January 2014 Clinical Advisor by The Clinical Advisor

THE CLINICAL ADVISOR • JANUARY 2014

A PEER-REVIEWED FORUM FOR NURSE PRACTITIONERS

NEWSLINE

■■Heart health for the obese ■■Bronchial thermoplasty ■■Five tips for headache care

ADVISOR FORUM

■■Folic acid in pregnancy ■■Hepatitis C and eczema ■■Nasal lubrication

JANUARY 2014

| www.ClinicalAdvisor.com

HIV TREATMENT FOR THE

OLDER ADULT Antiretroviral therapy has made HIV (virus shown) a chronic disease for many patients.

LEGAL ADVISOR

Even unintentional HIPAA violations are serious business

✶ FREE CE COURSES!

n Dermatology Clinic

ASYMMETRICAL BROWN PATCH PAGE 71

n Dermatologic Look-Alikes VOLUME 17, NUMBER 1

SCROTAL BUMPS AND LESIONS PAGE 79 Take the Scavenger Hunt Challenge and Win an iPad! Turn to p. 46 for details

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From The Editor Editor Joe Kopcha, editor@clinicaladvisor.com Managing editor Marina Galanakis Senior editor Delicia Yard Web editor Nicole Blazek Contributing editors Bruce D. Askey, MSN, CRNP; Rebecca H. Bryan, APRN, CNP; Eileen F. Campbell, MSN, CRNP; Philip R. Cohen, MD; Deborah L. Cross, MPH, CRNP, ANP-BC; Sharon Dudley-Brown, PhD, FNP-BC; Maria Kidner, DNP, FNP-C; Joan W. Kiely, MSN, CRNP; Debra August King, PhD, PA; Ann W. Latner, JD; Claire B. O’Connell, MPH, PA-C; Kathy Pereira, MSN, FNP-BC; Sherril Sego, FNP, DNP; Julee B. Waldrop, MS, PNP; Kim Zuber, PA-C Art director Andrew Bass Group art director, Haymarket Medical Jennifer Dvoretz Production director Kathleen Millea Circulation manager Paul Silver Audience development director John Crewe National accounts manager Alison McCauley, 646.638.6098 alison.mccauley @ haymarketmedical.com Group publisher Thomas P. Hennessy, 646.638.6085 tom.hennessy @ haymarketmedia.com Editorial director Jeff Forster Vice president, medical magazines and digital products Jim Burke CEO, Haymarket Media, Inc. Lee Maniscalco All correspondence to: The Clinical Advisor 114 West 26th Street, 4th Floor, New York, NY 10001 For advertising sales, call 646.638.6085. For reprints, contact Wright’s Reprints at 877.652.5295. Persons appearing in photographs in “Newsline,” “The Legal Advisor,” and “Clinical Challenge” are not the actual individuals mentioned in the articles.They appear for illustrative purposes only. The Clinical Advisor® (USPS 017-546, ISSN 1524-7317), Volume 17, Number 1, is published 12 times a year, monthly, for $75.00 per year in the United States; $85.00 in Canada; $110.00 for all other foreign, in U.S. dollars, by Haymarket Media, Inc., 114 West 26th Street, 4th Floor, New York, NY 10001. Single copy: $20 U.S.; $30 foreign. www.ClinicalAdvisor.com. To order or update your paid subscription, call 800.436.9269. Periodicals postage rate paid at New York, NY, and additional mailing offices. POSTMASTER: Send address change to DMD Data Inc. 10255 W. Higgins Rd, Suite 280, Rosemont, IL 60018. Call 800.430.5450 to change your address or make other subscription inquiries. Requests for subscriptions from outside the United States must be accompanied by payment. All rights reserved. Reproduction in whole or in part without permission is prohibited. Copyright © 2014.

Dear readers, Patients and providers have long complained of rising health-care costs, and with good reason. That is why I am excited to offer you the opportunity to pass significant savings on to your patients with the new Clinical Advisor Pharmacy Discount Card. After an extensive search, The Clinical Advisor has joined forces with a trusted pharmacy benefits manager to deliver the most compelling prescription savings card in the market today. The advantages of this new program include: • The highest available discounts in the industry, averaging 72% and up to as high as 94%; • Zero cost to the patient; • Universal eligibility with no paperwork or prequalification; • Acceptance at more than 62,000 pharmacies nationwide, including mail-order programs; • The only plan that delivers cash rebates, in addition to at-counter discount savings, directly back to the patient; • Use of the card and its associated benefits is not limited to the primary cardholder and extends to family members, friends, co-workers, and caregivers. Patient registration is key, and this could not be easier. The patient simply presents the card to his or her pharmacist, who enters the information into the patient’s personal profile. The savings will be calculated immediately and applied to this and all future prescriptions. There are no gimmicks, and the program is totally free. For patients concerned about privacy, you can assure them that no personal information will be sold to other marketers. The program is administered through the same system in which prescriptions are adjudicated, using the standard information on file with the pharmacy. You are instrumental in helping your patients realize these savings. Please recommend this program to each and every one by printing off copies of the card and handing them out to your patients during their office visits so they may have it immediately in hand as they go to fill their prescriptions. For additional information regarding the Clinical Advisor Pharmacy Discount Card—and to print and reprint cards—please visit ClinicalAdvisor.com/rx-savings. Joe Kopcha Editor

2 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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CONTENTS JANUARY 2014

NEWS AND COMMENT

DEPARTMENTS

18 Newsline ■■Steps toward heart health for the obese ■■Poor health causes vitamin D deficiency ■■Asthma treatment good for five years ■■AAP looks at antibiotics for URTIs ■■Some unaware of diabetes risk ■■“Choose Wisely” in headache care ■■Primary-care cancer screening disparities ■■Anxiety help may come slowly in primary care ■■No link between statins and cognition ■■Guidelines for HIV in primary care updated

68 Derm Dx Read the clinical descriptions, view the images, and make your diagnosis at ClinicalAdvisor.com. 69 Legal Advisor A hospital clinician is terminated from her job after she accidentally accessed patient information beyond her purview. Low vitamin D does not cause disease 22

71 CME/CE Dermatology Clinic n Numerous painless and itchless brown macules developed on the lips of a young woman who had recently had polyps removed from her colon. n A man with a history of working

36 Drug Update ■■Treatment for two types of hypertension ■■New therapy for adults with PAH

outdoors noted a brown lesion on his right nasal dorsum with asymmetry, uneven borders, and color variation.

89 Commentary A genetic cause of childhood obesity 56

FEATURES 47 Care of the older adult with HIV Primary-care clinicians need to be prepared for the unique challenges presented by the growing number of older patients with HIV.

Continues on page 13

56 CME/CE The challenges of identifying Prader-Willi syndrome Children with this condition develop an insatiable appetite that leads to obesity, diabetes, hypertension, and behavioral problems when not treated.

MAKING CONTACT

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76 Alternative Meds Update Bovine colostrum, the first milk produced by a lactating dairy cow following the birth of a calf, is being studied as a wound-healing agent, an antimicrobial, and an anticancer therapy.

Numerous, painless brown lip macules 71

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CONTENTS 79 CME/CE Dermatologic Look-Alikes Two men—one aged 21 years and the other aged 60 years—present with asymptomatic scrotal lesions. 83 CME/CE Posttest 84 Stat Consult Find out the most recent information on the diagnosis and treatment of upper respiratory infection.

ADVISOR FORUM

Asymptomatic “red bumps” on the scrotum 79

62 Consultations ■■Bisphosphonate treatment for a fragility fracture ■■Folic acid during pregnancy ■■And more 64 Clinical Pearls ■■Be specific when taking an adolescent’s sexual history ■■Blow away your pediatric patients ■■Go beyond TSH when testing for thyroid disease 64 Your Comments ■■More OTC relief for oral mucositis

A quick review of upper-respiratory infection 84

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EXCLUSIVE TO THE WEB AT

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Fracking chemicals linked to hormone disruption Industrial spills resulting from natural-gas drilling may lead to endocrine-disrupting chemical exposure in water sources.

Are human organs on a computer chip the future of medicine? It’s relatively easy to imagine a new medicine or a better cure for some disease. The hard part is testing it, and that can delay promising new cures for years. Geraldine Hamilton shows how her lab creates organs and body parts on a chip, simple structures with all the pieces essential to testing new medications—even custom cures for one specific person.

New hypertension guidelines raise BP thresholds The Eighth Joint National Committee recommendations include higher BP cutoffs for older individuals and for patients with chronic kidney disease or diabetes. Modest weight loss seen in obese children taking metformin A review provides moderate-strength evidence of reduction in BMI from baseline at six months.

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Jim Anderson, MPAS, PA-C, ATC Role transition 101: Facing a parent’s end of life Opening the door to real emotional engagement from a family member’s illness can make it hard to close when it comes to patients.

Derm Dx Interact with your peers by viewing the images and offering your diagnosis and comments. CliniAd.com/1cer0UL

Caitlin VanderWindt, NP Improving health-care access for undocumented immigrants Restricting health-care access to undocumented workers is short-term thinking and misses an opportunity to reduce spending in the long run.

A rash flares after stopping prednisone A more severe rash developed two days after discontinuing prednisone prescribed for a mild rash.The patient was shivering and tachycardic.

MAKING CONTACT

Leigh Montejo, MSN, FNP-BC A simplified approach to overcoming care barriers Overbooked schedules and unsatisfied patients come about because the demand for medical care is greater than the supply of health-care providers.

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Newsline

Low vitamin D caused by poor health page 22

J A N U A R Y 2 014

Patients may be unaware of their diabetes risk page 25

Primary-care anxiety therapy may take years page 31

Steps toward heart health for the obese

A BMI of 25 or more may raise risks of stroke and heart disease.

research finding supports the concept of maintaining a normal body weight: A systematic review and meta-analysis of observational studies published over the past 63 years suggests that there is no healthy pattern of increased weight—that is, the concept that a person can be overweight yet healthy is a false one (Ann Intern Med. 2013;159[11]:758-769). In a subset of evaluated studies that had follow-up periods of 10 years or more, obese persons who were metabolically healthy (having, for example, normal BP, lipid, and glucose levels) were at nearly 25% greater risk for death or cardiovascular events than were metabolically healthy, normal-weight individuals. All metabolically unhealthy groups had a similarly elevated risk, whether members were at normal weight, were overweight, or were obese.

Psychotropic medication use among adolescents Males were more likely to use ADHD drugs, and females were more likely to use antidepressants.

8 7

■ Males ■ Females

6.7 5.9

Percent

OV ERW EIGHT or obese patients may be able to reduce significantly their risk for cardiovascular disease by lowering their BP, serum cholesterol, and blood glucose levels. Majid Ezzati and fellow members of the Global Burden of Metabolic Risk Factors for Chronic Diseases Collaboration (BMI Mediated Effects) pooled data from 97 prospective cohort studies involving 1.8 million participants, aged 18 years and older, who were enrolled between 1948 and 2005. The investigators learned that interventions for high BP, high cholesterol, and high glucose might address approximately half of the increased risk of coronary heart disease and three-quarters of the excess risk of stroke associated with high body mass index (BMI). C ompa red w it h nor m a l weight (BMI of 20 to <25), both

overweight (BMI of 25 to <30) and obesity (BMI of ≥30) were associated with signif icantly increased risks of coronary heart disease and stroke: 50% of the excess risk of overweight and 44% of the excess risk of obesity for coronary heart disease were mediated by BP, cholesterol, and glucose. For stroke, the percentages were 98% for overweight and 69% for obesity. Of those three factors, hypertension was found to pose the greatest risk to overweight or obese people, accounting for 31% of the increased heart disease risk and 65% of the increased stroke risk. Ezzati and coauthors noted in their report for The Lancet that patients need to maintain optimum body weight to derive the full benef its of strategies that reduce BP, cholesterol, and glucose levels. Another recent

4.5

4.2

4 3

2.0

2.2

2 Source: CDC/NCHS, National Health and Nutrition Examination Survey, 2005–2010

1 0

Any psychotropic drug

Antidepressants

ADHD drugs

18 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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Newsline THE FINDINGS of a large systematic review indicate that vitamin D deficiency is a consequence rather than a cause of various acute and chronic diseases “Low serum concentrations of 25-hydroxyvitamin D (25[OH]D) have been associated with many nonskeletal disorders,” wrote Philippe Autier, MD, and colleagues in The Lancet Diabetes & Endocrinology (2014;2[1]:76-89). “However, whether low 25(OH) D is the cause or result of ill health is not known.” The researchers analyzed 290 prospective cohort and 172 randomized trials examining the effects of vitamin D levels on nonskeletal health outcomes. Most prospective studies reflected moderate to strong inverse associations between 25(OH)D

concentrations and cardiovascular diseases, glucose metabolism disorders, infectious diseases, declining cognitive function, and several other health problems as well as all-cause mortality. Observational studies indi cated that high vitamin D levels reduced risk of cardiovascular events by up to 58%, of diabetes by up to 38%, and of colorectal cancer by up to 34%. However, randomized trials did not confirm these benefits, and metaanalyses of recent randomized trials did not show that vitamin D supplementation had any effect on disease occurrence, severity, or clinical course. Supplementation in older people, mainly women, with 20 µg vitamin D per day seemed to slightly reduce allcause mortality.

Poor health causes vitamin D deficiency

Spinal rickets (shown) may be due to vitamin D deficiency.

The discrepancy between observational and interventional studies suggests that low 25(OH)D is a marker of ill health, and that inflammatory processes involved in disease occurrence would reduce vitamin D levels and thereby explains why low vitamin D is reported in a range of disorders.

THE BENEFITS of a nonpharmacologic treatment for severe persistent asthma last for at least five years, a study has demonstrated. Approved by the FDA in April 2010, bronchial thermoplasty involves the application of radiofrequency energy to heat lung tissue and reduce the thickness of smooth muscle in the airways. The therapy had previously been shown to improve asthma control for up to two years in persons with severe persistent disease. Now, a team headed by Michael E. Wechsler, MD, report on data

Bronchial thermoplasty is used to treat severe persistent asthma.

that reflect five-year durability of the benefits of bronchial thermoplasty with regard to both asthma control (based on maintained reduction in severe exacerbations and in ED visits for respiratory symptoms) and safety. The outcomes of 162 asthmatic patients who had been followed for f ive years after undergoing bronchial thermoplast y led Wechsler and colleagues to conclude that bronchial therapy should be considered for patients with severe persistent asthma who remain symptomatic despite taking inhaled corticosteroids

and long-acting β2 -agonists ( J Allergy Clin Immunol. 2013; 132:1295-1302). Separately, the Amer ican Thoracic Society and the European Respiratory Society have provided an updated definition of severe asthma and recommendations for treating the condition in new guidelines, available in European Respiratory Journal. According to Kian Fan Chung and coauthors, severe asthma is defined as asthma that requires treatment with highdose inhaled corticosteroids plus a second controller and/or systemic corticosteroids.

Asthma treatment good for five years

22 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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Newsline AAP looks Some unaware of diabetes risk at antibiotics for URTIs

Providers discuss risk reduction with most of their at-risk patients.

factors as often as providers report measuring these factors. Providers did say they discuss diabetes and heart disease risk reduction with most of their at-risk patients. According to the ADA statement, patients may not be making the connection between risk-factor reductions and disease development. In other diabetes news, the FDA has lifted certain restrictions on prescribing and use of rosiglitazone (Avandia), after the Rosiglitazone Evaluated for Cardiac Outcomes and Regulation of Glycaemia in Diabetes (RECORD) clinical trial showed no elevated risk of heart attack or death in patients using Avandia compared with standard-of-care diabetes medications. “These data do not confirm the signal of increased risk of heart attacks that was found in a meta-analysis of clinical trials first reported in 2007,” the FDA announced in a statement issued November 25, 2013.

“Choose Wisely” in headache care THE American Headache Society (AHS) has issued its top-five list of low-value care practices in headache medicine, as part of the American Board of Internal Medicine Foundation’s “Choosing Wisely” campaign (www.choosingwisely.org). The “Five Things” that AHS implores clinicians to do—or, perhaps more accurately, not do—are as follows: (1) Don’t perform neuroimaging studies in patients with stable headaches that meet criteria for migraine.

(2) Don’t perform computed tomography imaging for headache when MRI is available, except in emergency settings. (3) Don’t recommend surgical deactivation of migraine trigger points outside of a clinical trial. (4) Don’t prescribe opioid- or butalbitalcontaining medications as a first-line treatment for recurrent headache disorders. (5) Don’t recommend prolonged or frequent use of OTC pain medications for headache (Headache. 2013;53:1651-1659).

IN AN effort to quell the overuse of antibiotics, the American Academy of Pediatrics (AAP) has outlined principles for the judicious prescribing of these agents for bacterial upper respiratory-tract infections (URTIs), including acute otitis media, acute bacterial sinusitis, and streptococcal pharyngitis (Pediatrics. 2013;132:1146-1154). The clinical report emphasizes appropriate diagnosis, as this is the foundation for making judicious decisions related to prescribing antibiotics, wrote the authors. The three principles presented are each accompanied by specific information. • Principle 1: Determine the likelihood of a bacterial infection (e.g., the diagnosis of acute otitis media always requires an otoscopic examination to confirm inflammatory changes in the tympanic membrane). • Principle 2: Weigh benefits vs. harms of antibiotics (e.g., the AAP recommends antibiotic therapy for children with clinical features of acute bacterial sinusitis, particularly those with severe or worsening symptoms). • Principle 3: Implement judicious prescribing strategies (e.g., the rates of pneumococcal resistance to macrolides and oral third-generation cephalosporins make these drugs poor choices for treating most children with suspected bacterial URTIs).

