December 2014 Clinical Advisor by The Clinical Advisor

THE CLINICAL ADVISOR • DECEMBER 2014

A PEER-REVIEWED FORUM FOR NURSE PRACTITIONERS

NEWSLINE

■ Eczema: a fracture factor ■ Bariatric halt to diabetes ■ 5 more ER tests to avoid ADVISOR FORUM

■ Sign of digoxin toxicity ■ Causes of abdominal pain ■ More on craniosacral Tx

DECEMBER 2014

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KEEP INFANTS SAFE FROM

CONGENITAL CMV Cytomegalovirus (shown, in pink) is the leading cause of birth defects and developmental delays.

LEGAL ADVISOR

Misdiagnosis leads to fatal emergency for young man

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■ Dermatology Clinic

BULLAE ON FOOT AFTER A SWIM PAGE 67

■ Dermatologic Look-Alikes VOLUME 17, NUMBER 12

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Editor Delicia Honen Yard, editor@ClinicalAdvisor.com Senior editor Sandhya George Production editor Kim Daigneau Senior digital content editor Nicole Blazek Digital content editor Brianne M. Aiken Assistant digital content editor Hannah Dellabella Contributing editors Mark P. Brady, PA-C; Philip R. Cohen, MD; Deborah L. Cross, MPH, CRNP, ANP; Sharon Dudley-Brown, PhD, FNP; Abimbola Farinde, PharmD; Laura A. Foster, CRNP, FNP; Abby A. Jacobson, PA; Maria Kidner, DNP, FNP; Joan W. Kiely, MSN, CRNP; Debra August King, PhD, PA; Ann W. Latner, JD; Mary Newberry, CNM, MSN; Claire Babcock O’Connell, MPH, PA; Kathy Pereira, DNP, FNP; Sherril Sego, DNP, FNP; Julee B. Waldrop, DNP, PNP, FNP; Ann Walsh, PA-C, SCT(ASCP); Kim Zuber, PA-C Group art director, Haymarket Medical Jennifer Dvoretz Production director Ada Figueroa Circulation manager Paul Silver National accounts manager Alison McCauley, 646.638.6098 alison.mccauley @ haymarketmedical.com Publisher Thomas P. Hennessy, 646.638.6085 tom.hennessy @ haymarketmedia.com Editorial director Jeff Forster Senior vice president, digital products and medical magazines Jim Burke, RPh Senior vice president, clinical communications John Pal CEO, Haymarket Media, Inc. Lee Maniscalco

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CONTENTS DECEMBER 2014

NEWS AND COMMENT

DEPARTMENTS

18 Newsline ■■Eczema heightens fracture risk in adults ■■Chart: Age-adjusted rates for the top 10 causes of death, 2012 ■■Diabetes rate drops with bariatric surgery ■■Statin use advised in chronic kidney disease ■■BP of 150 adequate for older adults ■■Five more ER tests to avoid ■■New guidelines address IBS drugs

56 Derm Dx Make your dermatology diagnosis at ClinicalAdvisor.com. 60 Legal Advisor A young man dies when an emergent condition is mistaken for a nonemergent one. Eczema has a role in bone fracture. 18

66 CME/CE Dermatology Clinic n Purple plaque on foot after swim in river n Papules on girl with Down syndrome

81 Commentary Communication between clinicians is key for patient safety, continuity of care, and efficiency of care.

FEATURES 30 Breaking the silence about congenital CMV Congenital cytomegalovirus is a very common cause of birth defects, yet many clinicians are not well-informed about it.

CME/CE: Treatments for sleep apnea 36

MAKING CONTACT

TOC_CA1214.indd 8

71 CME/CE Dermatologic Look-Alikes A young boy and a middle-aged woman each present with hypopigmented, asymptomatic lesions. 77 CME/CE Dermatology posttest 78 Alternative Meds Update Fluoride is a boon to public health.

36 CME/CE Obstructive sleep apnea: an overview of treatments With obstructive sleep apnea rates rising in tandem with obesity, providers should know the therapeutic options. 45 CME/CE Feature posttest

64 Ortho Dx Make your orthopedics diagnosis at ClinicalAdvisor.com.

Continues on page 10

Fluoride has prevented pain and disability. 78

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CONTENTS ADVISOR FORUM 52

The Consultant Files ■ Yellow lights, digoxin toxicity

Your Comments ■ Think broadly for abdominal pain ■ More thoughts on craniosacral therapy, from the son of CST pioneer John E. Upledger, DO

Response ■ Sherril Sego, FNP-C, DNP, responds to Mr. Upledger’s letter.

