when the “reassuring fetal strip” continued on the monitor even though the neonate had delivered, indicating that the tracing was actually of maternal origin. This highlights the concept of signal ambiguity.1 Signal ambiguity is a situation in which the EFHR mistakenly detects and displays the maternal pulse on the fetal heart rate monitor. This case report shows how this can occur, when it should be suspected, and suggests methods for evaluation, confirmation, and correction. This helps physicians to intervene appropriately or avoid significant interventions based on misleading readings.

Figure 1A: The FHR began to decrease from 120s to 90s.

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Figure 1B: The FHR began to decrease from 120s to 90s.

Figure 2A: Maternal pulse tracing as fetal heart rate.

Case report – Signal Ambiguity A 19-year old Gravida 1 Para 0 had her initial prenatal visit in the first trimester. There were 17 prenatal visits. Her estimated due date (EDD) was established by first trimester ultrasound. Prenatally there were no complications. She presented at 39 weeks and 4 days with ruptured membranes and proceeded to active labor. Initially the cervix was 3cm dilated and 90% effaced. The fetal head was at -2 cm station, and the presentation was vertex. She was contracting every four minutes in a regular and painful pattern. She was admitted to the hospital in anticipation of delivery. During electronic fetal monitoring (EFM) fetal heart rate (FHR) ranged from 110 to 130s with an isolated peak to 150s.

At about seven hours after admission, patient was sat up for epidural placement, and the nurse noted that the FHR appeared to decelerate to the 90-100s and stayed down for seven minutes. During this time the nurse anesthetist was able to access the epidural space. Once the epidural catheter was in, the patient was turned on her side. An attempt to stimulate the fetal scalp and place an fetal scalp electrode (FSE) failed. Backup for a possible cesarean section was notified. The maternal pulse (read by the pulse oximeter) approximated the FHR by one to two beats. A review of the FHR tracing found subtle accelerations with each contraction. The maternal pulse tracing on the cardiograph was started. As noted in

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Figure 2B: Maternal pulse tracing as fetal heart rate.

the Figure 2A, the maternal pulse traced together with the fetal heart rate. To determine the location of the FHR, a bedside ultrasound was used to locate the fetal heart (see Diagram I). The fetal heart was located just above the pubis and right of midline. The Doppler sensor had been placed further superior. We repositioned the sensor and the FHR tracing was at 120s and the MHR tracing at 80-90s. Diagram I: Fetal heart location by Ultrasound.

Discussion Despite years of controversy, electronic fetal heart rate monitoring (EFHRM) is the standard of care for evaluation of fetal status during pregnancy and labor.4, 5 It is the sine qua non of prenatal risk management non stress testing (NST), biophysical profile (BPP), and contraction stress testing (CST). EFHRM is relied upon heavily to make the decision for emergent delivery by cesarean section. However, this technology is not 100% reliable for reasons such as signal ambiguity, as exemplified by the aforementioned case. Signal ambiguity describes the phenomenon whereby the EFHRM mistakenly detects the maternal heart rate instead of the fetal heart rate. This error may arise from faulty Doppler placement or inability of the cardiotocograph to differentiate FHR from MHR, or both. This phenomenon is not widely known and has been responsible for poor fetal outcomes in the face of seemingly excellent category I tracings.1

Doppler Sensor Location

Using the above case as an example, this discussion presents a way to suspect, evaluate, diagnose, and correct any case of signal ambiguity (in singleton pregnancies). This is very important as interventions in labor management are affected by EFHRM. In the above case a cesarean section would have been initiated for non reassuring fetal heart rate tracing (NRFHR) – fetal bradycardia4,5,6 had we not recognized that this was a case of signal ambiguity.

Location of Fetal heart by Ultrasound

The labor continued uneventful and MHR tracing was turned off. Eventually, the patient became 10cm dilated and started pushing as seen in figure 5A- C. After an hour-long second stage of labor, a live infant male was born with Apgars 9/9. However, as is demonstrated in Figure 6, the EFHR monitor continued to detect a fetal heart tracing in the postpartum period, raising the suspicion of signal ambiguity.