N E W I N F O R M AT I O N from the American Diabetes Association (ADA) indicates that many people who are at risk for prediabetes or diabetes have no idea that this is the case. After surveying 1,426 consumers aged 40 years and older, the ADA determined that four in 10 at-risk adults believe they have no risk for prediabetes or diabetes. In addition, only one in four of 601 health-care providers surveyed believed that at-risk patients are “extremely” or “very” knowledgeable regarding their heightened risk for type 2 diabetes. Because most at-risk patients don’t recognize or acknowledge the fact that they are, in fact, at elevated risk for diabetes, most are not at goal for key risk factors, according to an ADA press release of December 4, 2013. Fewer than half the at-risk patients said they have discussed blood glucose, BP, cholesterol, and similar metrics with their providers, and they don’t recall undergoing testing for these

www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JANUARY 2014 25

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Newsline CURRENT cancer-screening practices in primary care vary signif icantly, may not follow evidence-based practices, and may not be targeting patients considered most at risk. To determine actual rates of cancer screening in the primary-care setting, Kathryn J. Martires, MD, and co-investigators analyzed data from the National Ambulatory Medical Care Survey on 8,521 adult visits for preventive care made to office-based physicians from 2005 through 2010. The team calculated prevalence rates for breast, pelvic, and rectal examinations as well as rates for mammograms, prostate-specific antigen (PSA) tests, and Pap tests. As reported in the journal Cancer, the rates of most screening examinations and tests were

stable over time. Clinical breast examinations were conducted significantly more than mammography was ordered. White patients received more mammography, skin examinations, digital rectal examinations, and PSA tests than did patients of other races. The results demonstrated that racial and socioeconomic disparities are present in cancer screening in primary care. In another development involving cancer screening in primary care, the United States Preventive Services Task Force (USPSTF) has found insufficient evidence to recommend for or against screening for oral cancer in asymptomatic adults in the primary-care setting. Oral cancer is predominantly caused by tobacco and alcohol use.

Primary-care cancer screening disparities

White patients were screened more often than patients of other races.

In Annals of Internal Medicine, Virginia A. Moyer, MD, stated that the USPSTF found inadequate evidence that screening for oral cancer and treatment of screendetected oral cancer improves morbidity or mortality. However, the group also found inadequate evidence of the harms of screening.

SEVEN in 10 primary-care patients with anxiety disorders received potentially adequate medication or psychotherapy— eventually, as this took years to happen in many cases. A team led by Risa B. Weisberg, PhD, examined the adequacy of pharmacotherapy and psychotherapy for 534 primary-care patients with anxiety disorders at 15 U.S. sites. Study participants were interviewed at six months and 12 months after intake, and then yearly for up to five years. As the investigators reported in Depression and Anxiety, potentially

Adequate treatment was rarely received by primary-care patients.

adequate anxiety treatment was rarely received by primary-care patients with anxiety disorders at intake. However, rates improved over the course of the study. Overall, 28% of the 534 study participants stated at intake that they had received potentially adequate anxiety treatment—pharmacotherapy, psychotherapy, or both. Approximately one-fifth (19%) of patients stated at intake that they received appropriate pharmacotherapy, and 14% stated that they received potentially adequate cognitive-behavioral therapy (CBT).

Over five years of follow-up, 60% of patients said they received appropriate pharmacotherapy and 36% said they received potentially adequate CBT. Examined together, 69% of the participants stated that they had received any potentially adequate treatment during the follow-up period. Ethnic minority members were less likely to receive potentially adequate care. Persons who had major depressive disorder, panic disorder with agoraphobia, and Medicaid/Medicare insurance were more likely to receive appropriate anxiety treatment.

Anxiety help may come slowly in primary care

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Newsline A SYSTEMATIC review of available evidence uncovered no association between the use of statins and adverse cognitive outcomes, despite an FDA warning regarding such a relationship (Ann Intern Med. 2013;159[10]:688-697). On February 28, 2012, the FDA announced safety changes to the labeling of statins to warn users that memory loss and confusion had been reported in connection with these agents. The FDA affirmed that these events generally were not serious and disappeared with the discontinuation of statin use. (The agency also stated on July 3, 2012, that it continued to believe the cardiovascular benefits of statin therapy outweighed the small increase in cognitive risks.) Noting that despite the FDA warning, the relationship between statins and cognition remained

unknown, Karl Richardson, MD, and colleagues undertook an analysis of randomized controlled trials as well as cohort, case-control, and cross-sectional studies evaluating cognition in patients receiving statins. Low-quality evidence suggested no increased incidence of Alzheimer disease for statin users and no difference in cognitive performance related to procedural memory, attention, or motor speed. Moderate-quality evidence suggested no increased incidence of dementia or mild cognitive impairment, or any change in cognitive performance related to global cognitive performance scores, executive function, declarative memory, processing speed, or visuoperception. Interestingly, in cohort studies, the risk for dementia was 13% lower among statin users

No link between statins and cognition

Cohort studies show lower risk for dementia among statin users.

than among nonusers; the risk for Alzheimer disease was 21% lower for statin users; and the risk for mild cognitive impairment was 34% lower for statin users. Data showed that the reporting rate for cognitive-related adverse events with statins was low and was similar to the rates seen with other commonly prescribed cardiovascular medications.

THE Infectious Diseases Society of America (IDSA) has updated its 2009 guidelines for the primary care of persons infected with HIV. “Since 2009, new antiretroviral drugs and classes have become available, and the prognosis of persons with HIV infection continues to improve,” explained Judith A. Aberg, MD, and coauthors in the IDSA’s Primary Care Guidelines for the Management of Persons Infected with HIV, (available at cid.oxfordjournals.org/

More information on STDs has been included.

content/early/2013/11/12/cid. cit665.long, accessed December 15, 2013). “However, with fewer complications and increased survival, HIV-infected persons are increasingly developing common health problems that also affect the general population.” Among the general changes made to the guidelines, recommendations on the optimal way to diagnose HIV have given way to expanded recommendations on the initial evaluation and immediate follow-up for

HIV-positive patients. New recommendations are made regarding flu, pneumonia, varicella, and hepatitis vaccinations for these individuals, and for the screening of such patients for diabetes, osteoporosis, and colon cancer. Recommendations regarding long-term complications have been removed, and a new section on metabolic comorbidities has been added. A more robust section and table on sexually transmitted diseases also has been included. n

Guidelines for HIV in primary care updated

32 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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DrugUpdate New drug information from the publishers of MPR

Treatment for two types of hypertension Product: Adempas Company: Bayer

HealthCare Pharmacologic class:

Soluble guanylate cyclase (sGC) stimulator Active ingredient:

Riociguat 0.5 mg, 1 mg, 1.5 mg, 2 mg, 2.5 mg; tabs. Indication: Treatment of persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) (WHO Group 4) after surgical treatment or inoperable CTEPH to improve exercise capacity and WHO functional class. Pulmonary arterial hypertension (PAH) (WHO Group 1) to improve exercise capacity WHO functional class, and delay clinical worsening.

directly stimulates sGC via a different binding site, independently of NO. Riociguat stimulates the NO-sGC-cyclic guano sine monophosphate (cGMP) pathway and leads to increased cGMP generation with subsequent vasodilation. Clinical trials: CHEST-1, a double-blind, multicenter study, was conducted in 261 patients with CTEPH. Patients were randomized to Adempas titrated up to 2.5 mg three times daily (n=173) or placebo (n=88).

The primary end point was change from baseline in six-minute walking distance after 16 weeks based on imputed values. Improvements in walking distance were apparent from week 2 onward. At week 16, the placebo adjusted mean increase in six-minute walking distance within the Adempas group was 46 m (95% CI: 25 m–67 m; P <0.0001). The median difference in six-minute walking distance was 39 m (95% CI: 25 m–54 m). Patients treated with Adempas (83%) experienced an improvement in six-minute walking distance vs. placebo (57%). An open-label extension study, CHEST-2, included 237 patients who had completed CHEST-1. The mean treatment duration for the total population was 582 days (+/− 317 days). The probabilities of survival at one and two years were 97% and 94%, respectively. For more clinical trial data, see full labeling.

Company: Actelion Pharmacologic class:

Endothelin receptor antagonist Active ingredient: Macitentan 10 mg; tabs. Indication: Treatment of pulmonary arterial hypertension (PAH) (WHO Group 1) to delay disease progression. In clinical studies, treatment delayed disease progression (death, initiation of IV or subcutaneous [SC] prostanoids, or clinical worsening of PAH [decreased six-minute walk distance, worsened PAH symptoms, and need for additional PAH treatment]) and reduced hospitalization for PAH.

times daily; if intolerant, consider starting at 0.5 mg three times daily. Increase Continued pg. 38

Continued pg. 38

Adults: Initially 1 mg three

Adempas helps arteries relax to increase blood flow and decrease BP.

Product: Opsumit

Pharmacology: Macitentan prevents the binding of endothelin (ET)1 to both ETA and ETB receptors with displays of high affinity and sustained

Pharmacology: Riociguat

has a dual mode of action: it sensitizes sGC to endogenous nitric oxide (NO) by stabilizing the NO-sGC binding. Also, riociguat

New therapy for adults with PAH

For more products, visit www.eMPR.com

36 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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DrugUpdate Adempas from pg. 36

dose by 0.5 mg (three times daily) at intervals of two or more weeks as tolerated (systolic BP >95 mm Hg and no hypotension); maximum 2.5 mg three times daily. If intolerant at any time, decrease dose by 0.5 mg three times daily. Smokers: consider titrating to doses >2.5 mg three times daily; may require dose reduction after smoking cessation. Concomitant strong CYP450 and P-glycoprotein (P-gp)/breast cancer resistance protein (BCRP) inhibitors: initiate at 0.5 mg three times daily. Children: Not established. Contraindications:

Pregnancy (Category X). Concomitant nitrates or Opsumit from pg. 36

occupancy of the ET receptors in human pulmonary arterial smooth muscle cells. Clinical trials: The efficacy of macitentan was demonstrated in a multicenter, long-term, placebocontrolled study involving 742 patients with symptomatic (WHO functional class [FC] II-IV) PAH. Patients were randomized to placebo (n=250), macitentan 3 mg (n=250),

nitric oxide donors in any form, specific or nonspecific phosphodiesterase inhibitors (e.g., sildenafil [Revatio, Viagra], tadalafil [Adcirca, Cialis], vardenafil [Levitra, Staxyn], dipyridamole [Persantine], theophylline). Warnings/Precautions:

symptoms occur. Bleeding risk. Pulmonary venoocclusive disease: not recommended; discontinue if confirmed. Severe hepatic (Child Pugh C) or renal impairment (creatinine clearance <15 mL/min), or on dialysis: not recommended. Nursing mothers: not recommended.

In females of reproductive potential, exclude pregnancy prior to starting, monthly during, and for one month after treatment discontinuation; must use acceptable methods of contraception. Risk of hypotension or ischemia in hypovolemia, severe left ventricular outflow obstruction, resting hypotension, autonomic dysfunction; consider dose reduction if signs/

Interactions: See Contraindications. Potentiated by strong CYP450 and P-gp/BCRP inhibitors (e.g., ketoconazole [Nizoral], itraconazole [Onmel, Sporanox], ritonavir [Norvir]); monitor for hypotension. Antagonized by smoking. May be antagonized by strong CYP3A inducers (e.g., rifampin [Rifadin], phenytoin [Dilantin,

or macitentan 10 mg (n=242) once daily. The primary end point was death or time to the first occurrence of a significant morbidity event, defined as atrial septostomy, lung transplantation, initiation of IV or SC prostanoids, or “other worsening of PAH” during doubleblind treatment plus seven days. Other worsening was defined as: (1) a sustained ≥15% decrease from baseline in six-minute walk

In PAH, the right side of the heart is forced to work harder than normal.

Phenytek], carbamazepine, phenobarbital, St. John’s Wort). Separate dosing of antacids by one hour or more of riociguat. Adverse reactions: Headache, dizziness, dyspepsia/gastritis, nausea, diarrhea, vomiting, anemia, gastroesophageal reflux, constipation. Note: For all female patients: available only through the Adempas REMS program. To enroll call 855.4ADEMPAS or www.AdempasREMS.com. How supplied: Tabs—42,

90 For more information, call 855.4ADEMPAS or visit www.Adempas-us.com. distance; (2) worsening of PAH symptoms (worsening of WHO FC); and (3) need for additional treatment for PAH. A critical secondary end point was time to PAH death or PAH hospitalization. The median treatment durations were 101 and 118 weeks in the placebo and Opsumit 10 mg groups, respectively, with a maximum of 188 weeks. Treatment with Opsumit Continued pg. 40

For more products, visit www.eMPR.com

38 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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DrugUpdate 10 mg resulted in a 45% reduction (HR 0.55, 97.5% CI: 0.39–0.76; P <0.0001) in the occurrence of the primary end point up to end of treatment compared with placebo. The beneficial effect of Opsumit 10 mg was mainly due to a reduction in clinical worsening events. In the secondary end point, the risk of PAHrelated death or hospitalization for PAH was reduced by 50% in patients receiving Opsumit 10 mg vs. placebo (HR 0.50, 97.5% CI: 0.34– 0.75; P <0.0001). Treatment with Opsumit resulted in a placebo-corrected mean increase in six-minute walk distance of 22 meters at month 6 (97.5% CI: 3–41; P=0.0078), with significant improvement in six-minute

walk distance by month 3; in addition, improvement of at least one WHO FC at month 6 in 22% of patients was seen compared with 13% of patients treated with placebo. Adults: 10 mg once daily.

Doses >10 mg once daily are not recommended.

Children: Not established. Contraindications:

Pregnancy (Category X). Warnings/Precautions:

initiation and repeat during treatment as clinically indicated. Discontinue if clinically relevant aminotransferase elevations occur, or if elevations are accompanied by clinical symptoms of hepatotoxicity. Not recommended in patients with severe anemia. Measure hemoglobin prior to initiation and repeat during treatment as clinically indicated. Consider pulmonary veno-occlusive disease if signs of pulmonary edema occur; discontinue if confirmed. Potential decrease in sperm count; counsel men on fertility effects. Nursing mothers: not recommended. Interactions: Potentiated

by strong CYP3A4 inhibitors (e.g., ketoconazole

“O.K., we got it—big and dangerous.”

[Nizoral], ritonavir [Norvir]); avoid concomitant use. Antagonized by strong CYP3A4 inducers (e.g., rifampin [Rifadin]); avoid concomitant use. Adverse reactions: Anemia, nasopharyngitis/ pharyngitis, bronchitis, headache, influenza, urinary tract infection; decreased hemoglobin. Note: For all female

patients: available only through the Opsumit REMS program. To enroll call 866.228.3546 or www. OpsumitREMS.com. How supplied: Tabs—15,

30. For more information, call 866.228.3546 or visit www.Opsumit.com. n

“Do we want a time-share on Two-Tree Island?”

Opsumit from pg. 38

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40 THE CLINICAL ADVISOR • DECEMBER 2013 • www.ClinicalAdvisor.com

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Take the Scavenger Hunt Challenge January edition

Correctly answer the questions below— all of which can be found within this issue of The Clinical Advisor—and you’ll be entered into a random drawing to win an Apple iPad mini. To submit your responses, simply go to CliniAd.com/1evv5kL. QUESTIONS 1. According to observational studies, high vitamin D levels reduced risk of cardiovascular events by how much? (p. 22) 2. What percentage of primary-care patients with anxiety disorders receives potentially adequate medication or psychotherapy? (p. 31) 3. In what year was the deadly retrovirus HIV identified? (p. 47) 4. By 2015, what predicted percentage of all people living in the United States with HIV will be aged 50 years or older? (p. 48) 5. What is the estimated prevalence of Prader-Willi syndrome? (p. 56) 6. Approximately how many people in the United States are affected by a rare disease? (p. 58) 7. For most women, what is the recommended daily dosage of folic acid? (p. 63) 8. What are the most characteristic cutaneous findings of Peutz-Jeghers syndrome? (p. 72) 9. What is another name for colostrum? (p. 76) 10. In what year was angiokeratoma of Fordyce first described? (p. 81)

WHO MAY ENTER All nurse practitioners and physician assistants 21 and over who are on The Clinical Advisor’s subscriber list. Employees and families of employees of Haymarket Media Inc., its affiliates, printer, agencies, mailers, and advertisers are not eligible. RULES: No purchase necessary. Entries are limited to one per person. Void where prohibited. All entries must be received by February 15, 2013. Entries become the property of The Clinical Advisor and will not be acknowledged or returned. The Clinical Advisor is not responsible for late or misdirected entries, illegible entries, or electronic malfunctions. Entry constitutes acceptance of all rules. PICKING WINNERS Winners will be randomly selected from all accepted entries received by the deadline. Winners will be notified no later than March 1, 2013. Winners will be required to sign an affidavit of eligibility within 14 days of notification, or another winner will be chosen. Where permitted by law, winners agree to the use of their names, likenesses, and photographs for promotional purposes, without additional compensation. Odds of winning depend on the number of entries received. Winners agree to release and hold harmless The Clinical Advisor and Haymarket Media, Inc. from any liability arising from participation in this contest or acceptance and use of a prize. Names of winners will be published in a future issue of The Clinical Advisor. The winners’ names will be available upon written request after March 1, 2013, to individuals who send a stamped, self-addressed, business-sized envelope to Clinical Advisor Contest Winners, 114 W. 26th St., 4th Floor, New York, NY 10001.

46 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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FEATURE: KELLEY GAEDTKE FARBER, ANP

Care of the older adult with HIV Primary-care clinicians need to be prepared for the unique challenges presented by the growing number of older patients with HIV.