“I don’t care what your mother did— I’m not eating the fleas off your back.”

CONTEST 23

Scavenger Hunt Join us for our annual Scavenger Hunt Challenge! The 10 questions on page 23 all relate to content presented throughout this issue of The Clinical Advisor. Submit the 10 correct answers and you will automatically be entered into a drawing to win a $250 American Express Gift Cheque. Happy hunting!

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IEWE D FORU M FOR NUR SE PRAC TITIO

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ISO

R Misdiagnosis leads to fatal emergency for young man

NERS

DECE MBER

2014

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dvisor.com

KEEP INFA

NTS SAFE

CONGENIT AL CMV FROM

Cytomeg alovirus (show n, in pink) cause of birthis the leading defects and developm ental dela ys.

✶ FREE CE COURS

ES!

■ Dermatolo gy Clini

c BULLAE AFTER AON FOOT SWIM

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R 12

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FORUM

■ Sign of digo xin toxicity ■ Causes of abdominal pain ■ More on craniosacral Tx

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■ Eczema: a fracture facto ■ Bariatric r halt to diab etes ■ 5 more ER tests to avoid

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EXCLUSIVE TO THE WEB AT

ClinicalAdvisor.com Web Exclusives

Videos

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Novel hepatitis C vaccine promising in first human trials An investigational hepatitis C vaccine was safe in humans and successfully induced T-cell responses.

Can exercise increase brain function in kids? A study published in Pediatrics indicates that exercise may boost cognition in pediatric patients.

Medicare proposes to cover lung cancer screenings for at-risk patients CMS announced it has drafted a reimbursement proposal to cover annual computed tomography (CT) scans for high-risk patients. Nearly 140M Americans need weight-loss treatment Of the 140 million American adults recommended for weight-loss treatment, 83% could be considered for pharmacotherapy.

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The neurology of obesity Stephan J. Guyenet, PhD, specializes in the science of obesity, tying together research from several related fields to offer explanations and solutions for our global weight problem.

The Waiting Room Official Blog of The Clinical Advisor ClinicalAdvisor.com/TheWaitingRoom Jillian Knowles, MMS, PA-C How I learned to love dental blocks Researching and performing different care methods benefits patients and providers. Sharon M. O’Brien, MPAS, PA-C Be careful of sleepy drivers Patients who may be impaired by their medications or sleep disorders have a higher risk of fatal motor vehicle accidents. Sharon M. O’Brien, MPAS, PA-C Muscle pain in older adults may be polymyalgia rheumatica Asking older patients about their muscle pain can help prevent undiagnosed polymyalgia rheumatica and giant cell arteritis. Jim Anderson, MPAS, PA-C, DFAAPA Breast Cancer Action: systems, social causes of cancer A medical organization dedicated to discussing the root causes of cancer takes a different perspective on breast cancer awareness.

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Newsline D E C E M B E R 2 014

Statins good for patients with predialysis CKD page 19

Blood pressure target of 150 okay for adults page 20

New guidelines for irritablebowel drugs page 20

Eczema heightens fracture risk in adults

ECZEMA IN adulthood should be considered a risk factor for fracture and bone or joint injury, according to the authors of a study published online ahead of print in JAMA Dermatology. Fractures and bone or joint injuries can lead to loss of independence and an increased risk for early death among older adults in the United States, according to the Centers for Disease Control and Prevention. One study cited by the International Osteoporosis Foundation determined that the combined lifetime risk for hip, forearm, and vertebral fractures that will require clinical attention is approximately 40%, which is equivalent to the risk for cardiovascular disease (Kanis JA. Lancet. 2002;359[9321]:1929-1936). In the recent analysis of 34,500 adults aged 18 to 85 years with a history of eczema in the previous 12 months, authors Nitin Garg,

Eczema was linked with more fractures, joint injuries.