When to suspect signal ambiguity The key to recognizing signal ambiguity is to suspect it when the: 1. FHR is running in the low normal range;3 2. FHR accelerations4,5 (sometimes slight with absence of decelerations) are noted with >50% of contraction (especially when pushing);3

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Figure 4A: Fetal heart rate and maternal pulse tracing seen differently.

MHR

FHR

We repositioned the sensor and the FHR tracing was at 120s and the MHR tracing at 80-90s.

Figure 4B: Fetal heart rate and maternal pulse rate tracing as separate entities.

FHR

MHR

Figure 4C: Fetal heart rate and maternal pulse rate tracing as separate entities.

FHR

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MHR

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Figure 5A: Fetal heart rate tracing during pushing.

The labor continued uneventful and MHR tracing was turned off. Eventually, the patient became 10cm dilated and started pushing as seen in figure 5A- C.

Figure 5B: Fetal heart rate tracing during pushing.

Figure 5C: Fetal heart rate tracing during pushing.

After an hour-long second stage of labor, a live infant male was born with Apgars 9/9. However, as is demonstrated in Figure 6, the EFHR monitor continued to detect a fetal heart tracing in the postpartum period, raising the suspicion of signal ambiguity.

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Figure 6: Fetal heart tracing continues three minutes postpartum.

3. Apparent FHR deceleration to MHR (or maternal tachycardia into FHR) range that does not recover.3 There MAY be a signal break between the switch from fetal to maternal heart rate,1 as visualized by a distinct discontinuity in the heart tracing. Refer to Figures 1A1B/5A5B/5C/6.

How to evaluate a suspected case of signal ambiguity Once suspected, signal ambiguity can be evaluated by assessing maternal pulse and comparing it with FHR. Assess pulse by: 1. Counting maternal radial pulse for one minute; 2. Using a pulse oximeter to record a maternal nailbed pulse; 3. Start a MHR pulse oximeter tracing on cardiotocograph and watch rate and pattern (recommended option). If the external FHR and maternal pulse are the same, or closely approximate each other by one to four beats per minute, then signal ambiguity is likely present. Some hospitals already place a pulse oximeter on laboring patients as part of the protocol for epidural anesthesia. Refer to Figures 2A2B .

How to correct a confirmed case of signal ambiguity Find the fetal heart rate by: 1. Using a bedside Ultrasound Sound to locate fetal heart (recommended option because intrauterine fetal death (IUFD) is a possibility); 2. Relocate the Doppler sensor until another heart rate (i.e., the FHR) is located that is at least five to ten beats different from MHR; 3. Alternatively, place a fetal scalp electrode (FSE) if possible. Restart FHR and MHR tracings and watch rate and patterns. The difference should be at least five to ten beats. The MHR

tracing may have subtle acceleration with each (or almost each) contraction or pushing effort. However, the FHR would accelerate randomly or perhaps even decelerate with contraction.3 Refer to Figure 4A14B/4C.

Acknowledgement I wish to acknowledge Omoniyi Y. Adebisi, MD, CCFP, DABFP, DABPSFO, Surgical Family Medicine Obstetrics for his editing assistance in the preparation of this manuscript. Ikechukwu Emereuwaonu, MD, is Assistant Program Director, St. Joseph Family Medicine Residency, St. Joseph Hospital Medical Center, Phoenix. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

References 1.

Neilson DR, Freeman RK, Mangan S. Signal Ambiguity resulting in unexpected outcome with external fetal heart rate monitoring. American Journal of Obstetrics & Gynecology. 2008 June;198(6):717-24. Presented at Vanderbilt High risk OB conference.

2.

Nageotte MP. Avoiding 5 common mistakes in fetal heart rate monitoring. Contemporary Obstetrics and Gynecology. 2007;52:50-5.

3.

Sherman DJ, Frenkel E, Kurzweil Y, Padua A, Arieli S, Bahar M. Characteristics of maternal heart rate patterns during labor and delivery. Obstetrics and Gynecology. 2002;99:542-7.

4.

Rodney JRM, Huntley BJF, Rodney WM. Electronic fetal monitoring Update 2012. Family Medicine Obstetrics Clin. N. America. 2012. In Press.