HIV (shown) has a long incubation period, with a slow disease evolution.

n 1981, physicians in Los Angeles and New York City began reporting unusual occurrences of rare diseases, such as Pneumocystis jiroveckii pneumonia (formerly Pneumocystis carinii pneumonia) and a rare skin disorder called “Kaposi sarcoma,” in homosexual male patients. On further study, these patients were found to have a severe depletion of CD4 T cells, resulting in an immune system deficiency. In 1983, the deadly retrovirus HIV was identified. Since then, researchers and health-care providers have been in a race to identify the transmission, prevention, and treatment of HIV. Fast-forward to 2014, when thanks to the era of highly active antiretroviral therapy (HAART), HIV has become a chronic disease for many patients. More people in the United States are living with HIV than ever before. With new effective therapies, many people with HIV are living better and longer lives with chronic disease—into their 50s and beyond. At the same time, the number of HIV diagnoses in people aged 50 years and older is rising, creating a new subset of patients with unique risks and challenges for primary-care providers. The vast majority of studies on symptom management and disease sequelae done over the past 25 years have involved younger adults, literally excluding older patients with their unique comorbidities. This article was written to help primary-care health providers in addressing the prevention and treatment challenges specific to the older adult with HIV. Pender’s Health Care Promotion Model will

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HIV IN THE OLDER ADULT

be utilized to help the provider develop and implement care proactively and purposefully.1

POLL POSITION

Challenges in prevention and diagnosis

Generally speaking, 50-year-olds do not fall into the category of “older adults.” In the HIV-infected population, however, the definition of “older adult” requires some adjustment. Until recently, the CDC did not keep data on HIV patients older than 50 years. To this day, United Nations surveillance data are kept only until age 49 years.2 A 2008 report from the CDC showed HIV infection and acquisition rates trending upward in people older than age 50 years whereas in the past, HIV was thought of as a disease of “young adults” aged 18 to 30 years.3 In 2005, persons in the United States aged 50 years and older represented 15% of new HIV diagnoses, 24% of the population living with HIV/AIDS, and 35% of all deaths from AIDS.3 With these rates rising steadily since 1990, the prediction is that half of all people living in the United States with HIV will be aged 50 years or older by 2015.4 These trends make clear the importance of discussing with patients the topic of HIV and aging. Risk factors

Many of the same HIV risk factors exist for both younger and older adults. IV drug use and failure to practice safer sex are common transmission methods in both groups. However, older adults are less likely to perceive themselves as being at risk. Older women may not insist on condom use once they are past childbearing years. For their part, health-care providers may not appreciate the older adult’s risk of contracting HIV and, like older patients themselves, may not perceive these patients as being at high risk.This leads to a lack of education on safety and prevention and a delayed diagnosis in this subset of patients. Additionally, the symptoms that may lead to an HIV diagnosis in a younger adult, such as fatigue, weight loss, and mental confusion, could be attributed to the normal aging process in the older adult. The possibility of a missed diagnosis is ever-present in the older population, delaying the antiretroviral therapy that could improve the patient’s health and longevity. Clinicians need to screen patients routinely and assess their risk factors thoroughly. Using a “don’t ask, don’t tell” philosophy while taking a sexual history can delay diagnosis and treatment of patients at risk for HIV.

Do you support raising the age range for HIV screening from age 64 years to age 75 years? n=89 18%

n Yes: 82% n No: 18%

82%

For more polls, visit CliniAd.com/10TDwDb.

years, including pregnant women. Any patients older than 64 years who are believed to be at high risk for HIV exposure, including those who are IV drug users (IVDUs) and those who engage in unprotected sex when the serostatus of their partner is unknown, should be counseled about HIV testing. In a 2009 statement, the American College of Physicians suggested that the age range for screening be increased to 75 years because of the growing number of infections in this older age group.5 The goals of these recommendations are not only to identify new infections but to take away the stigma and fear of HIV testing in the United States and to encourage more patient/caregiver conversation about high-risk behaviors and prevention methods.3 The need for education must be reinforced among health-care professionals to avoid missed opportunities for discussion and risk-factor reduction with older patients. Clinicians need to question their older patients about their risk behaviors and discuss with them the risks of HIV and sexually transmitted infections. Many states now offer an “optout” testing script that clinicians can use when recommending HIV testing for patients, such as pregnant women; yearly HIV testing for high-risk populations, such as IVDUs or those practicing unsafe sex; or diagnostic testing for a patient with clinical symptoms.6 Patients must understand that they have the choice to decide whether or not they want to be tested for HIV and that health-care treatment cannot be denied if they decide not to be tested. (See “Obtaining consent for HIV testing,” p. 51.) Comorbidities of the older patient with HIV

Screening the older patient

The CDC’s current recommendation includes routine HIV screening at least once for all Americans aged 13 years to 64

Comorbid conditions are more likely to occur in older patients with HIV than in younger patients. Common comorbidities include hypertension, chronic obstructive

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HIV IN THE OLDER ADULT

pulmonary disease, diabetes mellitus, arthritis, dermatologic manifestations, hepatitis C, coronary artery disease, renal disease, and lipid disorders.7,8 Studies show an increase in neurologic conditions in older HIV patients, such as cognitive dysfunction/dementia and Parkinson disease, both of which involve basal ganglia and dopamine pathways.7 Older patients are more likely to use alcohol and experience depression and drug abuse or dependence.9 Osteopenia and osteoporosis are becoming more common in both older men and older women with HIV; infection with HIV is thought to cause increased osteoclast activity.10 Some studies suggest that HIV in older persons may elevate the risk of cardiovascular disease (CVD). Antiretroviral therapy is known to increase risk of metabolic abnormalities, such as increased lipid levels, but whether antiretroviral agents are solely responsible for the increase in CVD has yet to be determined.11 Liver disease occurs in older HIV patients at higher rates as a result of hepatitis B and C, alcohol use, diabetes, and toxicities associated with lipid-lowering drugs. Drug-related toxicity in this older group may result from age-associated alterations in albumin levels and from changes in the cytochrome P-450 enzyme, which aids in metabolizing two of the main classes of antiretrovirals. Thus, older patients may have significantly higher drug levels and drug toxicities than do younger patients due to decreased elimination. Renal function also declines with age, leading to additional reductions in drug elimination. Given the multitude of health problems affecting older patients, polypharmacy and the potential for drug interactions must be considered.11 Clinicians managing the care of persons with HIV must be aware of the interactions between antiretroviral agents and other commonly used drugs. Allopurinol, erectile dysfunction drugs, benzodiazepines, and proton pump inhibitors are just a few examples of the medications requiring special attention when used by persons on antiretroviral therapy.7 Clearly, antiretroviral therapy for HIV needs to be closely monitored and adjusted for hepatic and renal functioning. The immunosuppression resulting from HIV infection will lead to increased susceptibility to malignancy, and rates of cancer in HIV patients are expected to soar over the next 10 years. Cancer-screening guidelines will have to be adjusted for this high-risk population.7,8,12 Among the HIV-related malignancies diagnosed more frequently in older patients are non-Hodgkin lymphoma, Kaposi sarcoma, cervical cancer, rectal cancer, lung cancer, and hepatocellular carcinoma, related to the presence of human papillomavirus or hepatitis B and C coinfection. As the population of older patients with HIV

continues to grow, clinicians must continue to recommend routine preventive measures, such as colorectal screenings, Pap smears, and mammography, as well as immunizations and vaccinations. The guidelines for these preventive measures are likely to change over the next 10 years as more studies and trials include the older subset of the HIV population. Education and prevention

Pender’s Health Promotion Model challenges health-care providers to identify the factors that are known to affect health and categorize them into a model with variables that may help explain human health behavior. That information is then used as a basis for structuring protocols and interventions. The model urges health-care providers to address these issues with their patients and to encourage self-efficacy and the behaviors from which patients can derive personal health benefits before or after a diagnosis is made. The greater the level of knowledge and commitment on the patient’s part, the greater the likelihood that he or she will adhere to a plan that will encourage personal growth, positive changes, and stability in chronic illness.13-15 Table 1 incorporates Pender’s seven cognitive-perceptual factors into health promotion behaviors that can positively influence older Americans with HIV or those who are at risk of HIV exposure.1 Continues on page 54

Obtaining consent for HIV testing The following is a scripted example clinicians can use when seeking consent for HIV testing, based on Wisconsin Act 209:6 Step 1—Clinician: “In my practice, I recommend HIV testing for many of my patients, so I am planning to test you for HIV unless you decline to be tested.” Step 2—Provide written or verbal information on HIV infection, test results, reporting requirements (in Wisconsin, all positive tests must be reported to the state’s Division of Public Health), treatment options, and services available for people who test positive. Step 3—Clinician: “Do you have any questions about HIV or HIV testing?” Allow sufficient time for questions and the opportunity for the patient to decline. Step 4—Clinician: “As long as it is OK with you, I will go ahead and order an HIV test.” The provider then verifies that the patient understands that the test will be performed and that the patient’s acceptance is voluntary.

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HIV IN THE OLDER ADULT

TABLE 1. Pender’s Health Promotion Model as it relates to HIV infection in older adults1 Cognitive-perceptual factors

Health-promotion strategy for older adults who have HIV or are at risk of HIV

Health-promotion strategy for health-care providers

1. Health in general needs to be understood and valued

• Value your own health status and that of others so that you make changes to risk behaviors for exposure to, or transmission of, HIV. • Undergo regular yearly testing for HIV if you engage in risk behaviors, such as using IV drugs and sharing needles or having unprotected sex with a partner whose serostatus is unknown.

• Do not assume that an older/elderly person or one presumed to be in a monogamous relationship is not engaging in behaviors that could be high-risk. • Obtaining a sexual and social history is important. Explain the reasons that a person should be tested, and use the “opt-out of testing” script. • Encourage the need for risk behavior modification.

2. People need to have a perceived control of their health promotion behaviors.

• Education from such trusted community sources as public-health officials, HIV clinics, or health-care providers is vital to reducing transmission and exposure rates and improving treatment adherence.

3. Self-efficacy reinforces the idea that positive health behaviors can produce a positive or desired result.

• Avoid unsafe sexual practices by knowing the serostatus of partners, using condoms, and preventing pregnancy. • HIV-positive mothers should follow recommendations for perinatal care. • Understand the methods of transmission and how to stop the spread of HIV/AIDS. • Establish care with a clinic that treats HIV on a regular basis in order to ensure that you are working with an experienced health-care provider. • Exercise regularly, eat a nutritious diet, do not smoke or drink alcohol. • Be aware of possible drug interactions; check with your healthcare provider before taking new medications.

4. Adjust the definition of health from the idea of “absence of illness” to actions that make health the best that it can be.

• Become your own advocate when it comes to health care. • Participate in support groups. • Return to your health-care provider for follow-up care, medication checks, laboratory studies, immunizations, vaccinations, and preventive health screenings. • Watch for signs of depression.

• Encourage drug rehabilitation services or use of clean needles to reduce exposure to HIV.

5. Truly understand your health status. • Know the “measures” of your HIV status: CD4 cell counts, viral load, kidney and liver function, bone mineral density, and lipid levels. How does your health behavior affect these? 6. Understand the benefits of health-promoting behaviors.

• Know the signs that should prompt a call to your health-care provider: fever, vomiting, diarrhea, jaundice, decreased urinary output, sudden onset of pain or shortness of breath, forgetfulness, or tremors.

7. Know the perceived barriers to improving overall health.

• Educate patients on identification of opportunistic infections, action of antiretroviral medications, and healthcare maintenance required to monitor their chronic condition. • Ask about and identify barriers to improving overall health, which could include fear of stigma, racial mistrust, socioeconomic disparities, financial expense, lack of transportation, inaccessibility of health care, and lack of overall resources.

52 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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HIV IN THE OLDER ADULT

What clinicians can do

As the life expectancy of persons with HIV increases, more research will be needed to identify age-specific barriers, treatments, and toxicities. Compared with younger HIV patients, those older than age 50 years tend to have a more rapid disease progression and greater immune system suppression. Older patients also tend to have more opportunistic infections and cancers, shortened survival, and shorter time to AIDS compared with younger patients. Management of disease sequelae may be a challenging addition to the monitoring of other age-related conditions.7 More research and evidence-gathering is needed for this population. The misconception in the United States that elderly persons are not at risk for HIV infection must change and should be addressed by prevention programs. Few HIV-prevention programs target older adults specifically. Such groups as HIV Wisdom for Older Women (hivwisdom.org) and the National Association of HIV over Fifty (hivoverfifty.org/en/) provide support, education, and resources for older adults affected by HIV.8 Health-care providers must continue to advocate for and educate older patients in terms of HIV and AIDS, HIV testing, high-risk behaviors, and risk behavior modification. Early diagnosis and treatment of this older age group could help reduce mortality and morbidity, decrease the risk of HIV transmission, and provide much-needed support and resources. n

CLINICAL SLIDESHOW For more information on identifying skin conditions related to HIV/AIDS, view the slideshow at CliniAd.com/10nuwF5.

5. Qaseem A, Snow V, Shekelle MD, et al. Screening for HIV in health care settings: a guidance statement from the American College of Physicians and HIV Medicine Association. Ann Intern Med. 2009;150:125-131. Available at www.annals.org/content/150/2/125.full.pdf+html. 6. Wisconsin AIDS/HIV Program. Sample script for clinicians regarding verbal consent for HIV testing. Available at www.dhs.wisconsin.gov /aids-hiv/clinicianresources/providerscript052010.pdf. 7. Gebo K. HIV and aging: implications for patient management. Drugs Aging. 2006;23:897-913. 8. Luther V, Wilkin A. HIV infection in older adults. Clin Geriatr Med. 2007;23:567-583. 9. Kilbourne AM, Justice AC, Rabeneck L, et al. General medical and psychiatric comorbidity among HIV-infected veterans in the post-HAART era. J Clin Epidemiol. 2001;54:S22-S28. 10. Kearney F, Moore AR, Donegan CF, Lambert J. The ageing of HIV: implications for geriatric medicine. Age Ageing. 2010;39:536-541. Available at ageing.oxfordjournals.org/content/39/5/536.long.

Ms. Gaedtke Farber is an adult nurse practitioner in chronic pain management at Marshfield Clinic in Eau Claire, Wis.

11. Sabharwal C, Casau-Schulhof N. HIV and the older patient. Available at www.uptodate.com/contents/hiv-and-the-older-patient. 12. Volberding P. Hematology and oncology in patients with human immu-

References

nodeficiency virus infection. In: Goldman L, Ausiello D, eds. Cecil Medicine.

1. Pender N. Health Promotion in Nursing Practice. 3rd ed. Stamford, Conn.:

23rd ed. Philadelphia, Pa.: Saunders Elsevier; 2008:2601-2606.

Appleton and Lange; 1996.

13. Raile Alligood M, Marriner-Tomey A. Nursing Theorists and Their Work.

2. Emlet CA, Poindexter CC. Unserved, unseen, and unheard: integrating

Philadelphia, Pa.: Mosby Elsevier; 2006.

programs for HIV-infected and HIV-affected older adults. Health Soc Work.

14. Pender N. A conceptual model for preventive health behavior. Nurs

2004;29:86-96.

Outlook. 1975;23:385-390.

3. Centers for Disease Control and Prevention. HIV/AIDS among persons

15. Pender N, Barkauskas VH, Hayman L, et al. Health promotion and

aged 50 and older. Available at www.cdc.gov/hiv/pdf/library_factsheet_

disease prevention: toward excellence in nursing practice and education.

HIV_among_PersonsAged50andOlder.pdf.

Nurs Outlook. 1992;40:106-112, 120.

4. Administration on Aging. Older adults and HIV/AIDS. Available at www. aoa.gov/AoARoot/AoA_Programs/HPW/HIV_AIDS/.

All electronic documents accessed December 15, 2013.

For more news, opinion, and clinical features on HIV/AIDS and other infectious diseases, visit our Information Center at

CliniAd.com/12yUmEr. 54 THE CLINICAL ADVISOR â&#x20AC;˘ JANUARY 2014 â&#x20AC;˘ www.ClinicalAdvisor.com

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CME CE

PROGRAM OUTLINE JANUARY 2014

0.5 CREDITS

Page 56 FEATURE

The challenges of identifying Prader-Willi syndrome Susan Ann Vitale, PhD, RN, PNP, ANP-C, and Gina Kearney, RN, BC, AHN Susan Ann Vitale, PhD, RN, PNP, ANP-C, and Gina Kearney, RN, BC, AHN, have no relationships to disclose relating to the content of this article.

■■ LEARNING OBJECTIVES: • Explain the diagnostic factors associated with Prader-Willi syndrome in infants and adults. • Name the current genetic testing available. • Discuss comorbidities associated with Prader-Willi syndrome. • Identify resources that can be provided to patients and their families.

0.5 CREDITS

Page 71 DERMATOLOGY CLINIC

Painless brown macules on the lips and fingers Audrey Chan, MD, and William Longhurst Audrey Chan, MD, and William Longhurst have no relationships to disclose relating to the content of this article.

Growing nasal lesion with asymmetry Esther Stern, NP-C

Esther Stern, NP-C, has no relationships to disclose relating to the content of this article.

■■ LEARNING OBJECTIVE:

• Identify and diagnose dermatologic conditions and review up-to-date treatment.

Page 79 DERMATOLOGIC LOOK-ALIKES Asymptomatic scrotal lesions Joe Monroe, PA-C Joe Monroe, PA-C, has no relationships to disclose relating to the content of this article.

■■ LEARNING OBJECTIVE:

• Distinguish and properly treat dermatologic conditions with similar presentations.

Page 83 POSTTEST Physician Credit This activity has been planned and implemented in accordance with the Essential Areas and Policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of Medical Education Resources (MER) and Haymarket Medical. MER is accredited by the ACCME to provide continuing medical education for physicians.