MD, and Jonathan I. Silverberg, MD, PhD, MPH, found that those with eczema were more likely to have bone fractures and bone or joint injuries. They determined that the prevalence of eczema was 7.2% and the prevalence of any injury-causing limitation was 2%. A limitation that caused a bone fracture and bone or joint injury was reported by 1.5% of study participants, and limitations that caused other types of injury occurred in 0.6%. The investigators found that the prevalence of limitations that caused fractures and bone or joint injuries increased with age, peaking at age 50 to 69 years but decreasing at age 70 years and older. They also found that adults with eczema and fatigue, daytime sleepiness, or insomnia had higher rates of fracture and bone or joint injury than did those

with the same sleep issues but no diagnosis of eczema. Adults with both eczema and psychiatric and behavioral disorders also had higher rates of fracture and bone or joint injury than did adults with eczema alone or psychiatric and behavioral disorders alone. “Adult eczema is associated with an increased risk of injury, particularly fracture and bone or joint injury, which is only partially related to the presence of sleep symptoms and psychiatric and behavioral disorders,” concluded the authors “Taken together, these data suggest that adult eczema is a previously unrecognized risk factor for fracture and other injury, emphasizing the importance of developing safer and more effective clinical interventions for itch and sleep problems in eczema, as well as preventive measures for injury risk reduction in eczema.”

Age-adjusted rates for the 10 top causes of death, 2012 Heart disease and cancer continue to be the leading causes of death in the United States. Source: Xu JQ, Kochanek KD, Murphy SL, Arias E. Mortality in the United States, 2012. National Center for Health Statistics Data Brief. 2014;168.

170.5 166.5

Heart disease Cancer Chronic lower-respiratory diseases Unintentional injuries Stroke Alzheimer disease Diabetes Influenza and pneumonia Kidney disease Suicide

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

41.5 39.1 36.9 23.8 21.2 14.4 13.1 12.6 0

40 80 120 160 Deaths per 100,000 standard population

200

Diabetes rate drops with bariatric surgery BARIATRIC SURGERY was associated with a lower incidence of type 2 diabetes in obese men and women for up to 7 years after the procedure in a report published by The Lancet Diabetes & Endocrinology. Senior author Professor Martin C. Gulliford, FFPH, and colleagues found that undergoing laparoscopic adjustable banding, sleeve gastrectomy, or gastric bypass reduced the risk of developing type 2 diabetes by approximately 80% in obese participants enrolled from family practices in the United Kingdom. “Our results suggest that bariatric surgery may be a highly effective method of preventing the onset of new diabetes in men and women with severe obesity,” announced Gulliford in a statement issued by The Lancet. “We need to understand how

weight-loss surgery can be used, together with interventions to increase physical activity and promote healthy eating, as part of an overall diabetes prevention strategy.” The researchers studied 2,167 obese adults without diabetes who underwent laparoscopic adjustable banding, sleeve gastrectomy, or gastric bypass. Also evaluated were an equivalent number of controls who were matched for age (20 to 100 years), sex, body mass index (≥30 kg/m²), and hemoglobin A1c levels, but who did not have surgery or other treatments for obesity. After a maximum of 7 years of follow-up, 38 of the patients who underwent a bariatric procedure developed diabetes, compared with 177 patients who had no treatment for obesity. The incidence of

Diabetes was less likely up to 7 years after procedure.

new diabetes diagnoses was 5.7 per 1,000 person-years with bariatric surgery and 28.2 per 1,000 personyears without obesity treatment, for a reduction in risk for developing diabetes of approximately 80%, even after controlling for factors including smoking, high blood pressure, and high cholesterol.

MOST OLDER persons with predialysis chronic kidney disease (CKD) should take statins, indicate the findings of a study published by Journal of the American Society of Nephrology. Two guidelines on cholesterol management in CKD were compared—one from the American College of Cardiology and the American Heart Association (ACC/AHA) and the other from the Kidney Disease Improving Global Outcomes (KDIGO) Lipid Work Group. Using data from the Reasons for Geographic and Racial Differences

Most aged 50 to 79 years with CKD need statins.

in Stroke Study, lead author Lisandro Colantonio, MD, MSc, and fellow investigators compared the statin treatment recommendations in the two guidelines among more than 30,000 U.S. adults aged 50 to 79 years. All participants had CKD (estimated glomerular filtration rate <60 mL/min per 1.73 m2 or albuminuria ≥30 mg/g) but were not on dialysis. The major findings of the study were as follows: • The guidelines agree that nearly all persons aged 50 to 79 years with CKD should be on a statin regimen (92%, according to the