5.

American Academy of Family Physicians (AAFP). Advanced Life Support Obstetrics (ALSO). Chapter – Intrapaturm Fetal Surveillance. Kansas City, Mo. 2008.

6.

Macones GA, Hankins GD, Spongy CY, Hauth J, Moore T. NICHD updates guidelines and 3 tier system. Obstetrics and Gynecology. 2008 Sept;112(3):661-6.

Fetal Heart Rate Misrepresented by Maternal Heart Rate . . .

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Orthopedic Issues in Family & Emergency Medicine

Managing Common Fractures: The Heel Michael Jerkins, Medical Student Ravi Kumar Singh, MD

Introduction Physicians need skills in the diagnosis and management of commonly occurring injuries. In many places, subspecialist help is not available or affordable.1 These basic skills reflect the reality of community medical practice for a variety of specialties staffing rural emergency rooms, primary care offices, and mission hospitals. For younger physicians, most academic medical centers teach a curriculum of “Refer to Ortho.” Hatch et al. have described the epidemiology of common fractures in the community.2 Many training programs have no incentive to install and maintain basic imaging equipment. Patients with possible fractures are triaged to the hospital before they receive any evaluation at the point of initial service. This is a lost opportunity for the development of these skills.

fence onto the field. He fails to notice that the stadium floor is an additional six feet below and lands on both feet. Upon impact, he experiences a sharp pain in his left heel. With great pain and a limp, he walks across the field, evaluates the player, and returns to his seat. The next morning he is in great pain, has difficulty putting his shoe on, and comes to the office for an x-ray. Prior to the x-ray, his past medical history is reviewed and found to be unremarkable. Upon examination, there is point tenderness on the lateral posterior edge of the foot. Swelling is difficult to assess, but the foot appears normal. There is a subcutaneous bruise along the lateral posterior edge of the foot. An x-ray is taken. The image is available for review.

Most injuries require screening with imaging. When detected, most fractures can be managed conservatively while maintaining quality in the community. The trick is to find the fracture. Then physicians can start to develop the knowledge of selecting those cases that require surgery and conservatively managing the others.3-6 This series is dedicated to those physicians serving in areas where resources are scarce and the hours are long.

Case 1 A 54-year old male physician is observing a high school football game when he is called down to the field to evaluate a potential dislocated shoulder. In his enthusiasm he vaults the three-foot Orthopedic Issues in Family & Emergency Medicine


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1. Which statement best describes what the x-ray reveals? A. There is a metatarsal fracture. B. There is a phalangeal fracture. C. There is a calcaneal fracture. D. There is an ankle fracture. 2. At this point, the best advice would be: A. Non-weight bearing with crutches for one to two days is advised. B. Immediate weight bearing will promote healing. C. Splinting would be helpful.

D. Bone scan to evaluate a stress fracture is indicated. 5. The patient asks how long he can expect to have the pain. Although he can work, he would like to take his son hunting, which requires considerable ambulation over rough terrain. You advise him: A. The pain, if no further injury, will be gone in a week. B. Chronic pain can be expected at the area of injury. C. He should be fit for hiking in three to five days. D. The pain, if no further injury, will gradually diminish over four to six weeks.

D. A cast is indicated. 3. Regarding pain management in this case, which one of the following is most likely to manage the pain of this injury (check all that apply): A. Over-the-counter analgesics as needed.

Case 2 A twenty-one year old gymnast lands on her right heel after a forceful dismount. She has a limp and heel pain. An image of the foot is obtained.

B. Tylenol 650 mg with Benadryl 25 mg every six hours. C. Hydrocodone 5mg with acetaminophen 500mg every eight hours. D. Demerol 100mg IM now. The patient returns two weeks later for a follow-up visit. He has not taken any time off from work. He continues to have substantial but not disabling pain in the heel. He walks with a limp, and there is tenderness to palpation on the lateral posterior heel. A second radiographic image is obtained.