Credit Designation Medical Education Resources designates this enduring material for a maximum of 1 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity. American Academy of Physician Assistants AAPA accepts certificates of participation for educational activities certified for Category I credit from AOACCME, Prescribed credit from AAFP and AMA PRA Category 1 Credit™ from organizations accredited by ACCME or a recognized state medical society. Physician Assistants may receive a maximum of 1 hour of Category I credit for completing this program. Nursing Credit Medical Education Resources is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. This CE activity provides 1 contact hour of continuing nursing education. Medical Education Resources is a provider of continuing nursing education by the California Board of Registered Nursing, Provider #CEP 12299, for 1 contact hour.

www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JANUARY 2014 55

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CME CE

n EDUCATIONAL OBJECTIVES: After completing this activity, the participant should be better able to: • Explain the diagnostic factors associated with Prader-Willi syndrome in infants and adults. • Name the current genetic testing available. • Discuss comorbidities associated with Prader-Willi syndrome. • Identify resources that can be provided to patients and their families. n COMPLETE THE POSTTEST: Page 83 n ADDITIONAL CME/CE: Pages 71, 79

FEATURE

Turn to page 55 for additional information on this month’s CME/CE courses.

SUSAN ANN VITALE, PhD, RN, PNP, ANP-C, AND GINA KEARNEY, RN, BC, AHN

The challenges of identifying Prader-Willi syndrome Children with this condition develop an insatiable appetite that leads to obesity, diabetes, hypertension, and behavioral problems when not treated.

Parents of children with PWS may consider locking the refrigerator and kitchen cabinets.

pproximately 17% of children and adolescents in the United States are overweight or obese, which represents more than 12.5 million young people.1 Screening for obesity includes tracking weight, height, and BMI. Less desirable scores may be attributed to poor nutritional habits, a sedentary lifestyle, or a differential diagnosis (e.g., hypothyroidism). Comorbidities, including hypertension and diabetes, are often present in overweight or obese individuals. Although classified as a rare disease, Prader-Willi syndrome (PWS) is the most common genetic cause of lifethreatening obesity in children. Gender and race differences have not been reported in this syndrome. Prevalence of PWS is estimated at 1 in 10,000 to 1 in 15,000.2 The following case of a boy with PWS will illustrate the importance of considering the etiology of childhood overweight and obesity. After PWS was properly identified, this child’s family was able to access resources that were used to address specific diet- and disease-related problems. The case is followed by a

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TABLE 1. Office visits of Bobby T Visit 1

Family health history: Negative Birth history: No pregnancy abnormalities, 5 lb. 2 oz. infant, 18” length, breech delivery; APGAR unknown History first year: Fussy baby, feeding difficulties, underweight, pale History years 1-2: Short stature, global delays, poor appetite until age 2 years; insatiable appetite and behavior difficulties then developed History years 3-4: Overweight progressed to obesity, no illnesses reported, lack of medical evaluations, no schooling or activities outside home Examination: Obese 4-year-old boy; BMI above the 95th percentile; vital signs stable except BP (prehypertensive): pale, hypopigmented skin; small skin lesions picked at by the child during the exam; short stature; mild strabismus; slightly small hands and feet; significant developmental delays in language, cognition, fine-motor skills, and gross-motor skills; generalized hypotonia; clumsy gait Assessment: Exogenous weight gain including overeating, rule out endogenous etiology. Siblings need health evaluations. Preliminary diagnosis: Obesity, prehypertension risks, global developmental delay, family stress Differentials: Exogenous obesity, hypothyroidism, diabetes, genetic disorder Evaluations ordered: Complete blood count, chemistry panel, urine analysis, ECG Referrals: Social work, early intervention Return: One week

Visit 2

BP: Repeat BP checks confirmed prehypertension. Laboratory analysis: Serum glucose 104 mg/dL; all other results normal ECG: Unsuccessful due to child’s behavior Social work assessments: In progress, insurance applications made Referrals: Endocrinology, medical nutrition therapy, genetics Return: One month

Visit 3

Nutrition: Consult started, but mother did not follow through; felt accused of being a bad mother. Endocrine: Gonadotropism, genetic evaluation reinforced ECG: Normal Early intervention: In progress

Sibling Visits

Young sibling evaluations. 12-month-old sister: Developmentally normal and healthy 2-year-old sister: Overweight (BMI slightly above 85th percentile); motor and speech delays

summary of current treatment options, caregiver guidelines, and resources for individuals with PWS and their family. Case study

Bobby T, aged 4 years, was an obese child whose mother, a trim woman of slight build, brought him to a primary-care clinic for a checkup. Bobby had a round face that made his facial features less distinct. The boy walked awkwardly into the examination room and had to hold his mother’s hand to steady his gait. His sister, aged 3 years, was also slightly overweight, mainly in the midsection. In the waiting room, Bobby ignored his mother’s pleas to stop ripping pages out of magazines. Mrs. T apologized and indicated that she could no longer control her son’s behavior. In the examination room, Mrs. T explained that her husband (Bobby’s father) had recently separated from the family and moved out of the home. He blamed his wife for Bobby’s behavior problems and obesity and accused her of overfeeding the child. Bobby was apparently a very small infant at birth but began gaining weight at age 2 years, gradually growing

from overweight to obese. According to Mrs. T, Bobby was always hungry and never satiated, and this was a constant source of family arguments. Lacking financial security, Mrs. T had moved in with her own mother, who insisted on health checkups for the children. Their prior medical care was almost nonexistent due to poor finances and a lack of insurance. Mrs. T also kept Bobby secluded at home because of his disruptive behavior. Bobby received multiple health-care evaluations over the next three months (Table 1). Mrs. T was uncomfortable with health care at first and tried to avoid the follow-up visits. She expressed concern that she was an inadequate mother and kept blaming herself for Bobby’s obesity and bad behavior. Bobby’s grandmother was able to provide support for the series of evaluations. Family genetics evaluations. Several family visits with the geneticist and subsequent laboratory evaluations revealed that Bobby had PWS. It was also established that his siblings did not have the disorder. Children with PWS are frequently of normal or small size at birth and can even be diagnosed with

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In the second phase of PWS, a child previously characterized as a poor eater or even a failure to thrive will begin to want to eat all the time. failure to thrive. Bobby’s physical signs, behavioral problems, and developmental delays were characteristic of a child with PWS. His weight gain was consistent with a diagnosis of PWS as well, as children with the syndrome can become overweight or obese without proper monitoring. Subsequent care. Bobby received occupational, physical, and speech therapy initially at home and subsequently within a school setting. Mrs. T obtained counseling and eventually found relief. She no longer blamed herself for Bobby’s condition. She then was able to better control her son’s insatiable appetite and difficult behavior. The entire family ate healthier meals and engaged in appropriate exercise. Mrs. T and her mother became actively involved in PWS support organizations (Table 2). Rare diseases

Any disease that affects fewer than 200,000 people is considered rare; there are currently approximately 7,000 such diseases. In total, rare diseases affect more than 6 million people in the United States alone (worldwide incidence remains unknown). A lack of wide-scale classification and tracking systems for each rare disease has been an obstacle to more precise estimates. Data are limited as to specific or grouped rare diseases. Knowledge is deficient as to progression, treatment, and anticipatory guidance. It is common for afflicted families to experience isolation from others living with a similar disease. Individuals and family members coping with PWS may be acutely aware of its uncommon nature and can find support through the Prader-Willi Association (www.pwsausa.org). Genetic testing

There are several rare genetic causes of childhood obesity, including PWS, Cohen syndrome, congenital leptin deficiency, leptin receptor deficiency, pro-opiomelanocortin defects, prohormone convertase 1 deficiency, and human melanocortin receptor deficiency.3 PWS is the most commonly occurring genetic syndrome among the causes of childhood obesity. Prader, Labhart, and Willi were the endocrinologists who first described the disorder in 1956. By 1981, PWS was recognized as a microdeletion syndrome and became the first known genomic imprinting disorder.4 Approximately 75% of PWS cases result from an absence or failure of normally activated paternally inherited genes on the proximal long arm of chromosome 15. An estimated 20% of

PWS cases have two maternal copies of chromosome 15 (no paternal copies). This abnormality is referred to as maternal uniparental disomy and has been associated with increased maternal age.5 An additional 5% of individuals with PWS have some other translocation or abnormality involving chromosome 15. The few PWS cases that recur within the same family (approximately 1%) are caused by a microdeletion in the imprinting center.4 PWS occurs in both genders and in all races. Estimates of reported incidence range from 1 in 8,000 live births to one in 29,000 live births. Incidence may be underestimated. Many cases of PWS are not diagnosed at an early age. Chromosomal microdeletions are not performed in standard amniocentesis or in chorionic villus sampling.4 A family health history may be helpful when determining risk for PWS. The U.S. Department of Health and Human Services has created a website to facilitate this activity (familyhistory.hhs.gov/). After entering the family health history, the user can print out his information to share with other family members or a health-care provider. The confidential information can also be shared electronically. Once saved, the family history can be updated over time. The clinical picture

PWS is considered a multistage disorder that includes three distinct developmental phases. Consensus criteria for diagnosis were developed in 1993.4,5 First phase (prenatal to infancy). The first, or hypotonic, phase of PWS may be noted prenatally and is marked by decreased fetal movement, abnormal fetal position at delivery, and increased incidence of assisted delivery or cesarean section. Birth size is small to normal with possible low APGAR scores. Neonates will have some degree of hypotonia, poor suck, or even be considered a failure to thrive. Possible signs of PWS include dysmorphic facial features (small upturned nose, thin upper lip, downturned corners of the mouth), hypogonadism, respiratory issues, and delayed speech and motor development. Second phase (childhood, age 1 year to age 6 years). The second hyperphagic phase is characterized by weight gain, insatiable appetite, and high BMI. A child previously characterized as a poor eater or even a failure to thrive will begin to want to eat all the time.6 This change occurs at some point between age 1 year and age 6 years. Hypopigmentation, short stature, small hands and feet, early dental caries, strabismus, scoliosis,

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TABLE 2. Resources for family members and health-care providers Prader-Willi Syndrome Association (www.pwsausa.org) Provides links and resources regarding medical care, advocacy, research, and information on other PWS associations Foundation for Prader-Willi Research (www.fpwr.org) Established to eliminate the challenges of PWS through the advancement of research International Prader-Willi Syndrome Organisation (www.ipwso.org) A source of information regarding medical reports, conferences, support, dietary management, publications, and fundraising/donations Prader-Willi Alliance of New York (www.prader-willi.org) Provides lobbying, advocacy, and support for people with PWS living in New York state, organizes an annual conference, and promotes PWS Awareness Month (May) National Organization for Rare Disorders (www.rarediseases.org) Offers links for information on advocacy, news, and events concerning rare diseases and resources for patients/families, patient organizations, medical professionals. Genetics Home Reference (www.ghr.nlm.nih.gov/condition/ prader-willi-syndrome) Helpful questions and answers on PWS and a list of references

are all characteristic of PWS. When compared with children and adults who do not have PWS, persons with PWS are always hungry and plagued by a body that requires much fewer calories. Individuals with PWS burn less of their intake and are less physically active due to their hypotonia. Behavioral issues

Ritualistic and maladaptive behaviors are frequently associated with PWS. Rigid insistence on sameness, ordering or arranging items, hoarding, food preoccupation, and preferred routines are common.7-12 Emotional lability, temper tantrums, compulsive behavior, verbal or physical aggression, hyperactivity and impulsivity, or lethargy and hypoactivity are frequently described. An early onset of skin-picking, self-inflicted lesions, and repetitive scratching may develop. The prevalence rate of such behavior is 65% to 95%. This behavior is not necessarily attributable to an anxiety disorder but may be associated with mood swings.13 Care and treatment

Medical Home Portal (www.medicalhomeportal.org/diagnoses-andconditions/prader-willi-syndrome/resources) Information and support, tools, services, and studies for patients, parents, and providers Management of Prader-Willi Syndrome, a book edited by Merlin Butler, Phillip D.K. Lee, and Barbara Y. Whitman, is endorsed by the Prader-Willi Syndrome Association as a comprehensive resource and features contributions from professionals with expertise in the diagnosis and management of PWS.

developmental delay, maladaptive behaviors, and skin-picking may be noted during this phase. Third phase (adolescence and adulthood). Hyperphagia can continue throughout life. Diabetes and hypertension can result from uncontrolled weight gain. Scoliosis, osteoporosis, delayed sexual development, infertility, and psychiatric issues are often noted during the third phase of PWS.4,5 Adults with PWS historically have a shortened life span if obesity persists.5 Verbal skills may be strong but articulation may be a problem. Mild intellectual or learning disabilities may be seen. Strengths may be noted in reading, visual-spatial skills, vocabulary, or long-term memory. An interest and skill in doing jigsaw puzzles or word searches is common.4 Insatiable hunger and obesity

Insatiable hunger is an ongoing problem for a child with PWS and his or her caregiver. A low metabolic rate, decreased caloric requirements, inability to vomit, reduced physical activity, and a poor expenditure of caloric intake

PWS can be a difficult condition for a family to manage. Medical, educational, and behavioral interventions begin optimally in infancy and continue throughout life.5 Appropriate care and treatment requires a multidisciplinary approach involving collaboration with specialists from genetics, endocrinology, developmental-behavioral pediatrics, nutrition, psychology, special education, and nursing.4,14 It is imperative that all family members and caregivers be involved in all aspects of planning and be provided with sufficient information and support.14,15 Because of the obesity-related comorbidities, weight control should be the primary treatment focus for a child with PWS.14 Treatment recommendations include: • Early diagnosis and genetic counseling • Early intervention and input from a child-development team in the first five years of life, to include occupational therapy, physical therapy, speech therapy, special education, and nutrition • Family support and education • Ongoing pediatric dietary input to reduce and restrict caloric intake • Growth hormone therapy as indicated • Endocrine evaluations and management for hypogonadism, adrenal insufficiency, and diabetes • Intermittent psychology/psychiatry treatment, as required • Cooperation among providers, social services, and teachers to assist with management at home and in school.15 Continues on page 60

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Environmental modifications are important when treating PWS and may include locking kitchen cabinets and the refrigerator. Close supervision of money is required since older children may steal to secretly purchase food.4 Additional adjunctive treatments have included chiropractic care, aquatic physical therapy, mindfulness-based strategies, parental education, and occupational/play therapy.16-20 Nutrition and dietary control. The caloric-intake requirements of patients with PWS, which are typically 60% lower than those of individuals without the condition, must be managed. The goal is to keep the patient’s BMI below 30 and to increase physical activity.4 Professional recommendations for managing behavioral and food-related issues are widely available. Much less information is available on how parents and families actually cope with the recommendations and the strategies they employ.21,22 Research shows that parental focus is required to restrict access to food, keep the child engaged in activity, and establish routines for daily food management. Family and socioeconomic status may have an effect on the types of food-management approaches used.22 Growth hormone treatment. In 2000, the FDA approved the use of recombinant human growth hormone (rhGH) for children with PWS.23 This treatment can begin in infancy or at the time of diagnosis and helps to increase rates of growth, normalize height, decrease body fat, and increase muscle and bone mass. Growth hormone treatment also helps to control obesity to some degree and improve motor development. Few adverse effects of this therapy have been noted, and it should be considered an option for treatment when medically appropriate.4 In this therapy, parents administer a daily injection of somatropin to the child with PWS. Recent guidelines advise that rhGH therapy be employed in conjunction with dietary, environmental, and lifestyle interventions. Exclusion criteria should include severe obesity, uncontrolled diabetes mellitus, untreated severe obstructive sleep apnea, active cancer, or psychosis.24 Due to reports of possible increased risk of death, the FDA cautions that obese children should be evaluated for upper-airway obstruction and sleep apnea before initiating rhGH therapy.25 Behavior management. Parents and caregivers of children with PWS may need guidance on managing repetitive and rigid behaviors and habit-reversal training.13 Behavioral psychotherapy may help in dealing with associated obsessivecompulsive symptoms.9 Pharmacologic interventions include antidepressants, antipsychotics (risperidone [Risperdal]), and mood stabilizers.14,26 Caution must be exercised to minimize the risks of side effects, weight gain, toxicity, and suicide.14 Parents and caregivers face an ongoing struggle between controlling the behavior of a child diagnosed with PWS and

preserving his or her freedom and autonomy.22,27 Successful treatment requires an individualized management approach within the everyday context of the family.22 Family and parental needs

Education and support become paramount for the parents of a child with PWS. The disorder can have a significant impact on the entire family. Parents may become upset, angry, or frustrated with their child’s dishonesty about food intake. They may feel embarrassed by their child’s behavior and have difficulty effectively communicating with the child or with medical professionals.28 Parents have reported that weight- and behavioral-control issues are their greatest concerns and are a source of family stress.21 These parents may experience marital problems and lower parental consistency than do parents of children with other intellectual disabilities, including Down syndrome.28,29 Maternal stress appears to be greater than paternal stress due to the burden of care on the mother as the primary caregiver.30-32 Parents may experience a decreased locus of control and adaptability as well as pessimism or depression, low selfesteem, uncertainty, loss of control, or fears related to real or perceived consequences for their child. Support and professional counseling may assist parents in dealing with such feelings.28,30,31 Although definitive treatment strategies for PWS remain unclear, there is consensus as to the high need for information and continuous support for parents.13,28,30 The ability to understand treatment, therapeutic recommendations, and future needs—particularly those regarding behavioral management, education, and rehabilitation—is critical.13,28,33 Conclusion

Clinicians who are unfamiliar with the presentation of PWS or neglect to consider a rare or genetic etiology for childhood overweight and obesity may overlook the diagnosis. Missing the diagnosis can be detrimental to both the child and the family’s well-being. A child’s obesity, insatiable appetite, and potential developmental delays can be addressed with appropriate support services and interventions once the syndrome is recognized. Appropriate anticipatory guidance is integral to the child’s health and the family’s wellness. n Dr. Vitale is an associate professor in the Nursing Division at Molloy College in Rockville Centre, N.Y. Ms. Kearney is the Director of Community Education at South Nassau Communities Hospital in Oceanside, N.Y., and a PhD candidate in the Nursing Doctoral Program at Molloy College.