ACC/AHA guideline; 100%, according to the KDIGO guideline). • Half (50%) of all persons with CKD in the study who should have been taking statins according to these guidelines, were not. • Pooled cohort risk equations, which can estimate a 10-year predicted risk for atherosclerotic cardiovascular disease of 7.5% or more, offer moderately good discrimination for clinicians to use it as a tool to estimate risk for heart disease, and thereby guide therapy, in persons with CKD.

www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • DECEMBER 2014 19

IMAGES: © THINKSTOCK

Statin use advised in chronic kidney disease

Newsline BP of 150 Five more ER tests to avoid adequate for older adults

Avoid lumbar spine imaging for atraumatic back pain.

1. Avoid computed tomography (CT) of the head in asymptomatic adult patients with syncope, insignificant trauma, and a normal neurologic evaluation. 2. Avoid CT pulmonary angiography in patients with a low pretest probability of pulmonary embolism and either a negative assessment for Pulmonary Embolism Rule-Out Criteria (PERC) or D-dimer. 3. Avoid lumbar spine imaging in adults with atraumatic back pain unless the patient has severe or progressive neurologic deficits or is suspected of having a serious underlying condition, such as vertebral infection or cancer with bony metastasis. 4. Avoid prescribing antibiotics for uncomplicated sinusitis. 5. Avoid CT of the abdomen and pelvis in young but otherwise healthy persons with known histories of ureterolithiasis presenting with symptoms of uncomplicated kidney stones.

New guidelines address IBS drugs The American Gastroenterological Association (AGA) has issued new guidelines to help clinicians navigate drug options for irritable bowel syndrome (IBS). Published by senior author Shahnaz Sultan, MD, MHSc, in Gastroenterology, the evidence-based guidelines (2014;147[5]:11461148) are accompanied by a technical review (1149-1172.e2). For patients with IBS with constipation, the group strongly recommended linaclotide over no drug treatment, with a conditional

20 THE CLINICAL ADVISOR • DECEMBER 2014 • www.ClinicalAdvisor.com

recommendation for lubiprostone over no drug treatment. The AGA made conditional recommendations for drugs with only moderate- or low-quality evidence. For patients with IBS with diarrhea, the authors conditionally recommended rifaximin, alosetron, and loperamide. Tricyclic antidepressants, selective serotonin reuptake inhibitors, and antispasmodics were suggested for persons with IBS, but only as conditional recommendations due to the low quality of available evidence.

MILD CONTROL of systolic blood pressure (SBP), in which SBP is maintained at less than 150 mmHg, is adequate for people aged 65 years and older, according to a systematic review published online ahead of print by the journal Drugs & Aging. Senior author David S. H. Lee, PharmD, PhD, and fellow investigators analyzed findings from 31 studies on the use of antihypertensive agents in persons aged 65 years and older with a diagnosis of hypertension. They found that these patients had lower cardiovascular morbidity and mortality with antihypertensive treatment than without. However, Lee’s team determined that strict control, in which SBP is maintained at less than 140 mmHg, offered no more benefit than did mild control in this older population. Although treatment of hypertension with medication is important to help prevent myocardial infarction, kidney disease, and stroke, the researchers pointed out that antihypertensive agents can have side effects that increase with higher dosages. “There’s clearly a value to controlling blood pressure enough to keep it at 150 or less,” pointed out Lee in a statement accompanying the release of the study results. “But as people get older, there’s less clear evidence that stringent control of systolic blood pressure is as important.”

A SECOND LIST of 5 tests and procedures that should be questioned before use has been issued by the American College of Emergency Physicians (ACEP) as part of the ABIM Foundation’s “Choosing Wisely” campaign. The initiative is designed to reduce the use of medical testing in situations in which the harm of using these tests may outweigh the benefits. The first 5 procedures and practices flagged by ACEP in October 2013 are listed at choosingwisely.org/ doctor-patient-lists/americancollege-of-emergency-physicians. The evidence-based recommendations were approved by ACEP’s board of directors and are specific to patients in the emergency room (ER). The risks of these medical tests often cited by the group include radiation exposure, use of potentially harmful dyes, and cost. The new list includes the following 5 recommendations:

Take the Scavenger Hunt Challenge December 2014 edition

WHO MAY ENTER All nurse practitioners and physician assistants aged 21 and older 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 January 15, 2015. 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, 2015. 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, 2015, to individuals who send a stamped, self-addressed, business-sized envelope to The Clinical Advisor Contest Winners, 114 W. 26th St., 4th Floor, New York, NY 10001.