6. You should conclude: A. The patient has a fracture of the heel. B. Comparison views of both feet are not automatically necessary. C. Comparison views are distracting, and a new film should be taken. D. A bone scan to evaluate a stress fracture is indicated. 4. At this point you conclude:

7. Management advice should include:

A. Callus formation is clearly visible at this point.

A. Non-weight bearing crutches for two weeks.

B. The patient has a fracture without visible x-ray change over two weeks.

B. Return to gymnastic workouts tomorrow.

C. CT or MRI would be helpful to make a more accurate diagnosis.

C. Weight bearing as personal tolerance allows. D. Immediate weight bearing may lead to disability.

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Answers and Explanations

References

1. The only observable fracture on this radiograph is mid-calcaneus. This fracture would fit in the category of extra-articular calcaneus fracture, which makes up about 30% of all calcaneus fractures.7 It is important to make sure that the fracture is not in one of the articular surfaces of the bone, because it typically has more complex management and poorer prognosis.8 Answer: C

1.

Rodney WM. Foreword in: Pfenninger JL, Fowler GC. Procedures for Primary Care Physicians. St. Louis: Mosby; 1994. New edition 2002.

2.

Hatch RL, Rosenbaum CI. Fracture care by family physicians: A review of 295 cases. J Fam Pract. 1994;38:238-244.

3.

Warren JS, Lara K, Hahn RG. Correlation of emergency department radiographs: results of a quality assurance review in an urban community hospital. J Am Board Fam Pract. 1993;6:255-9.

4.

Halvorsen JG, Kunian A, Gjerdingen D, et al. The interpretation of office radiographs by family physicians. J Fam Pract. 1989;28:426-432.

2. This type of calcaneus fracture (extra-articular) has a good prognosis.9 Patients can temporarily ice and elevate the injured foot, and some may be able to bear weight within a few days after injury.8,10 However, immediate full weight-bearing or strenuous activity is not advised. Answer: A

5.

Simon HK, Khan NS, Nordenberg DF, Wright JA. Pediatric emergency physician interpretation of plain radiographs: Is routine review by a radiologist necessary and cost-effective? Ann Emerg Med. 1996;27:295298.

6.

Smith P, Temte J, Beasley J, Mundt M. Radiographs in the Office: is a second reading always needed? J Am Board Fam Prac. 2004;17:256-263.

7.

Kumar R, Matasar K, Stansberry S, et al. The calcaneus: normal and abnormal. Radiographics. 1991 May;11(3):415-40.

8.

Rammelt S, Zwipp H. Calcaneus fractures: facts, controversies and recent developments. Injury. 2004 May;35(5):443-61.

9.

Daftary A, Haims AH, Baumgaertner MR. Fractures of the calcaneus: a review with emphasis on CT. Radiographics. 2005 Sep-Oct;25(5):1215-26.

3. Simple anti-inflammatory medications are unlikely to be sufficient for this pain. Answer: C 4. Again, the patient’s mid-calcaneal fracture is visible on this radiographic image. It is common to see a therapeutic lag in time before the appearance of callus healing over a fracture. In this case the patient is dramatically improved from a clinical point of view, but the radiograph appears unchanged. Answer: B

10. Carl E. Horn. Fractures of the Calcaneus—Diagnosis and Treatment. Calif Med. 1968 March;108(3):209-215. 11. F. G. Day. Fractures of the Os Calcis. Can Med Assoc J. 1950 October;63(4):373–376.

5. Each individual heals at his/her own pace. Age, personality characteristics, environment, and co-morbidities – all enter into the equation. Generally patients gradually perceive insignificant pain by six to eight weeks. Answer: D 6. Taking comparative views of the foot can be helpful in accurately diagnosing the patient. In this case the patient has a minor bone spur on the inferior surface of one calcaneus. Although incorrectly presumed to be a source of pain, these bone spurs are almost always an incidental finding. Answer: B 7. Conservative treatment is appropriate. It should be noted that a conservative strategy has been proven effective for the last 50 years in extra-articular calcaneus fractures. Although returning to strenuous activity immediately is not advised, many patients can tolerate bearing weight on the injured foot within a few days of injury. 9,11 Answer: C Ravi Kumar Singh, MD, is Clinical Assistant Professor Family Medicine, Primary Care Specialists, Memphis. Michael Jerkins, is a medical student at the University of Tennessee School of Medicine, Memphis. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The authors have stated that no such relationships exist. ®