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Advisor Forum These are letters from practitioners around the country who want to share their clinical problems and successes, observations, and pearls with their colleagues. Responding consultants are identified below. We invite you to participate.

Inside the Forum JANUARY 2014

Consultations Folic acid during pregnancy. . . . . . . . 63 Do vaccines have to be administered intramuscularly?. . . . . . 63 Hepatitis C and eczema. . . . . . . . . . . 63 Nasal lubrication guidelines. . . . . . . . 64

Clinical Pearls Be specific when taking an adolescent’s sexual history. . . . . . . . .64 Blow away your pediatric patients. . . . 64 Go beyond TSH when testing for thyroid disease. . . . . . . . . . . . . . . . . 64

Your Comments More OTC relief for oral mucositis . . . . . . . . . . . . . . . . . 64

Send us your letters with questions and comments to: Advisor Forum, The Clinical Advisor, 114 West 26th Street, 4th Floor, New York, NY 10001. You may also fax (646) 638-6117, or contact us by e-mail at letters@ clinicaladvisor.com. If you are writing in response to a published letter, please indicate so by including the number in parentheses at the end of each item. Letters are edited for length and clarity. The Clinical Advisor’s policy is to print the author’s name with the letter. No anonymous contributions will be accepted.

CONSULTATIONS BISPHOSPHONATE TREATMENT FOR A FRAGILITY FRACTURE A woman, aged 58 years, suffered a fragility fracture when she slipped and fell on the ice, breaking her distal radius. By definition, this fracture classifies her as having osteoporosis. However, the results of a dual-energy x-ray absorptiometry are quite good. The woman’s femoral neck T-score is 1.0, and her L1-L4 T-score is -0.7. Is treatment with a bisphosphonate advised?—MICHELLE SPENCER, NP, Salt Lake City A fragility fracture is defined as a fracture resulting from a fall from a standing height or less. The Fracture Risk Assessment Tool (FRAX) (www.shef.ac.uk/FRAX) from the World Health Organization (WHO) may be useful in this case. This tool considers all risk factors for osteoporosis, such as smoking status, age, sex, family history, and previous fracture. FRAX also considers nationality and ethnicity when calculating risk. Using the FRAX tool and based on the above information—with the fracture and postmenopausal status as the woman’s only risks—and assuming Caucasian ethnicity, nonsmoking status, no family history of osteoporosis, no history of steroid use, no excessive alcohol intake, and average height and weight, this patient’s 10-year risk of a major osteoporosis-related fracture is 8.8% and risk of hip fracture is 0.1%. The WHO recommendations are to start treatment in cases of hip or vertebral fracture, T-score <-2.5 at the femoral neck or spine after excluding secondary causes, and a

OUR CONSULTANTS

Rebecca H. Bryan, APRN, CNP,

Eileen Campbell, MSN, CRNP,

Philip R. Cohen, MD,

is a lecturer in the Family Health NP Program, University of Pennsylvania School of Nursing, Philadelphia.

is associate program director, Family Health NP Program, University of Pennsylvania School of Nursing, Philadelphia.

is clinical associate professor of dermatology, University of Texas Medical Center, Houston.

Deborah L. Cross, MPH, CRNP, ANP-BC, is associate program

director, Gerontology NP Program, University of Pennsylvania School of Nursing, Philadelphia.

Maria Kidner, DNP, FNP-C,

is a nurse practitioner with Cheyenne Cardiology Associates in Cheyenne, Wyo.

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10-year probability of hip fracture >3% or 10-year probability of major osteoporosis-related fracture >20%. Based on these results, I would not recommend bisphosphonate treatment for this patient. I would assure adequate nutrition and caloric intake, maintenance of normal calcium and vitamin D levels, regular exercise, and rescreen bone density in one to two years.—Kathy Pereira, MSN, FNP-BC, assistant professor, co-coordinator, family nurse practitioner program, Duke University School of Nursing, Durham, N.C. (183-1)

FOLIC ACID DURING PREGNANCY Folic acid is frequently given to women of childbearing age who are taking antiepileptic drugs. During pregnancy, the dose of folic acid is often increased, depending on whether the woman is on an inducer medication. Dosing can vary between 1 and 4 mg of folic acid daily. Studies have suggested no benefit at a higher dose. Is there a recommended dose of folic acid in general and/or during pregnancy at this time?—SHARI COMBE, MPAS, PA-C, Salt Lake City Folic acid supplementation at least one month prior to conception and during early pregnancy is recommended to reduce the incidence of neural tubal defects (NTDs). For most women, the recommended daily dosage of folic acid is 400 µg to 800 µg; a higher dosage is advised for women with such risk factors as a history of a child with a NTD, pregestational diabetes, and use of certain antiepileptic drugs (i.e., valproic acid [Depakene, Depakote, Stavzor] and carbamazepine) that have been shown to decrease the concentration of folate. Although there are insufficient data to support a particular recommended dosage of folic acid in women on one of these epileptic medications, the American Congress of Obstetricians and Gynecologists recommends that all women at-risk for having a child with an NTD take 4 mg of folic acid per day. Until further notice, this recommendation seems to be a safe and reasonable option.—Mary Newberry, CNM, MSN (183-2)

Debra August King, PhD, PA,

is senior physician assistant at New York-Presbyterian Hospital, New York City.

Mary Newberry, CNM, MSN

provides well-woman gynecologic care as a midwife with Prima Medical Group, Greenbrae, Calif.

DO VACCINES HAVE TO BE ADMINISTERED INTRAMUSCULARLY? Why do vaccines have to be given intramuscularly? If nitroglycerin tablets are absorbed enough to have immediate effects on the heart, why can’t vaccines be given as droplets or transdermal patches?—CATHERINE PATNODE, ARNP, Yakima, Wash. Administration route depends on the pharmacokinetic properties of the medication. Many agents, when given orally, undergo extensive metabolism via the GI tract and hepatic portal circulation and are rendered inactive after this “first pass” effect. Insulin is a good example of this activity. Many oral preparations of insulin have been attempted, but all undergo such thorough inactivation in the GI tract that there is no bioavailable drug remaining. A parenteral route of administration avoids this effect. Other medications are given parenterally because of their caustic effects on the GI tract. In the case of some vaccines, the intramuscular route also provides a slow absorption (especially depending on whether the drug is suspended in fat or water) and release of the vaccine and vaccine action.—Kathy Pereira, MSN, FNP-BC, assistant professor, co-coordinator, family nurse practitioner program, Duke University School of Nursing, Durham, N.C. (183-3)

HEPATITIS C AND ECZEMA What is the etiology of neuropathic flexion deformities of the fingers and dry, scaling palms related to hepatitis C infection?—MARY CARTER, CANP, Penrose, Calif. Several dermatologic manifestations of hepatitis C infection have been described. These manifestations include pruritus, porphyria cutanea tarda, cryoglobulinemia and vasculitis, salivary gland lesions (lymphocytic capillaritis and lymphocytic sialadenitis), lichen planus,

Claire O’Connell, MPH, PA-C,

teaches in the PA Program at the New Jersey Medical School and Rutgers University, Piscataway, N.J.

Sherril Sego, FNP-C, DNP,

is a primary-care nurse practitioner at the Department of Veterans Affairs Medical Center in Kansas City, Mo.

Julee B.Waldrop, DNP,

is associate professor at the University of Central Florida (UCF), and practices pediatrics at the UCF Health Center.

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Advisor Forum CLINICAL PEARLS

BE SPECIFIC WHEN TAKING AN ADOLESCENT’S SEXUAL HISTORY Specific questions will elicit more useful information about a teenager’s sexual history. Instead of asking, “Do you use condoms?” ask, “For every 10 times that you have sex, how many times would you say that you use a condom?” A more detailed question will provide a better understanding of the patient’s contraceptive risk-taking behavior and result in more effective counseling.—SUSAN COOKSEY, NP, Yacolt, Wash. (183-6) Lichen planus (shown) and other dermatologic conditions have been found to be associated with hepatitis C infection.

polyarteritis nodosa, urticaria, erythema nodosum, and erythema multiforme (Int J Dermatol. 1997;36:251-254). Combination therapy with interferon alpha-2b plus ribavirin for 24 to 48 hours has been used as initial treatment for chronic hepatitis C infection. Either agent, when used individually, can cause dermatologic side effects. Several reports of eczema have been described in patients with hepatitis C infection who were treated with interferon alpha2b and ribavirin (Arch Dermatol. 2004;140:215-217; available at archderm.jamanetwork.com/article.aspx?articleid=480239, accessed December 15, 2013). A literature search does not reveal any citations for the symptoms described by Ms. Carter.—Philip R. Cohen, MD (183-4)

NASAL LUBRICATION GUIDELINES Many individuals use petroleum jelly to lubricate the nose and prevent epistaxis caused by dry air. In patients who also use an intranasal steroid for seasonal allergies, will the petroleum jelly interfere with steroid absorption? If the steroid is instilled first, how long should the patient wait before applying the petroleum jelly?—JILL PURTEE, CPNP, Taos, N.M. Even though use of intranasal petroleum jelly is common, it is not recommended. The heaviness of the base does not allow normal secretion and/or absorption of fluids or medications. Vitamin E oil is a much more appropriate and equally available remedy. This lighter nonpetroleum lubricant has a natural emollient effect. Concomitant use of intranasal sprays is more effective since absorption is less inhibited. For a non-oil-based lubricant, such OTC products as Ayr Saline Nasal Gel are also effective.—Sherril Sego, FNP-C, DNP (183-5)

BLOW AWAY YOUR PEDIATRIC PATIENTS When performing the respiratory portion of a pediatric examination, tear off a sliver of table paper approximately 1 in × 5 in. Dangle the paper a few inches in front of your face, and ask the child to blow just enough to make the paper barely move. This technique gives the child something to focus while you to listen to his or her chest.—JANIS MANDAC-DY, CPNP, San Francisco (183-7) GO BEYOND TSH WHEN TESTING FOR THYROID DISEASE Evaluating for thyroid disease requires measuring thyroidstimulating hormone (TSH), free thyroxine (T4), and free triiodothyronine (T3). Looking at just the TSH will not give you the whole story. Normal TSH and free T4 combined with elevated free T3 may indicate hyperthyroidism or thyrotoxicosis.—PATRICIA SCHROEDER, APRN, FNP-C, Abilene, Tex. (183-8)

YOUR COMMENTS MORE OTC RELIEF FOR ORAL MUCOSITIS I really enjoyed the pearl about using ice chips to treat oraltissue swelling caused by chemotherapy or radiation (Item 182-9). I also like to suggest using OTC Peroxyl mouth-sore rinse to clean the lesions, even in patients with stomatitis. After rinsing, the patient can apply topical oral pain reliever paste with benzocaine (Orajel). This will form a protective barrier over the lesion that will prevent irritation caused by food or drink. This paste can be reapplied frequently and is safe for children (per the label directions).—LETHA COLQUITT, FNP-BC, Texarkana, Tex. (183-9) n

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Download the new app for the iPhone, iPad,and Android— created specifically for nurse practitioners and physician assistants from the publishers of the highest rated journal for these health-care professionals. With the Clinical Advisor app you can: • Take Derm Dx quizzes to learn about difficultto-identify dermatology conditions, and then see how you performed against your peers. • Use medical calculators to do things like assess liver function, convert HbA1C to mean plasma glucose, evaluate BP, determine BMI and more. • Read the latest news about breakthrough treatments, disease outbreaks, drug approvals and recalls, and clinical research.

• Use the medical slideshows to educate patients in-office about clinically relevant topics, including the detrimental effects of smoking, the benefits of breastfeeding, diabetes complications and healthy lifestyle tips, etc. • Access hundreds of NP- and PA-specific accredited courses from the myCME education library and claim your certificate instantly. • Search the NPPR/PAPR drug database

The best part? IT’S FREE! So don’t wait. Download the Clinical Advisor app today to start experiencing the benefits of this essential resource at the point-of-care.

Derm Dx

EXCLUSIVE TO THE WEB

INTERACT WITH YOUR PEERS by viewing the images and offering your diagnosis and comments. To post your answer, obtain more clues, or view similar cases, visit CliniAd.com/10KIbCF. Learn more about diagnosing and treating these conditions, and see how you compare with your fellow colleagues.

A rash flares after discontinuing prednisone A woman, aged 48 years, had initially presented with a milder rash several weeks earlier and had been prescribed a prednisone taper. Two days after she stopped taking the medication, she developed a more severe rash. WHAT IS YOUR DIAGNOSIS?

• Stevens-Johnson syndrome • Generalized pustular psoriasis • Toxic epidermal necrolysis • Necrolytic migratory erythema

● See the full case at CliniAd.com/1cer0UL

Progressive yellow skin changes A 63-year-old man with monoclonal gammopathy of undetermined significance developed yellow patches of skin on every surface of his body. The patches had worsened over the past three years. WHAT IS YOUR DIAGNOSIS?

• Normolipemic plane xanthoma • Tuberous xanthoma • Tendinous xanthoma • Eruptive xanthoma ● See the full case at CliniAd.com/1f4tDHh

Have you missed any recent Derm Dx cases? Go to CliniAd.com/10KIbCF for a complete archive of past quizzes as well as additional images of last month’s other cases.

Circular areas of erythema

A “warty” patch on the scalp

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LEGAL ADVISOR CASE

Hospitals take privacy seriously

BY ANN W. LATNER, JD

Ms. B was a nurse practitioner working in a hospital obstetrics/gynecology department. It was her first job out of school, and she’d been working there for seven years. She liked her work well enough, and best of all, over the past two years, she had been able pay off her student loans and purchase her first home. Her reviews from her supervisors were all generally positive, and Ms. B felt that her life was finally beginning. Ms. B’s feelings about her job all changed one morning when she was called into her supervisor’s office. The supervisor looked unusually grim, and the director of human resources was there as well. Ms. B was asked to take a seat. “As you know,” began the supervisor, “we recently conducted a random audit of our computer system to determine if there have been any HIPAA violations. We take patient privacy very seriously here as you know.You have been working here about seven years, right?” Ms. B nodded. “Well, then,” the supervisor continued, “you already know how seriously we take HIPAA, because you must have seen the mandatory

A clinician is terminated from her job for accessing patient information that was beyond her purview.

By accident, Ms. B accessed the records of people who were not her patients, but she immediately closed the computer screen when she realized her mistake.

multimedia presentation.We show it in the hospital about twice each year.” “I have seen that,” confirmed Ms. B. “Then you know that it’s a violation of HIPAA to access the records of people who are not your patients?” “Yes,” said Ms. B. “I am sorry to inform you that the hospital is terminating your employment immediately on the basis of your actions. We have discovered that you accessed the records of four elderly male patients whom you were not treating. In addition, we also discovered that you were accessing your own records last year. I’m sorry, but I need to ask you to gather your belongings so that you can be escorted out.” Ms. B immediately tried to explain that she occasionally had trouble using the computer system and had pulled up the records of the four Cases presented are based on actual occurrences. Names of participants and details have been changed. Cases are informational only; no specific legal advice is intended. Persons pictured are not the actual individuals mentioned in the article.

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LEGAL ADVISOR male patients accidentally, but had closed the files as soon as she realized her mistake, within a minute. “And my own records are mine!” protested Ms. B.“How am I violating anyone’s privacy by looking at my own records?” The supervisor told Ms. B that looking at her own records was a violation of the hospital policy and that she was supposed to request them the same as any other patient. Ms. B had never been fired from a job before, and fought the urge to cry. Before she knew it, she was standing outside her car with a half-filled cardboard box containing the few small items that represented seven years at that job.As the days went by, Ms. B’s initial shock and embarrassment at being fired turned to anger.

“No actual harm or risk of harm in exposing private information occurred when Ms. B consulted her own medical records.” “I don’t need them,” she told herself.“I was getting tired of working there anyway. I’d rather work in a physician’s office.” She began to apply for other positions, but found it was much more difficult to gett a job in the current economy. Meanwhile, the bill for her health insurance arrived, her mortgage payment had to be made each month, and bills had to be paid. Ms. B’s meager savings were soon gone, but she took comfort in knowing that at least she had applied for unemployment insurance benefits, and that should be starting soon. The situation went from bad to worse when Ms. B received a letter from the unemployment office rejecting her claim. According to the letter, she was ineligible for unemployment benefits because she had been discharged for “employment misconduct.” She appealed the ruling, but an unemployment law judge rejected the appeal. Months had now passed. Ms. B was applying for jobs, but rarely getting interviews. And when she did get an interview, she worried about who she might use as a reference from her old job. No employment offers were forthcoming, her mortgage was now in arrears, and Ms. B was seriously considering dropping her expensive COBRA insurance coverage. She finally called a local legal- services office that offered low-cost help to financially strapped people.There Ms. B met with an attorney who listened to her story and suggested that she contact the state appellate court. The Court of Appeals accepted the case, and papers were filed by both the hospital and Ms. B.