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 drawing to win a $250 American Express Gift Cheque. To submit your responses, simply go to ClinicalAdvisor.com/Dec2014Hunt. QUESTIONS 1. What is an adequate systolic blood pressure maximum for hypertensive adults aged 65 years or older, according to a recent study? (p. 20) 2. How is congenital cytomegalovirus cured? (p. 32) 3. What is the treatment of choice for mild, moderate,or severe obstructive sleep apnea? (p. 38) 4. What vision abnormality is associated with digoxin toxicity? (p. 52) 5. In a recent case of undiagnosed myocarditis, what symptom in particular led the clinician to a different diagnosis? (p. 62) 6. Which two agents comprise the preferred antibiotic regimen for treatment of Vibrio vulnificus? (p. 68) 7. What test can confirm a diagnosis of syringomas? (p. 70) 8. Do most cases of vitiligo fall into the category of localized, generalized, or universal? (p. 74) 9. True or false: Fluoridated water was found to be much more effective than topical fluoride in preventing dental caries (p. 80) 10. What technological advance has reduced face-to-face communications between clinicians regarding patients? (p. 81) www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • DECEMBER 2014 23

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FEATURE: ANNA LILIA PIÑA, APRN, MSN, NP-C

Breaking the silence about congenital CMV Congenital cytomegalovirus is a very common cause of birth defects, yet many primary-care providers are not well-informed about this infection.

he prevalence of cytomegalovirus (CMV) infection in human hosts has been welldocumented for many decades, as has the virus’s disastrous effects on the developing fetus. The burden of congenital CMV (cCMV) has been growing steadily for more than a century, with one of its first documented observations made by Philadelphia pathologists performing an autopsy on a stillborn infant in 1910.1 This virulent infection has become the leading cause of birth defects, developmental delay, and disability in U.S. children,2 as well as the leading infectious cause of hearing loss.3 Despite the prevalence of cCMV and the severity of its effects, public awareness regarding this infection is quite low: Less than 15% of women have any knowledge of this disease.3 Because nurse practitioners, physician assistants, and other primary-care providers are generally the first line for medical care, it is imperative that these clinicians be knowledgeable about cCMV in order to educate patients, particularly those who are pregnant or who are planning to have children. With no effective vaccine, cure, or treatment for cCMV, prevention is crucial.3 It also is important to remember that most affected children are born asymptomatic but can experience symptom manifestations as they continue to grow, and close follow-up is warranted.4

Cytomegalovirus (shown, in pink) has no effective vaccine, cure, or treatment.

30 THE CLINICAL ADVISOR • DECEMBER 2014 • www.ClinicalAdvisor.com

Epidemiology and transmission

CMV is a member of the Herpesviridae family and is one of the 8 herpesviruses to cause human infection (Table 1). CMV is acquired

through contact with infected bodily fluids, and like the other herpesviruses, CMV remains permanently within its host, with periods of latency and activation throughout the person’s life span.5 In more than 95% of cases, CMV enters the immunocompetent host without causing any symptoms of infection, as it has developed numerous gene products that will evade or interfere with the immune response.5 Once infected, the person develops an extensive immune response that involves all arms of the human adaptive immune system, making vertical transmission less common in previously infected mothers.6 However, when the woman is infected for the first time during pregnancy, the virus can be vertically transmitted to the fetus through the placenta, resulting in detrimental effects for the newborn.3 Up to 90% of the U.S. population will be infected with CMV by age 80 years.3 Yet approximately 30% to 50% of women of childbearing age have never been infected with CMV, and up to 4% of them will develop their first (primary) CMV infection during pregnancy.7 Within that group of women, 30% to 40% will transfer the infection to the fetus through the placenta.3 Mothers who had an earlier CMV infection and experience a period of reactivation during pregnancy or become infected with a new strain of the virus have about a 1% risk of transmitting the virus to the fetus.3 Following in utero transmission, CMV causes more cases of congenital illness and disease than do the 29 currently recommended conditions for which newborns are screened in the United States.6 The Centers for Disease Control and Prevention (CDC) warns expectant mothers about acquiring CMV during pregnancy and encourages them to take steps to prevent infection.8 However, prevention can be particularly challenging for these women, who often already have young children. Young children, particularly those up to age 3 years, are the most common vectors of CMV transmission due to the fact that they lack control of their bodily fluids and the virus is shed largely in the saliva and urine. Approximately 50% of children in day care are actively shedding CMV9 and could ultimately be transmitting the infection to their mothers, who may be pregnant. Yinon and colleagues estimate that approximately 40,000 U.S. infants per year are born with CMV infection. Of these children, 20% will be affected, meaning they will be born with active symptoms. This translates to roughly 8,000 children per year who will have long-term disabilities due to this infection and approximately 400 who will die in childhood each year. In addition, 5% to 15%