Orthopedic Issues in Family & Emergency Medicine


American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

Case Report Unusual Presentation of Aggressive Neuroendocrine Tumor Brian W. Harris, PhD, MD, FAAR Jordan Gularek, DO

Abstract

Case Report

Neuroendocrine tumors (NETs) are rare neoplasms that occur in 1 out of 100,000 people in the United States and can occur in any body tissue, although most commonly located in the lung and small intestine. They arise from various neuroendocrine cells and, while many are benign, malignant cases are reported. While NETs are known to excessively produce hormones, 50% of NETs are non-functional in nature and clinically silent.1,2 This feature can allow the tumor to grow to massive sizes or metastasize to other organs, making early recognition and treatment difficult and dramatically changing prognosis. It is reported that 40-93% of gastrointestinal NETs metastasize to the liver.1

A 47-year-old Caucasian gentleman, with a past medical history significant for seizure disorder, presented to the emergency department with intractable seizures. He described getting a strange feeling in his head prior to the seizures. The patient was unable to recall what happened after the episode. He denied any epigastric discomfort, weight loss, dysphasia, nausea, emesis, constipation, or diarrhea.

The following report is a rare case of a massive metastatic liver neuroendocrine tumor found incidentally in a 47-year-old gentleman who presented to the emergency department complaining of seizures. The pathophysiology, etiology, and treatment options for metastatic liver tumor from NETs will be discussed.

On physical examination, the patient’s vital signs were stable. Neurological examination was benign. All other systems were within normal limits except for his abdominal examination. His abdomen was firm and distended. Organomegaly was appreciated with the liver edge to be estimated 8-10 cm below the costal margin. At that time, ultrasound of the abdomen was obtained, which revealed hepatomegaly with diffusely inhomogeneous echotexture and splenomegaly. Figure 1: Axial view demonstrating hepatomegaly with multiple lesions.

Introduction Neuroendocrine tumors (NETs) are uncommon groups of heterogeneous neoplasms that occur in roughly around 1 out of 100,000 people in the United States.1,2 While NETs are uncommon in our population, metastatic disease to the liver is very common, ranging from 40-93% of the gastrointestinal NETs and is a major cause of morbidity related to these neoplasms. While NETs can occur in a wide variety of organ systems, they most commonly occur in the bronchopulmonary and gastrointestinal tract.1,5 Case-Report: Unusual Presentation of Aggressive Neuroendocrine Tumor

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Subsequently, CT scan of the abdomen and pelvis with oral and intravenous contrast was obtained for further evaluation. The CT scan demonstrated massive hepatomegaly spanning the abdomen from the left flank wall to the right flank wall over a distance of 27 cm (Figure 1). The liver also extended from the dome of the diaphragm into the right hemipelvis measuring 36 cm in length (Figure 2). The appearance of the liver was suggestive of extensive metastatic disease. There was also a large mass extending towards the tail of the pancreas (Figure 3). Figure 2: Coronal reformatted image demonstrating the liver occupying majority of the abdominal space.

Subsequently, a core needle biopsy of the liver was obtained, which demonstrated a nested and organoid growth pattern comprised of relatively uniform tumor cells. Mild nuclear pleomorphism was present. No mitoses were identified. No necrosis was noted. Furthermore, immunohistochemical stains were performed to characterize the tumor cells. The results showed positivity for chromogranin, synaptophysin, and Neuron-specific enolase (NSE) of the tumor cells. The thyroid transcription factor 1 (TTF-1) stain was negative. A diagnosis of neuroendocrine carcinoma grade I was made at this time.

Discussion Neuroendocrine tumors (NETs) are rare groups of neoplasms that can commonly metastasize to the liver.1,2,4 While NETs have the ability to secrete polypeptides with hormonal activity (functional), roughly around 50% of NETs can be categorized as non-functional in nature.1 Examples of functional NETs are Carcinoid, VIPoma, Insulinoma, Prolactinoma, Pheochromocytoma, and Gastrinoma. These NETs usually present clinically based on which hormone they secrete excessively. The concern with non-functional NETs is that they can be clinically silent until they reach a large size.1 It is estimated that the median survival duration for NETs that metastasize to the liver is 72 months.5 While treatment for NETs is specific for their location, systemic symptoms, and type, the following will explain the treatment options for NETs that metastasize to the liver.