After reviewing all the motions, the Court of Appeals overturned the unemployment judge’s decision and ruled that Ms. B was entitled to collect unemployment benefits. Legal background

The Court of Appeals agreed with Ms. B that her accessing the records of the four patients was clearly inadvertent, as evidenced by the fact that she logged off as soon as she realized her error. The main point left for the court to decide was whether accessing one’s own records was a violation of HIPAA. The court noted that Ms. B accessed her records three times during a one-week period when she was trying to reach her supervisor about a laboratory result. The court also noted that the hospital’s multimedia HIPAA training presentation, which employees were required to view, did not include any information about looking up one’s own records, just the records of others. Even if the hospital had a policy against accessing one’s own records, and even if Ms. B knew about this policy (which she claimed she did not), the court held that Ms. B’s actions were not “employment misconduct sufficient to disqualify this long-term employee from receiving unemployment benefits.” “No actual harm or risk of harm in exposing private information occurred when Ms. B consulted her own medical records,” stated the court. “Ms. B’s consultation of her own medical records does not show a serious violation of the standards of behavior the employer has the right to reasonably expect of the employee that would meet the definition of employment misconduct.” The court declined to address the issue of whether Ms. B should have been fired over the hospital’s policy against accessing one’s own records, but ruled that she did not violate HIPAA and did not commit employment misconduct, and that she was entitled to unemployment benefits. Protecting yourself

Complying with HIPAA is very important, and employers take this rule very seriously. Practitioners have been fired for accessing the records of relatives, friends, and even celebrities who were not their patients.Yet, mistakes can happen. If you work in the same place where you receive healthcare services, it is not a HIPAA violation to view your own records, but it may be a violation of your employer’s policy. Before you access any records that are not those of a patient you are currently treating, you should find out whether there is a protocol in place for this. n Ms. Latner, a former criminal defense attorney, is a freelance medical writer in Port Washington, N.Y.

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CME CE

Dermatology Clinic n LEARNING OBJECTIVE: To identify and diagnose dermatologic conditions and review up-to-date treatment. n COMPLETE THE POSTTEST: Page 83

n ADDITIONAL CME/CE: Pages 56, 79

Turn to page 55 for additional information on this month’s CME/CE courses.

CASE #1

Painless brown macules on the lips and fingers AUDREY CHAN, MD, AND WILLIAM LONGHURST

A young woman, aged 18 years, presented for evaluation of “moles” that developed on her lips in early childhood. No pain or pruritus associated with these lesions was reported. Medical history was significant for removal of polyps in the patient’s colon. Review of systems was negative for fever, chills, and weight loss. No family history of similar mucocutaneous lesions was reported. Physical exam was notable for numerous 2-mm to 3-mm brown macules scattered over the upper and lower cutaneous and mucosal lips; similar lesions were found on the woman’s fingers. No longitudinal melanonychia, nail dystrophy, or alopecia was noted. What is your diagnosis? Turn to page 72

CASE #2

Growing nasal lesion with asymmetry ESTHER STERN, NP-C

A man, aged 67 years, presented for a full skin examination. No personal history of skin cancer or family history of malignant melanoma was reported. As a teenager and young adult the man worked as a lifeguard and used no sun protection. For the past 20 years, he has worked as a contractor, spending most of the day outdoors without any sun protection. The patient noted that a brown spot on his nose had been slowly getting larger over the past 10 years. Physical exam revealed a 9-mm × 11-mm brown patch on the right nasal dorsum with asymmetry, uneven borders, and color variation. What is your diagnosis? Turn to page 74 www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • JANUARY 2014 71

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CME CE

CASE #1

Dermatology Clinic

Peutz-Jeghers syndrome

Peutz-Jeghers syndrome is a genodermatosis characterized by mucocutaneous pigmented lesions, GI polyposis, and an increased risk of cancer. Peutz-Jeghers syndrome is inherited in an autosomaldominant fashion; however up to 25% of cases are sporadic.1 The incidence is estimated to be approximately 1 in 8,300 to 1 in 200,000 live births.1 There are no reported variations based on ethnicity. Given the autosomal-dominant inheritance, males and females are equally affected. The majority of cases of Peutz-Jeghers syndrome are caused by mutations in the STK11/LKB1 gene on chromosome 19p13.13. This gene encodes a serine/threonine protein kinase that is thought to act as a tumor suppressor. This mutation is found in 52% to 70% of cases.1 The exact mechanisms by which the loss of STK11/LKB1 activity triggers the cutaneous findings and increased risk of cancer is unknown.2 This serine/threonine kinase may be involved in transduction of intracellular growth signals and in Wnt signaling.1 It has also been hypothesized that STK11/LKB1 regulates p53-mediated apoptotic pathways and regulates cell proliferation via G1 cell-cycle arrest and WAF1 signaling.3,4 Mutations in STK11/LKB1 gene may also alter cell polarity, which is thought to play a role in tumor formation.3 Brown macules on the lips and oral mucosa are the most characteristic cutaneous findings of Peutz-Jeghers syndrome. The lower lip is most commonly affected. These macules tend to develop in infancy or early childhood. In terms of the oral mucosa, the buccal mucosa, tongue, or gingiva may be affected. Similar lesions may also be found on the genital mucosa. Up to 95% of patients may have perioral lesions. Two-thirds of patients may have lesions on the hands and feet, especially the fingers, toes, dorsal hands, and dorsal feet.5 GI polyposis most commonly affects the small intestine and is involved in 64% of patients. These polyps also commonly affect the stomach and colon in approximately 49% of patients. The rectum is only involved in one-third of patients.5 The median time of first presentation with GI polyposis is age 11 years to age 13 years.6 Presenting signs include abdominal pain, vomiting, GI bleeding, and intussusception.5

It has been estimated that Peutz-Jeghers syndrome confers a 15-fold greater lifetime risk of GI and non-GI cancers compared with the general population.5,7 The increased lifetime risk of GI cancer is as follows: colon (39%), pancreas (36%), stomach (29%), small intestine (13%), and esophagus (0.5%).8 Persons with Peutz-Jeghers syndrome have an increased lifetime risk of breast cancer (54%), lung cancer (15%), ovarian cancer (21%), and uterine cancer (9%).8 Breast cancer can be bilateral.8 Cervical and thyroid cancers have also been reported in the literature. Sertoli-Leydig cell stromal tumors can occur in males, and sex-cord tumors with annular tubules (SCAT) can occur in female patients with Peutz-Jeghers syndrome.5 This latter finding is considered to be a classic feature of Peutz-Jeghers syndrome, as 36% of women who are diagnosed with SCAT are found to have Peutz-Jeghers syndrome.9 Even in patients without the STK11/LKB1 mutation, there is a 40% risk of cholangiocarcinoma.5 The diagnosis of Peutz-Jeghers syndrome is made clinically by the presence of two or more of the following features: (1) two or more Peutz-Jeghers polyps of the small intestine characterized by a distinctive pattern of arborization of the muscularis mucosa; (2) characteristic pigmentation of the mouth, lips, nose, eyes, genitalia, or fingers; and (3) family history of Peutz-Jeghers syndrome.9 The differential diagnosis of Peutz-Jeghers syndrome includes inherited patterned lentiginosis, Laugier-Hunziker syndrome, and Cronkhite-Canada syndrome. Inherited pattern lentiginosis was first described in 10 patients of African-American heritage.10 More recently, however, familial generalized lentiginosis was described in a fourgeneration Chinese family.11 Unlike Peutz-Jeghers syndrome, there are no internal abnormalities in inherited pattern lentiginosis. Similar to Peutz-Jeghers syndrome, Laugier-Hunziker syndrome is characterized by macular hyperpigmentation of the oral mucosa, including the lips. However, the hyperpigmented macules on the oral mucosa in LaugierHunziker syndrome develop in the early- to mid-adult years, which is a much later onset than that seen in Peutz-Jeghers syndrome.12 A distinctive feature of Laugier-Hunziker syndrome is longitudinal melanonychia that can occur on the fingernails, toenails, or both.12 In contrast to Peutz-Jeghers syndrome, patients with Laugier-Hunziker syndrome lack the pigmented macules on the hands and feet and also do not develop GI polyposis.12 Cronkhite-Canada syndrome is a noninherited condition also characterized by hyperpigmentation and GI polyposis.

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Although both Cronkhite-Canada syndrome and PeutzJeghers syndrome can present with brown macules with a predilection for the lips, face, oral mucosa, hands, and feet, Cronkhite-Canda syndrome has distinctive cutaneous features of diffuse hyperpigmentation, discoloration and dystrophy of the fingernails and toenails, and alopecia. Other possible skin findings include xanthelasma and papillary atrophy of the tongue. Cronkhite-Canada syndrome is also characterized by edema, muscle wasting, ocular manifestations (streaks of tan pigment in the retina, cataracts), and acute psychotic symptoms.13,14 The treatment of mucocutaneous pigmentation associated with Peutz-Jeghers syndrome is only necessary if the lesions are cosmetically distressing to the patient. Surgical excision, cryosurgery, electrodesiccation, dermabrasion, and ablative laser (CO2 or argon) therapy are not recommended due to the risks of incomplete removal, scarring, and dyspigmentation.15 Successful treatment of mucocutaneous lentigines has been reported with potassium titanyl phosphate, intense pulsed light, ruby, Q-switched ruby, and Q-switched alexandrite lasers.15-18 GI polyposis may be treated with laparotomy, polypectomy, and/or bowel resection. Colectomy may be considered for recurrent or unresectable polyps.19 Because of the high risk of cancer, the following screening guidelines have been recommended for all patients with Peutz-Jeghers syndrome: (1) upper endoscopy, colonoscopy, and abdominal CT with oral contrast every two to three years beginning at age 10 years; and (2) magnetic resonance cholangiopancreatography and/or endoscopic ultrasound every one to two years by age 30 years. Female patients are advised to undergo: (1) annual mammogram and breast MRI with clinical breast exam every six months beginning by age 25 years; and (2) annual pelvic examination, pelvic ultrasound, and Pap smear by age 20 years. Male patients are advised to undergo annular testicular exam and observation for feminizing changes by age 10 years.9 Unfortunately, the patient described in this case was lost to follow-up. n

2. Trojan J, Brieger A, Raedle J, et al. Peutz-Jeghers syndrome: molecular analysis of a three-generation kindred with a novel defect in the serine threonine kinase gene STK11. Am J Gastroenterol. 1999;94:257-261. 3. Heymann WR. Peutz-Jeghers syndrome. J Am Acad Dermatol. 2007;57:513-514. 4. McGarrity TJ, Amos C. Peutz-Jeghers syndrome: clinicopathology and molecular alterations. Cell Mol Life Sci. 2006;63:2135-2144. 5. James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, Pa.: Saunders Elsevier; 2011:851. 6. McGarrity TJ, Kulin HE, Zaino RJ. Peutz-Jeghers syndrome. Am J Gastroenterol. 2000;95:596-604. 7. Taheri D, Afshar-Moghadam N, Mahzoni P, et al. Cancer problem in Peutz-Jeghers syndrome. Adv Biomed Res. 2013;2:35. Available at www. ncbi.nlm.nih.gov/pmc/articles/PMC3748667/. 8. Giardiello FM, Brensinger JD, Tersmette AC, et al. Very high risk of cancer in familial Peutz-Jeghers syndrome. Gastroenterology. 2000;119:1447-1453. 9. Gammon A, Jasperson K, Kohlmann W, Burt RW. Hamartomatous polyposis syndromes. Best Pract Res Clin Gastroenterol. 2009;23:219-231. Available at www.ncbi.nlm.nih.gov/pmc/articles/PMC2678968/. 10. O’Neill JF, James WD. Inherited patterned lentiginosis in blacks. Arch Dermatol. 1989;125:1231-1235. 11. Xing Q, Chen X, Wang M, et al. A locus for familial generalized lentiginosis without systemic involvement maps to chromosome 4q21.1-q22.3. Hum Genet. 2005;117:154-159. 12. Koch SE, LeBoit PE, Odom RB. Laugier-Hunziker syndrome. J Am Acad Dermatol. 1987;16(2 Pt 2):431-434. 13. Bruce A, Ng CS, Wolfsen HC, et al. Cutaneous clues to CronkhiteCanada syndrome: a case report. Arch Dermatol. 1999;135:212. 14. Medscape. Cronkhite-Canada syndrome clinical presentation. Available at emedicine.medscape.com/article/1096789. 15. Zaheri S, Chong SK, Harland CC. Treatment of mucocutaneous pigmentation in Peutz-Jeghers syndrome with potassium titanyl phosphate (KTP) laser. Clin Exp Dermatol. 2005;30:710-712. 16. Remington BK, Remington TK. Treatment of facial lentigines in PeutzJeghers syndrome with an intense pulsed light source. Dermatol Surg. 2002;28:1079-1081. 17. Ohshiro T, Maruyama Y, Nakajima H, Mima M. Treatment of pigmentation of the lips and oral mucosa in Peutz-Jeghers’ syndrome using ruby and

Dr. Chan is a third-year dermatology resident at Baylor College of Medicine in Houston. Mr. Longhurst is a fourth-year student at the University of North Dakota School of Medicine & Health Sciences in Grand Forks.

argon lasers. Br J Plast Surg. 1980;33:346-349. 18. DePadova-Elder SM, Milgraum SS. Q-switched ruby laser treatment of labial lentigines in Peutz-Jeghers syndrome. J Dermatol Surg Oncol. 1994;20:830-832. 19. Dong K, Li B. Peutz-Jeghers syndrome: case reports and update on

References

diagnosis and treatment. Chin J Dig Dis. 2004;5:160-164.

1. Schachner LA, Hansen RC, eds. Pediatric Dermatology. 4th ed. Philadelphia, Pa.: Mosby Elsevier; 2011:493-495.

All electronic documents accessed December 15, 2013.

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CME CE

CASE #2

Dermatology Clinic

Lentigo maligna

Dermoscopic examination of the man’s nasal lesion revealed an asymmetric reticular pigment network with gray circles, as well as varying perifollicular hyperpigmentation. A shave biopsy was performed, and histologic exam confirmed the suspected diagnosis of lentigo maligna, also known as lentiginous melanoma on sun-damaged skin or Hutchinson melanotic freckle. Lentigo maligna is a precursor to lentigo maligna melanoma, a form of melanoma unique to older individuals with heavily sun-damaged skin that appears specifically in areas of chronic sun exposure. Physical characteristics of lentigo maligna include:1 • Large size (typically >6 mm and often several cm in diameter at the time of diagnosis); • Irregular borders; • Varied pigmentation (may include light brown, tan, dark brown, pink, red, or white); • Smooth surface. After five to 20 years of peripheral growth, if undetected and untreated, the lentigo maligna lesion may grow vertically, and an invasive melanoma develops. In rare cases, transformation to invasive melanoma can occur more rapidly, even over the course of only a few months. Persons most at risk for lentigo maligna are those with a history of excess UV radiation exposure and signs of actinic damage. Incidence peaks between age 65 years and age 80 years. Persons with fair skin, multiple melanocytic nevi, a history of severe sunburn, occupational exposure to sun, and a family history of melanoma are at heightened risk. The incidence of lentigo maligna is highest in Hawaii, whereas incidence of melanoma is highest among those who have lived in Australia for many years. Lentigo maligna typically starts as a tan, brown, or black macule that slowly expands peripherally over the course of many years. As it progresses, the lesion demonstrates more atypical features, such as uneven pigmentation and irregular borders. The most commons sites of lentigo maligna are the head and neck. Interestingly, lentigo maligna is more commonly seen on the left side of patients who have spent a great deal of time driving, and it appears more commonly on the right side in those who were most often passengers in the front seat.

Physical examination of suspicious lesions should include use of the ABCDE rule.2 Inspect for Asymmetry, irregular Border, Color variegation, and larger Diameter, and inquire about history of Enlargement. Clinical diagnosis of lentigo is typically challenging, as lesions are frequently buried among benign lentigines, nevi, actinic keratoses, and seborrheic keratoses. Dermoscopy may aid in the diagnosis of lentigo maligna and decrease unnecessary biopsies.3 Four criteria often noted in lesions of lentigo maligna are asymmetric pigmented follicular openings, rhomboidal structures, annular-granular structures, and gray pseudo-network.4 Biopsy of the suspicious lesion is necessary to confirm the diagnosis. Although excisional biopsy is generally preferred for suspected melanomas, because suspected lentigo maligna lesions are usually on the face, results are often cosmetically unacceptable. Therefore, a deeper incisional shave biopsy is reasonable. Studies have shown that incisional biopsies have no negative effect on mortality.5 Beyond biopsy and full skin examination, such additional workup as x-ray and lymph-node biopsy is usually reserved for patients with invasive melanomas. Early surgical excision is the preferred treatment of lentigo maligna. For in situ melanomas, the National Comprehensive Cancer Network practice guidelines recommend a margin of 0.5 cm.6 However, in lentigo maligna melanoma, subclinical extension of the in situ tumor often extends beyond 0.5cm, with disproportionate expansion being common. In such scenarios, Mohs micrographic surgery or a staged excision may be preferred and appear to offer higher cure rates.7 Staged excision involves initial excision with narrower margins, often guided by the use of a Wood’s lamp. Permanent sections are then examined by a dermatopathologist, usually within 24 hours. Further excisions are performed based on the histologic findings. In patients who are poor surgical candidates, other treatment considerations may include the immune response modifier imiquimod (Aldara, Zyclara), cryotherapy, or radiation.8,9 None of these options are typically ideal as solo treatment. Close evaluation of patients undergoing imiquimod therapy is essential and should include posttherapy histopathologic investigation.10 Prognosis for patients with lentigo maligna melanoma is similar to prognosis for patients with malignant melanoma in situ (i.e., 95%-100% five-year survival rate).11 Patients with a history of LM should be educated to perform monthly skin and lymph-node self-examinations. Any new or changing lesions should be evaluated by a

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dermatology provider. Clinical full-skin examinations should be performed every six months. Patients should be reminded to practice strict sun protection with the use of sunscreens and protective clothing. The man describe in this case underwent a shave biopsy of the suspicious lesion on his nose. Hematoxylin and eosin stains confirmed the diagnosis of lentigo maligna. The patient was referred to a surgical oncologist for staged excision of the lesion. After the margins were deemed clear of lentigo maligna melanoma, the defect was closed by a plastic surgeon. Three months later, the patient returned with a wonderful cosmetic outcome and no evidence of recurrence of the previous lentigo maligna. A full-skin examination revealed no suspicious lesions elsewhere. n Ms. Stern is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J.