TABLE 1. Herpesviruses in humans Formal name

Common name

Human herpesvirus 1

Herpes simplex virus 1

Human herpesvirus 2

Herpes simplex virus 2

Human herpesvirus 3

Varicella-zoster virus

Human herpesvirus 4

Epstein-Barr virus/Mononucleosis

Human herpesvirus 5

Cytomegalovirus

Human herpesvirus 6

Roseola infantum

Human herpesvirus 7

Roseola infantum

Human herpesvirus 8

Kaposi sarcoma

Compiled by Anna Lilia Piña, APRN, MSN, NP-C

of the children born asymptomatic will develop sequelae later in childhood.3 cCMV syndrome presentation

Newborns with cCMV who are born symptomatic face many challenges as they can be critically ill for the first few weeks and months of life. More than 75% of these neonates will have been born with a petechial rash, elevated alanine aminotransferase (ALT) levels, elevated bilirubin levels, and thrombocytopenia. More than 50% of affected infants are born with microcephaly, periventricular calcifications, and hepatosplenomegaly, and are small for gestational age.3 These newborns may be further afflicted with hypotonia, pneumonitis, chorioretinitis, hearing loss, poor suck, hemolysis, or seizures. Some of these infants will die soon after birth or in early childhood.10 Infants born affected should also be screened for other congenital conditions and viral infections in order to receive optimal treatment.3 The majority of children who are symptomatic at birth, especially those with neurologic symptoms, will develop long-term sequelae that can include hearing loss, vision loss, cerebral palsy, mental retardation, seizure disorder, and developmental delays.3,11 Hearing loss associated with cCMV is progressive, and children congenitally infected with CMV will require close monitoring and follow-up throughout childhood as well as early intervention therapy to help decrease the severity of developmental delays.3 Diagnosis of cCMV

Neither pregnant women nor newborns are routinely screened for CMV infection.11 The clinician may suspect that a pregnant woman has primary CMV infection if the woman experiences a mononucleosis-like illness, has been

www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • DECEMBER 2014 31

CONGENITAL CYTOMEGALOVIRUS

Treatment options

POLL POSITION

Should pregnant women undergo routine screening for cytomegalovirus (CMV) infection? n=131 7.63%

■ Yes ■ No ■ Undecided

7.63% 84.73%

For more polls, visit ClinicalAdvisor.com/Polls.

exposed to somebody with CMV infection, or may have suffered occupational exposure (such as if she works for a children’s day-care facility).5 When congenital CMV is suspected or a general screening is desired during pregnancy, the mother should undergo serum testing for CMV antibodies. The provider must keep in mind that antibodies can take more than 2 weeks to appear and can be present for more than 6 months following infection.3 With this in mind, if the initial testing is negative but the suspicion is high, the test should be repeated in 2 to 4 weeks. If immunoglobulin (Ig) G alone is present, or both IgG and IgM are present, an IgG avidity test should be performed to determine the maturity of the IgG present.9 Avidity of less than 30% indicates that the infection was acquired within the previous 3 to 4 months, and the diagnosis of primary infection can be made.3 If an expectant mother is found to have primary CMV infection, her care should be handled by a high-risk-pregnancy specialist who can closely monitor the fetus, as hydrops fetalis and fetal death are possibilities.5 An amniocentesis should be done after 7 weeks of suspected infection but after 20 weeks’ gestation. Amniotic fluid, not fetal serum, should be tested by polymerase chain reaction (PCR) method for CMV infection, because higher levels of virus are excreted by the fetal kidneys through the urine into the amniotic fluid. However the renal system in the fetus is not functional until after 20 weeks, so the procedure cannot be performed until after this time. Regardless of the results of the amniocentesis, the infant’s urine should be tested by viral culture and PCR shortly after birth and within the first 3 weeks of life for proper diagnosis of congenital infection.5