Figure 3: This parasagittal view suggests site of origin to be pancreatic with caudal displacement secondary to massive hepatic enlargement.

Currently, the gold standard for treatment for liver metastases from NETs is resection. Surgical resection achieves a survival rate of 60-80% at five years out with a mortality of 0-5%.1,2 While resection is the gold standard, there are other modalities that are used for palliative treatment. Radiofrequency ablation (RFA) is a modality for a certain select group of patients. RFA can be considered for the following conditions: lesions less than 3 cm, palliation of carcinoid symptoms, patients medically unsuitable for surgical resection, and/or management for recurrent lesions after primary resection.2 Another modality for palliative treatment for unresectable metastatic liver lesions is embolization. This technique acts by targeting the tumor’s blood supply. While the majority of blood supply to the liver is made up by the portal vein, NET’s primary blood supply is via the hepatic artery. This modality shows a 50-96% response rate in reduction in size of lesions.2 This technique can also be used to subselectively direct medication towards the lesion. There are recent studies showing the use of miriplatin-lipiodol emulsion for chemoembolization. The results showed a significant reduction in size of the liver lesions.3 Another case from 2004 showed a considerable reduction in size of liver lesions from a patient receiving chemoembolization with four cycles of peptide radioreceptor therapy with 111Luotcreotate. This case also demonstrated a significant reduction of Chromogranin A serum markers.1 Most recently, a study was performed using doxorubicin-impregnated drug-eluting bead as chemoembolization. This study, which was performed on 18 patients, showed similar reduction in progression of disease as comparable to previous chemoembolization studies.5

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American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

Liver transplantation is another form of treatment that is only considered in a select few. Patients considered for transplant should have no extrahepatic disease with controlled primary tumor and meet the Milan criteria. Transplantation shows a 4796% five-year survival rate. Five-year recurrence rate was also found to be 24-80%.1,2 In conclusion, treatment options for NETs with hepatic metastases have increased in recent years. While liver resection is currently the gold standard of treatment, other modalities are currently available for palliative treatment. In the case of this patient, during his further evaluation by oncology his disease progressed rapidly, and he succumbed prior to initiation of appropriate therapy. Brian Harris, PhD, MD, FAAR, is Diagnostic Radiologist at Quantum Imaging and Therapeutic Associates, Inc., in Harrisburg, PA. Brian W. Harris, PhD, MD, FAAR, is Diagnostic Radiologist at Quantum Imaging and Therapeutic Associates, Inc., in Harrisburg, PA.

References 1.

de Herder WW, Mazzaferro V, Tavecchio L, Wiedenmann B. Multidisciplinary approach for the treatment of neuroendocrine tumors. Tumori. 2010 Sep-Oct;96(5):833-46.

2.

Gamblin TC, Christians K, Pappas SG. Radiofrequency ablation of neuroendocrine hepatic metastasis. Surg Oncol Clin N Am. 2011 Apr;20(2):273-9.

3.

Iwazawa J, Ohue S, Yasumasa K, Mitani T. Transarterial chemoembolization with miriplatin-lipiodol emulsion for neuroendocrine metastases of the liver. World J Radiol. 2010 Dec 28;2(12):468-71.

4.

Clayton AC, Wasserman PG, Souers RJ, Chmara BA, Renshaw A, Wilbur DC, Moriarty AT. The multiple faces of carcinoid tumor: performance characteristics of low-grade neuroendocrine carcinoma metastatic to the liver in an educational interlaboratory slide comparison program. Arch Pathol Lab Med. 2011 Mar;135(3):354-60.

5.

Gaur SK, Friese JL, Sadow CA, Ayyagari R, Binkert CA, Schenker MP, Kulke M, Baum R. Hepatic arterial Chemoembolization Using DrugEluting Beads in Gastrointestinal Neuroendocrine Tumor Metastatic to the Liver. Cardiovasc Intervent Radiol. 2011 Mar 24.