“Remember—we’re not begging. We’re crowdfunding.”

References 1. Kallini JR, Jain SK, Khachemoune A. Lentigo maligna: review of salient characteristics and management. Am J Clin Dermatol. 2013;14:473-480. 2. Shenenberger DW. Cutaneous malignant melanoma: a primary care perspective. Am Fam Physician. 2012;85:161-168. Available at www.aafp. org/afp/2012/0115/p161.html. 3. Stante M, Giorgi V, Stanganelli I, et al. Dermoscopy for early detection of facial lentigo maligna. Br J Dermatol. 2005;152:361-364. 4. Tanaka M, Sawada M, Kobayashi K. Key points in dermoscopic differentiation between lentigo maligna and solar lentigo. J Dermatol. 2011;38:53-58. 5. Bong JL, Herd RM, Hunter JA. Incisional biopsy and melanoma prognosis. J Am Acad Dermatol. 2002;46:690-694. 6. Coit DG, Andtbacka R, Bichakjian CK, et al. Melanoma. J Natl Compr Canc

Netw. 2009;7:250-275. Available at www.jnccn.org/content/7/3/250.long. 7. Clark GS, Pappas-Politis EC, Cherpelis BS, et al. Surgical management of melanoma in situ on chronically sun-damaged skin. Cancer Control. 2008;15:216-224. 8. Wong JG, Toole JW, Demers AA, et al. Topical 5% imiquimod in the treatment of lentigo maligna. J Cutan Med Surg. 2012;16:245-249. 9. McLeod M, Choudhary S, Giannakakis G, Nouri K. Surgical treatments for lentigo maligna: a review. Dermatol Surg. 2011;37:1210-1228. 10. Wolf IH, Cerroni L, Kodama K, Kerl H. Treatment of lentigo maligna (melanoma in situ) with the immune response modifier imiquimod. Arch Dermatol. 2005;141:510-514. Available at archderm.jamanetwork.com /article.aspx?articleid=393575. 11. Koh HK, Michalik E, Sober AJ, et al. Lentigo maligna melanoma has no better prognosis than other types of melanoma. J Clin Oncol. 1984;2:994-1001. All electronic documents accessed December 15, 2013.

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ALTERNATIVE MEDS UPDATE

What you should know about the herbs and supplements patients use

By Sherril Sego, FNP-C, DNP. Ms. Sego is a staff clinician at the VA Hospital in Kansas City, Mo., where she practices adult medicine and women’s health. She also teaches at the nursing schools of the University of Missouri and the University of Kansas.

Bovine colostrum

Colostrum contains high concentrations of leukocytes (shown here), which destroy bacteria.

Background Bovine colostrum is particularly rich in immunoglobulin A (IgA) and the antimicrobial peptides lactoferrin and lactoperoxidase.2 Before the discovery of penicillin, bovine colostrum was used to treat various infectious processes in humans.2 While human colostrum is rich in nutrients, bovine colostrum contains nearly three times the energy and fat and five times the protein.2 Bovine colostrum is being studied as a woundhealing agent, an antimicrobial, and an anticancer therapy.2

Science Bovine colostrum appears to be especially useful in inflammatory conditions and gastric infections. In a small clinical study to determine the extent of immune response in humans when supplemented with this agent, 18 healthy volunteers were randomized to either bovine colostrum or placebo and then inoculated with an attenuated Salmonella typhi vaccine.3

Students of both neonatology and veterinary science are well aware of the powerful nutritional components of colostrum. Defined as the first milk produced by, in this context, a lactating dairy cow in the immediate period after the birth of a calf, colostrum is “super-rich” in multiple immunoglobulins that protect the neonate from environmental pathogens.1 Production of this antibody-dense substance, also known as “foremilk,” confers passive immunity on the newborn.2 The lactating mother will generate this milk for two to four days after delivery.2

After eight days, serum studies were conducted and analyzed for immune globulin concentrations. When compared with baseline, participants in the bovine colostrum group showed a 150% increase in IgA and a 246% increase in IgG.3 In a study of adults suffering from ulcerative colitis, participants with mild to severe distal colitis were randomized to either placebo or bovine colostrum for a four-week period.4 Results were based on self-report of disease symptoms based on the validated Powell-Tuck scoring tool and histologic mucosal changes obtained during sigmoidoscopy. At the end of the one-month period, participants randomized to the colostrum product showed a mean reduction in symptoms of nearly three points whereas the placebo participants experienced an increase in symptoms of 0.5 points. Histology findings demonstrated a greater than 50% reduction in mucosal damage in the colostrum group and no change with placebo. Bovine colostrum has also been studied for its effect on two other common enteric pathogens, Clostridium difficile and Escherichia coli. C. difficile infections are common but have lead

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KEYNOTE SPEAKER:

Ron Culberson, MSW, CSP Speaker, Author, Humorist

Do it Well. Make it Fun. Keystone Conference Center Keystone, Colorado  Highest quality speakers  Economical CE rates  Fantastic Rocky Mountain setting Offering over 30 hours of CE for Nurse Practitioners, Nurse Midwives and Physician Assistants through ACCME, ANCC and AANP, with acceptance of pertinent content by ACNM, AAPA and NAPNAP.

“I LOVE this conference! I have been to many conferences and this one has a wonderful balance of topics and great speakers. The location is beautiful and relaxing, just what I needed. I did not want to leave.” —Previous NNPS attendee

ALTERNATIVE MEDS UPDATE to significant morbidity and mortality, with death rates approaching 17% and hospitalization stays increased by nearly four days.5 With the significant increases in IgM and IgA found with colostrum use, researchers have noted a toxin-neutralizing activity against this bacterium. Bovine colostrum has been studied as a potential adjunctive therapy for victims of E. coli. In calves, the method of action of bovine colostrum against such bacteria appears to be multifaceted.6 Through its immune-enhancing actions, colostrum inhibits the ability of E. coli to attach to the mucosal lining of the intestine, thereby rendering the bacterium unable to lyse and release its deadly endotoxin. Finally, colostrum has also been studied as a preventive agent for upper-respiratory-tract infections (URTIs). Having determined that respiratory tract mucosal IgA concentrations play a large role in susceptibility to infection, researchers examined self-report diaries of 174 healthy men.7 These participants had been randomized to daily consumption of either bovine colostrum or placebo and recorded daily symptoms scores typically associated with URTI.At the end of eight weeks, the treatment group reported 16% fewer URTI symptoms than did the placebo group; duration of symptoms did not change.

Safety, interactions

colostrum when performed commercially.9 A one-month supply of capsules costs about $50.

Summary For patients suffering from acute infectious emergencies or those with other forms of immune weaknesses, bovine colostrum seems to be worth its cost.With no known interactions, colostrum can be recommended without fear of hidden dangers when used with other treatments. n Bovine colostrum is thought to help fight E. coli (above) infections.

Bovine colostrum appears to be especially useful in inflammatory conditions and gatric infections.

References 1. Steele J, Sponseller J, Schmidt D, et al. Hyperimmune bovine colostrum for treatment of GI infections. Hum Vaccin Immunother. 2013;9:1565-1568. 2. Godhia ML, Patel N. Colostrum—its composition, benefits as a nutraceutical: a review. Curr Research Nutr Food Sci. 2013;1:37-47. 3. He F, Tummola E, Arvilommi H, Salminen S. Modulation of human humoral immune response through orally administered bovine colostrum. FEMS Immunol Med Microbiology. 2001;31:93-96. 4. Hedge DD, Strain JD, Heins JR, Farver DK. Use of the “nutraceutical,” bovine colostrum, for the treatment of distal colitis: results from an initial study. Aliment Pharmacol Ther. 2002;16:1917-1922. 5. Hedge D, Strain J, Heins J, Farver D. New advances in the treatment of Clostridium difficile infection (CDI). Ther Clin Risk Manag. 2008;4:949-964. Available at www.ncbi.

Bovine colostrum is very well tolerated, and only those with a known milk allergy should avoid its use. No herb or drug interactions have been reported. Use should be avoided in areas in which bovine spongiform encephalopathy (mad cow disease) has been reported.

How supplied, dose, cost

nlm.nih.gov/pmc/articles/PMC2621401/. 6. Vilte D, Larzabal M, Cataldi A, Mercado EC. Bovine colostrum contains immunoglobulin G antibodies against Intimin, EspA, and EspB and inhibits hemolytic activity mediated by the type three secretion system of attaching and effacing Escherichia coli. Clin Vaccine Immunol. 2008;15:1208-1213. 7. Brinkworth GD, Buckley JD. Concentrated bovine colostrum protein supplementation reduces the incidence of self-reported symptoms of upper respiratory tract infection in adult males. Eur J Nutr. 2003;42:228-232. 8. Colostrum page. Web MD website. Available at www.webmd.com/vitamins-supplements/ingredientmono -785-COLOSTRUM.aspx. 9. McMartin S, Godden S, Metzger L, et al. Heat treatment of bovine colostrum. I: effects of temperature on viscosity and immunoglobulin G level. J Dairy Sci. 2006;89:2110-2118. All electronic documents accessed December 15, 2013.

There are no well-established dosing guidelines for bovine colostrum. One source recommends 10 g to 20 g of colostrum daily for 10 days for treating infectious diarrhea.8 Colostrum supplements may be found in a variety of forms including powder-filled capsules and concentrated liquids. Pasteurization of all dairy products is recommended and has been shown to have little effect on the immune globulin content of 78 THE CLINICAL ADVISOR • JANUARY 2014 • www.ClinicalAdvisor.com

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CME CE

Dermatologic Look-Alikes n LEARNING OBJECTIVE: To distinguish and properly treat dermatologic conditions with similar presentations. n COMPLETE THE POSTTEST: Page 83

n ADDITIONAL CME/CE: Pages 56, 71

Turn to page 55 for additional information on this month’s CME/CE courses.

Asymptomatic scrotal lesions JOE MONROE, PA-C

CASE #1

CASE #2

A man, aged 21 years, was referred to dermatology for evaluation of scrotal lesions. The man first noted the asymptomatic lesions several months earlier. The lesions were confined to the scrotum but were of considerable concern to the patient, who was about to be married. The man’s primary-care clinician had diagnosed the lesions as warts and prescribed podophyllin, but the lesions continued to appear, and the medicaiton was extremely irritating to the scrotal skin. While awaiting his dermatology appointment, he tested negative for HIV.

A man, aged 60 years, had been unaware of the “red bumps” on his scrotum until his girlfriend noticed them and insisted that he see a dermatologist. The lesions were asymptomatic and gave no indication of their presence. Since the man was somewhat overweight, he could only see the area with the use of two mirrors or in the photograph his girlfriend took. The patient denied ever having had any other problems in this area, or skin problems in general, and he claimed to be otherwise healthy.

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CME CE

CASE #1

Dermatologic Look-Alikes

Molluscum contagiosum

Although somewhat uncommon on the scrotum, molluscum contagiosum (MC) is far from unknown in this area where, especially in young adults, it is considered to be sexually transmitted. The appearance of these smooth, firm, and centrally umbilicated papules is all but pathognomic for MC, which is caused by a member of the Poxviridae family.1 The condition was first described by Bateman in 1817, but the contagious nature of the lesions was first described by Paterson in 1841. Worldwide, the incidence appears to be steadily increasing, currently accounting for approximately 1% of all skin disorders diagnosed.2 The MC virus (MCV) only affects the epidermal layer, gaining access through minimal disruptions in the skin, eventuating in a firm, glassy, papule marked by a central caseous plug. The caseous plug is the result of the central accumulation of large hyaline bodies (molluscum bodies), which can be seen microscopically. The molluscum bodies account for the telltale umbilication, which can be highlighted by application of liquid nitrogen. Although MCV cannot be grown in cultures, restrictive endonuclear analysis of the viral genomic material has allowed the delineation of four types of the virus. MCV type I has proven to be the cause in more than 96% of clinical lesions; MCV type II is the culprit in 60% of MC cases in HIV patients; and MCV types III and IV are rare. No difference has been seen between the severity, morphology, or anatomic distribution of the lesions associated with each MCV type.3 Only slightly smaller than the smallest bacteria, MCV is one of the largest known viruses. The virus is thought to spread through direct contact with persons with MC and objects contaminated by persons with MC. There appears to be an association between MC and the use of swimming pools and participation in school athletics, particularly among boys. Susceptibility is increased markedly in children with atopic dermatitis, whose eczema is often worsened by the presence of mollusca.4 MC in the genital area of young, immunocompetent adults can be considered a sexually transmitted disease (STD). Another clinical form of MC has been noted

in immunocompromised adults and children, especially those positive for HIV, but also in those being treated with such medications as methotrexate (Rheumatrex, Trexall) and prednisone and the antirejection medications used in transplant patients. Typical incubation time for MC is two to seven weeks, but as much as six months’ latency has been described.5 The classic papules of MC are subject to autoinoculation and tend to form in linear patterns along lines of trauma. These lines of trauma can be accidental or caused by scratching, particularly in young children. Strictly speaking, this is not a true isomorphic response (i.e., the Koebner response) since this phenomenon only applies to such conditions as lichen planus or psoriasis, in which the cause is unknown. Occasionally, lesions will progress beyond local proliferation and become inflamed. In a fair imitation of bacterial infection, edema, erythema, and increased vascularity are caused by an infiltration of neutrophils, lymphocytes, and monocytes. These lesions almost invariably resolve without treatment, as do most MC lesions. Resolution can be frustratingly slow, however, varying from two months to as long as five years. This lengthy resolution is why one of the mainstays of treatment for this benign but vexing condition is patient/parent reassurance. The diagnosis of MC is usually straightforward; however, the lesions can be so small as to resemble a rash. MC that coexists with eczema can be difficult to diagnose unless the clinician remembers that these two conditions are frequent traveling companions, especially on the arms. Small, flat warts and lichen planus can do a fair imitation of MC, but more often than not, the elicitation of the umbilication with light application of liquid nitrogen confirms the diagnosis of MC. No single treatment has proven consistently effective. There is no FDA-approved topical medicine for treatment of molluscum, but existing treatment choices tend to fall into one of four categories.6,7 1. Benign neglect. Since MC is self-limited, not harmful, and rarely scars, and because almost all of the various treatments are problematic, patient/parent education is sufficient for many cases. 2. Direct lesional trauma. Curettage of individual lesions, for example, is highly effective, but may not be practical in younger children or in cases of multiple lesions. The same could be said for application of liquid nitrogen, or such blistering agents as cantharidin. 3. Antiviral treatment. The use of antiretrovirals in HIV patients with MC can result in improvement in the number and size of the lesions.

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4. Immune-response stimulators. These include topical imiquimod (Aldara, Zyclara), which increases the body’s immune response to viral infection. Many other types of treatments have been tried, including potassium hydroxide, adhesive tape, dinitrochlorobenzene, squaric acid, and green-tea extract. None of these therapies has been consistently effective, and most have resulted in irritation, blistering, and pain. The poxvirus responsible for MC is not strongly immunogenic,8 but the condition eventually resolves on its own. Complications are few but may include periocular lesions that can trigger a reactionary keratoconjunctivitis. MC lesions confined to the genital areas on young children may necessitate the consideration of possible sexual abuse.9 In this case, the patient’s lesions resolved following treatment with liquid nitrogen on three different occasions, each one month apart from the last. Patient education included information regarding potentially more serious STDs. Since the man’s lesions were on the scrotum, where condoms would be useless, he was urged to assume that he was contagious until several disease-free months had passed.

CASE #2

Angiokeratoma of Fordyce

John Addison Fordyce, who had seen the lesions on the scrotal floor of a 60-year-old man, first described angiokeratoma of Fordyce (AF) in 1896. Since that time, this condition has been widely recog n i zed a s com mon among older adults. AF can also appear on the labia majora, the inner thigh, the lower abdomen, and, occasionally, on the penile shaft.10 AF lesions are seldom painful but occasionally bleed. As in this case, the mere discovery of such lesions can cause significant anxiety. Misdiagnosis often leads to overly aggressive treatment, the results of which then become problematic in this sensitive area.11,12 The soft papules, ranging in size from 1 mm to 3 mm, represent ectatic and thin-walled vessels in the superficial dermis with overlying epidermal hyperplasia. Angiokeratoma is a broad term, describing various conditions of asymptomatic hyperkeratotic vascular disorders

that display a combination of superficial dermal vascular ectasia with overlying epidermal hyperkeratosis on histologic sectioning.13 The two forms of angiokeratoma described are localized and systemic. In addition to AF, other localized forms of angiokeratoma include the solitary papular angiokeratoma (usually located on the leg), a rare and congenital form (angiokeratoma circumscriptum naviforme) with multiple hyperkeratotic papules and plaques appearing unilaterally on the lower extremities and buttocks, and bilateral multiple angiokeratomas appearing on the dorsa of fingers and toes, termed Mibelli type.10 The most common systemic form of angiokeratoma is angiokeratoma corporis diffusum (ACD), also known as Fabry disease.14 ACD is a storage disease in which glycosphingolipids accumulate in the skin and viscera, manifesting on the skin and lips as widespread punctate telangiectatic vascular papules that resemble purpura. A few papules will demonstrate hyperkeratotic surfaces. The resulting accumulation of deposits of glycolipids eventually leads to cardiomyopathy, renal failure, and death in the fifth decade. ACD is caused by a deficiency of alpha-galactosidase A and inherited through an X-linked recessive route. Since the histologic findings are identical for each type of angiokeratoma, localized and systemic forms must be differentiated on clinical grounds. In individuals with Fabry disease, the presence of widespread multiple angiokeratomas, especially on the mucosal surface of the lower lip, can help with diagnosis. The cause of AF is unknown, but most potential explanations revolve around increases in venous pressure. However, no such connection has been substantiated.15 No epidemiologic studies of AF have been conducted, but the condition is considered common, particularly among men. Increasing age appears to be a factor, but AF has been reported in children.11,16 The differential diagnsis for AF includes pyogenic granuloma, squamous cell carcinoma, condyloma, and cherry angioma, among other conditions. Treatment of AF is seldom necessary. Electrodessication or laser treatment can be used in cases in which the lesion bleeds following trauma.2,17 The patient described in this case was satisfied with simply knowing the benign nature of the problem and declined any treatment. n Mr. Monroe specializes in dermatology at Dawkins Dermatology in Oklahoma City.

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CME CE

Dermatologic Look-Alikes

References 1. Randall CMH, Shisler JL, Molluscum contagiosum virus. Future Virology. 2013;8:561-573. Available at www.medscape.com/viewarticle/805709. 2. Medscape. Molluscum contagiosum. Available at emedicine.medscape. com/article/910570. 3. Scholz J, Rösen-Wolff A, Bugert J, et al. Epidemiology of molluscum contagiosum using genetic analysis of the viral DNA. J Med Virol. 1989;27:87-90. 4. Olsen JR, Gallacher J, Piguet V, Francis NA. Epidemiology of molluscum contagiosum in children: a systematic review. Fam Pract. 2013 Dec 2. [Epub ahead of print]. 5. Centers for Disease Control and Prevention. Clinical information: Molluscum contagiosum. Available at www.cdc.gov/ncidod/dvrd/ molluscum/clinical_overview.htm. 6. Smolinski KN, Yan AC. How and when to treat molluscum contagiosum and warts in children. Pediatr Ann. 2005;34:211-221. 7. National guideline for the management of molluscum contagiosum. Clinical Effectiveness Group (Association of Genitourinary Medicine and the Medical Society for the Study of Venereal Diseases). Sex Transm Infect. 1999;75 Suppl 1:S80-S81. 8. Konya J, Thompson CH. Molluscum contagiosum virus: antibody responses in persons with clinical lesions and seroepidemiology in a representative Australian population. J Infect Dis. 1999;179:701-704. Available at jid.oxfordjournals.org/content/179/3/701.long. 9. Hornor G. Ano-genital warts in children: Sexual abuse or not? J Pediatr Health Care. 2004;18:165-170. Available at www.jpedhc.org/article/ S0891-5245(04)00018-5 10. Medscape. Angiokeratoma of the scrotum. Available at emedicine. medscape.com/article/1056046. 11. Schiller PI, Itin PH. Angiokeratomas: an update. Dermatology. 1996;193:275-282.

“And this tattoo is an old English proverb.”

A case report on response to surgical treatment of varicocele. Acta Derm Venereol. 1970;50:221-224. 13. Yamazaki M, Hiruma M, Irie H, Ishibashi A. Angiokeratoma of the clitoris: a subtype of angiokeratoma vulvae. J Dermatol. 1992;19:553-555. 14. James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, Pa.: Saunders Elsevier; 2011:527-528. 15. Erkek E, Basar MM, Bagci Y, et al. Fordyce angiokeratomas as clues to local venous hypertension. Arch Dermatol. 2005;141:1325-1326. 16. Patrizi A, Neri I, Trevisi P, et al. Congenital angiokeratoma of Fordyce. J Eur Acad Dermatol Venereol. 1998;10:195-196. 17. Ozdemir M, Baysal I, Engin B, Ozdemir S. Treatment of angiokeratoma of Fordyce with long-pulse neodymium-doped yttrium aluminium garnet laser. Dermatol Surg. 2009;35:92-97. All electronic documents accessed December 15, 2013.

“I can’t protect you from everything, but I can read you stories that make you believe I can protect you from everything.”

12. Agger P, Osmundsen PE. Angiokeratoma of the scrotum (Fordyce).

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POSTTEST Expiration date: January 2015

Physician Credit This activity has been planned and implemented in accordance with the Essential Areas and Policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of Medical Education Resources (MER) and Haymarket Medical. MER is accredited by the ACCME to provide continuing medical education for physicians. Credit Designation Medical Education Resources designates this enduring material for a maximum of 1 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity. American Academy of Physician Assistants AAPA accepts certificates of participation for educational activities certified for Category

I credit from AOACCME, Prescribed credit from AAFP and AMA PRA Category 1 Credit™ from organizations accredited by ACCME or a recognized state medical society. Physician Assistants may receive a maximum of 1 hour of Category I credit for completing this program. Nursing Credit Medical Education Resources is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. This CE activity provides 1 contact hour of continuing nursing education. Medical Education Resources is a provider of continuing nursing education by the California Board of Registered Nursing, Provider #CEP 12299, for 1 contact hour.

CREDITS: 0.5

Feature

Dermatology Clinic

Dermatologic Look-Alikes

page 56

page 71

page 79

The challenges of identifying Prader-Willi syndrome

Case #1: Peutz-Jeghers syndrome

Case #1: Molluscum contagiosum

1. Persons with Peutz-Jeghers syndrome are at the highest lifetime risk of which cancer? a. Lung b. Breast c. Ovarian d. Uterine

1. Susceptibility to molluscum contagiosum (MC) is markedly increased in children with a. Psoriasis b. Atopic dermatitis c. Lichen planus d. Pityriasis rosea

2. Successful treatment of mucocutaneous lentigines has been reported with a. Surgical excision b. Dermabrasion c. Intense pulsed light d. Cryosurgery

2. In determining the most effective treatment for MC, what is the clinical course in most cases? a. Resolves without treatment b. Responds well to topical imiquimod (Aldara, Zyclara) c. Requires curettage to eradicate d. Recurs even with aggressive treatment

1. The first phase of Prader-Willi syndrome (PWS) is marked by a. Decreased fetal movement b. Abnormal fetal position at delivery c. Increased incidence of cesarean section d. All of the above 2. Adults with PWS syndrome have difficulty with a. Long-term memory b. Reading c. Articulation d. Visual-spatial skills 3. Compared with children and adults who do not have PWS, persons with PWS a. Are less physically active due to hypotonia b. Experience dramatic fluctuations in appetite c. Require additional calories to feel sated d. Burn their food intake more rapidly 4. Which antipsychotic is used in the treatment of patients with PWS? a. Chlorpromazine b. Risperidone (Risperdal) c. Haloperidol d. Fluphenazine

TO TAKE THE POSTTEST please go to CliniAd.com/1i2wH84

Case #2: Lentigo maligna 3. What is a physical characteristic of lentigo maligna? a. Irregular borders b. Varied pigmentation c. Smooth surface d. All of the above 4. What is the preferred treatment of lentigo maligna? a. Surgical excision b. Imiquimod (Aldara, Zyclara) c. Electrodesiccation d. Radiation

Case #2: Angiokeratoma of Fordyce 3. The solitary papular angiokeratoma is usually located on the a. Labia majora b. Buttocks c. Leg d. Dorsa of fingers and toes 4. The presence of widespread multiple angiokeratomas, especially on the mucosal surface of the lower lip, helps to diagnose a. Pyogenic granuloma b. Fabry disease c. Cherry angioma d. Condylom

TO TAKE THE POSTTEST please go to CliniAd.com/19Fh1rt

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Stat Consult

A quick review of common conditions, using the best global evidence

Description

Upper-respiratory infection BY ALAN DRABKIN, MD

Dr. Drabkin is a clinical editor for DynaMed (www.ebscohost.com/ dynamed), a database of comprehensive updated summaries c overing more than 3,200 clinical topics, and Assistant Clinical Professor of Population Medicine at Harvard Medical School.

• Acute infection of upper airway, generally producing nasal congestion, rhinorrhea, cough, sneezing, and sore throat Also called

• Common cold • Upper-respiratory-tract infection (URTI) • Acute rhinitis/nasopharyngitis • Nonspecific URTI Who is most affected

• Children • Adults with increased exposure to children Incidence/prevalence

• The average U.S. adult has an estimated two to four colds per year, and the average U.S. schoolchild has six to 10 colds per year. Causes

• Generally viral in origin ——Most commonly rhinovirus ——Other viruses may include enterovirus, influenza C. • No infectious agent identified in 50% of patients with acute upper-respiratory symptoms • Common cold in elderly due to same infectious agents Pathogenesis

• Pathogenesis of each respiratory virus can differ.

Likely risk factors

Acute infection of the upper airways (shown) can lead to fits of coughing and difficulty breathing.

• Higher levels of psychological stress may increase susceptibility to upper-respiratory infection (URI). • Exposure to young children (e.g., in day care) • Shorter sleep duration and poorer sleep efficiency in weeks prior to rhinovirus exposure Possible risk factors

• Exposure to persons with respirator y complaints Continues on page 86

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You Asked for a Simpler CME Search

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Stat Consult • Lack of social ties • Biomass fuels • Exposure to tobacco smoke • Recent airplane travel • Antibiotic treatment for acne • Negative emotional style Factors not associated with increased risk

• Asthma may not increase risk of infections in primarycare patients. • Exposure to dampness or mold at home Complications

• Otitis media • Sinusitis • Asthma exacerbation • Exacerbation of other pulmonary diseases • Loss of olfactory function History

• Chief concern ——Nasal congestion, rhinorrhea, cough ——Other symptoms can include sore throat, headache, hoarseness, malaise, lethargy, and myalgias. • History of present illness ——Initial symptoms typically are sore throat, malaise, and low-grade fever. ——Typical presenting symptoms of nasal congestion, rhinorrhea, and cough appear within 24 to 48 hours. ——Duration of symptoms ■■ Usually peak at day 3 to day 4 ■■ Resolve or significantly improve by day 7 ■■ Usually last one to two weeks, but may be more than three weeks ■■ Common cold often lasts >10 days.

Making the diagnosis

• Diagnosis is usually made clinically, based on symptoms of nasal congestion, rhinorrhea, cough, or sore throat in the absence of identified cause. Differential diagnosis

• Influenza • Streptococcal pharyngitis • Acute sinusitis • Acute bronchitis • Asthma • Allergic rhinitis • Pneumonia • Infectious mononucleosis Diagnostic testing

• Not usually indicated or needed for diagnosis • Nose and throat swabs appear sufficiently sensitive to rule out influenza A and respiratory syncytial virus. —— xTAG Respiratory Viral Panel FDA-approved to test for 12 respiratory viruses from single sample. ■■ Nucleic acid test ■■ Viruses tested include »»Influenza A subtypes H1 and H3 »»Influenza B »»Human metapneumovirus »»Respiratory syncytial virus subtypes A and B »»Parainfluenza 1, 2, and 3 »»Rhinovirus »»Adenovirus Imaging studies

• Not usually indicated • Radiographic sinusitis common during the common cold Treatment

Physical

• General physical examination ——Mild fever occasionally present in adults and is fairly common in children. ——The signs and symptoms may have insufficient sensitivity and specificity to differentiate viral from bacterial URIs. • Head, eyes, ears, nose, and throat examination ——Typical signs may include ■■ Nasal discharge ■■ Sinus tenderness ■■ Erythema of oropharynx

• No randomized trials that evaluate increasing fluid intake have been identified. • Receiving information/reassurance strongly associated with patient satisfaction. • Acetaminophen might reduce fever in children, but evidence is limited and inconclusive. • Nonsteroidal anti-inflammatory drugs may reduce discomfort or pain. • Treatments that may reduce symptom duration or overall symptom severity ——Pelargonium sidoides extract (Umcka ColdCare) Continues on page 88

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Stat Consult ——Homeopathy ——Intranasal sodium cromoglycate does not appear to be effective for reducing cold symptoms in children. ——Hypertonic saline • Goldenseal should not be used due to reported serious adverse effects. • There is limited evidence for the use of Chinese herbal medicines to treat common cold. • American Geriatrics Society Beers Criteria ——Avoid first-generation antihistamines in older adults. ——Avoid inhaled anticholinergic agents or strongly anticholinergic drugs in men with lower-urinary-tract symptoms and/or benign prostatic hyperplasia. Prevention

• Interventions that may reduce incidence of acute respiratory infections ——Educational programs to promote hand-washing ——Hand disinfection ——Physical activity ——Vitamin C prophylaxis may slightly reduce incidence, duration, and severity of common cold. • No evidence for reduction of incidence of acute respiratory infections with ——Multivitamins ——Vitamin E—200 mg daily • Insufficient evidence to support or refute use of vitamin D. n

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——Echinacea (evidence inconsistent) ——Zinc lozenges ≥75 mg/day may decrease duration but not severity of cold symptoms in adults (intranasal zinc not recommended due to possible loss of smell). ——Andrographis paniculata in adults (KalmCold and Kan Jang tablets) ——Iota-carrageenan (sulfated galactose polymer derived from Rhodophyceae seaweed) ——OTC combination products containing antihistamines, decongestants, and/or analgesics in older children and adults ——Isotonic saline nasal wash (seawater) six times/day during acute illness, then three times/day in children ——Heated, humidified air ——Vapor rub may improve nighttime symptoms in children. ——Sipping hot water or hot chicken soup has been reported to increase nasal mucus velocity. • Treatments for cough ——Recommendations from the American College of Chest Physicians ■■ Benefit suggested with either »»Bromphen iram ine plus sustained-release pseudoephedrine »»Ipratropium oral inhalation (Atrovent HFA) ■■ Cough suppressants should not be used. ——Most OTC oral medicines for acute cough do not have good evidence of benefit; medicines that have some evidence of efficacy in adults (but not children) are ■■ Dextromethorphan ■■ Guaifenesin ■■ Bromhexine ■■ Dexbrompheniramine/pseudoephedrine ——Honey may reduce nocturnal cough and sleep disruption in children with acute cough, and might be more effective than dextromethorphan or diphenhydramine. • Treatments for nasal symptoms ——Decongestants (nasal or oral) are moderately effective for short-term relief in adolescents and adults. ——Ipratropium nasal spray may improve rhinorrhea but not nasal congestion. • Medications that do not appear effective ——Antibiotics ——Antivirals ——Nonprescription medicines for acute cough in children ——Antihistamines alone ——Vitamin C at onset of cold symptoms ——Intranasal corticosteroids

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COMMENTARY Sharon M. Fruh, PhD (far left), teaches at University of South Alabama College of Nursing, and Marcia D. Greenblum, MS, RDN, is with the International Food Information Council Foundation.

Why dietary-protein intake matters Clinicians can greatly improve the health of their patients as well as themselves simply by ensuring that dietary protein intake meets recommended levels of 10% to 35% of caloric intake. Ample calorie intake from other macronutrients—carbohydrates and fats—allows protein to be spared for its important roles in lean body mass preservation, hormone and antibody synthesis, and blood acidity and bone health maintenance, among other actions. Consumers are looking for protein when shopping for food, according to the International Food Information Council Foundation’s 2013 Food & Health Survey (available at bit.ly/14F02yU; accessed

Protein is vitally important for wellness among those requiring medically prescribed bed rest.

December 15, 2013). The majority of 576 respondents reported seeking protein in their food and beverage choices for energy, strength, and satiety (p. 49). However, consumers are inclined to believe that children and teens get the greatest benefit from higher protein intake, and that persons aged 55 years and older benefit the least (p. 48). But because muscle synthesis declines with age, adequate protein intake is essential for aging adults, who need this nutrient to defend their health through immune response to infection or to heal from injury. Yet protein is indeed especially important during periods of growth, such as infancy and the teenage growth spurt, when nitrogen demands are particularly high. Additionally, for athletes, the nitrogen supply that only protein-rich foods offer encourages muscle synthesis and bodytissue repair, vital for strengthening muscles. For persons struggling with weight management, a higher ratio of protein to carbohydrates can reduce hunger for longer periods following meals, making it easier to resist the temptation to snack (Am J Clin Nutr. 2013;97:677-688; available at ajcn.nutrition.org/content/97/4/677 .long, accessed December 15, 2013). Protein is vitally important for wellness among those requiring medically prescribed bed rest. For aging adults, bed rest or acute inactivity during hospitalization or a disease state poses a potent threat to muscle tissue and functional

capacity. After the age of 30, adults lose 3% to 8% of their muscle mass per decade. Over time, the loss of lean tissue contributes to decreased muscle strength and power, which are important predictors of balance, falls, and mortality. Sarcopenia is highly prevalent in America: More than 50% of adults aged 80 years and older (and approximately 20% of adults older than age 70 years) can be characterized as sarcopenic. With advancing age, even a brief period of bed rest is increasingly likely to initiate a serious decline in muscle strength and functional capacity, from which full recovery is often unattainable. Researchers have found the following steps can have a great impact on limiting loss of muscle mass and function: 1) Consume a moderate amount (25 g to 30 g) of high-quality protein, such as milk, yogurt, eggs, fish, soy isolates, poultry, or meat, with each meal. 2) Consume protein shortly after exercising. 3) Combat the accelerated loss of muscle mass and function during acute catabolic crises including fever, burns, or other trauma and limited periods of physical inactivity with targeted amino acid supplementation (Curr Opin Clin Nutr Metab Care. 2010;13:34-39). Suggestions of easy ways to incorporate protein into the daily diets of young children, young adults, women, and baby boomers can be found at www.foodinsight. org (input “protein fact sheets” into the search function available at that website). n

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