Jordan Gularek, DO, is Chief Internal Medicine Resident at Pinnacle Health System in Harrisburg, PA. Potential Financial Conflicts of Interest: By AJCM policy, all authors are required to disclose any and all commercial, financial, and other relationships in any way related to the subject of this article that might create any potential conflict of interest. The author has stated that no such relationships exist. ®

F O R T O D AY ’ S H O S P I TA L I S T

SM

For Further Information Please Contact: American Board of Hospital Medicine 5550 West Executive Drive • Suite 400 Tampa, Florida 33609-1035 (813) 433-2277 www.abhmus.org

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Manuscript Criteria and Information The American Journal of Clinical Medicine® (AJCM®), the official journal of the American Association of Physician Specialists, Inc. (AAPS), is a peer-reviewed journal dedicated to improving the clinical practice of medicine by publishing educational and informational articles. AJCM® is the official journal of the American Association of Physician Specialists, Inc. Send all manuscripts via email to editor@aapsus.org in Microsoft Word format. No other file formats will be accepted. Manuscripts submitted by fax or mail to the Journal WILL NOT BE ACCEPTED AND WILL NOT BE RETURNED. Manuscripts received are not to be under simultaneous consideration by another publication. Accepted manuscripts become the permanent property of the American Journal of Clinical Medicine® and may not be published elsewhere without permission from the publisher. Authorship Responsibility, Financial Disclosure, Assignment of Copyright, and Acknowledgment Forms: Authorship responsibility forms must be completed and signed by each author and accompany submitted manuscripts. Each author must submit a statement that specifies whether he or she has financial or proprietary interest in the subject matter or materials discussed in the manuscript. These forms may be downloaded from the AAPS website www.aapsus.org or may be obtained by request to the AAPS office at 813-433-2277 ext 18 or 30. Authorship Responsibility: All accepted manuscripts are copyedited; an edited typescript is sent for the author’s approval. The author is responsible for all statements in the work, including the copy editor’s changes. Data Access and Responsibility: For reports containing original data, at least one author (e.g., the principal investigator) should indicate that he or she “had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis” (DeAngelis CD, Fontanarosa PB, Flanagin A. Reporting financial conflicts of interest and relationships between investigators and research sponsors. JAMA. 2001;286:89-91). Units of Measure: Conventional units of measure are preferred, with Système International (SI) units expressed secondarily (in parentheses). In tables and figures, a conversion factor to SI may be presented in the footnote or legend to economize space. Exceptions to this policy include calories, hematocrit, glycosylated hemoglobin, blood cell counts, and ejection fraction, for which conventional units alone should be expressed. The metric system is preferred for length, area, mass, and volume. Manuscript Preparation: Manuscript preparation should generally follow the guidelines outlined in The International Committee of Medical Journal Editors: “Uniform requirements

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American Journal of Clinical Medicine® • Winter 2012 • Volume Nine Number One

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UPDATED INFORMATION Fall 2011 Volume Eight, Number Three Mark Mauriello, MD, is Medical Director and Assistant to Research and Case Management at St. Michael’s Medical Center, Norwick, NJ.


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Responsible for directing and implementing program activities and services related to AAPS/ABPS Government Affairs, PR/Marketing Communications and Outreach Programs.

Responsible for Public Relations, Media Relations, Image

Jeffery L. Morris, Jr., Manager of Governmental Affairs

James G. Marzano, Director of Public Relations & Marketing

Advertising, Products and Services Marketing

We welcome your ideas and suggestions. Don’t hesitate to call on your AAPS Team.


PRESORTED STANDARD U. S. POSTAGE PAID PERMIT # 100 TAMPA, FL

5 5 5 0 We s t E x e c u t i v e D r i v e • S u i t e 4 0 0 Ta m p a , F l o r i d a 3 3 6 0 9 - 1 0 3 5

Save the date

H o u s e o f Delegates & Annual Scientific Meeting

June 25 - 29, 2012 Ritz-Carlton Hotel, Marina del Rey Los Angeles, CA


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