Although there is currently no cure for cCMV, trials have shown benefit from maternal, and at times fetal, transfusions of CMV-specific intravenous immunoglobulin (IVIG),12 and this should be offered to parents as an option. Further, a study done by Maidji and colleagues in 2010 demonstrated that IVIG transfusions received by the mother resulted in improved placental vasculature, reduced placental viral replication, and decreased necrotic villi. This in turn improved blood flow and nutrient delivery to the fetus.10 Providers should offer parents of infants born with symptomatic cCMV the option to treat the newborn with intravenous ganciclovir or oral valganciclovir to help reduce the incidence of progressive hearing loss.6 Although the clinician should make clear to the parents that this is not a cure and will not reverse any neurologic damage, these treatments have been associated with some neurodevelopmental improvement.3 Hearing loss has been shown to be reduced or prevented with 6-week courses of intravenous ganciclovir or oral valganciclovir,6 with roughly 84% of children having either maintained or improved their hearing throughout the 6 months following treatment.3 Of children treated with either regimen, 21% will continue to experience deterioration in hearing, compared with 68% of those not treated.3 In a retrospective study of children with asymptomatic cCMV and normal hearing at baseline, reduced rates of hearing loss were seen in those who underwent 6 weeks of intravenous therapy followed by 1 year of oral therapy.6 Parents should be cautioned, however, that the risk of neutropenia associated with these medications is roughly 63% for intravenous ganciclovir and 38% for oral valganciclovir, and can be severe enough to warrant discontinuation of treatment.6 This side effect, however, does resolve with cessation of treatment. Antiviral therapy should be initiated in infants born with evidence of central nervous system involvement and hearing loss, and should be strongly considered for newborns with end-organ disease such as hepatitis, thrombocytopenia, and pneumonia.3

CLINICAL SLIDESHOW For general information on the prevention of birth defects, please view the slideshow at ClinicalAdvisor.com/PreventingBirthDefects

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

CONGENITAL CYTOMEGALOVIRUS

Online resources for parents

Role of the primary-care provider

Although the urine test is not adequate for cCMV testing in later childhood due to the virus’s permanent presence after initial infection, any infant who failed the hearing screen at birth or has developed other concerning symptoms that may be indicative of cCMV can still be tested by the primarycare provider within the first 3 weeks of life. However, if the child develops symptoms after the first 3 weeks of life, and there is clinical suspicion for cCMV, the child’s dried blood spots (DBS) can be tested for CMV by means of PCR as the virus is stable on DBS cards for up to 18 years.6 Drawbacks of this method of testing include the fact that the length of time the cards are kept varies by state, but most important, if viral replication at birth is low or absent, the test may render a false-negative result.6 The primary-care provider must promptly make all necessary referrals to clinicians specializing in pediatric infectious disease, hearing, vision, and neurology; and to physical therapy, speech therapy, and occupational therapy services. This will facilitate early intervention for the diagnosed infant as well as for infants with other developmental delays or medical conditions.3 Many insurance plans, specifically state Medicaid plans, require the child’s primary-care provider to order these referrals, which makes it even more important for these practitioners to be knowledgeable about cCMV and vigilant about clinical presentation. Audiology assessments should be conducted a minimum of every 6 months in infected children, even if asymptomatic, for the first 3 years of life, and then routinely at the audiologist’s discretion.3 If at any time the primary-care provider has clinical suspicion of undiagnosed maternal or congenital CMV infection but is not comfortable performing the testing, he or she should refer the case to a perinatologist and/or a pediatricinfectious-disease specialist as soon as possible. Prevention strategies

Considering the detrimental effects of cCMV on affected children and families, the lack of treatment options, and the related health-care costs, the primary-care provider should focus on preventing primary CMV infection in women who are pregnant or who are planning a pregnancy. It has been noted that CMV incidence among hospital workers is no higher than CMV incidence in the rest of the population, validating the effectiveness of standard precautions in preventing transmission of CMV infection.5 Prevention and risk reduction consist of educating patients regarding simple hygienic measures that help reduce the spread of most infections, including CMV.5

National Congenital CMV Disease Registry, Baylor College of Medicine Department of Pediatrics bcm.edu/departments/pediatrics/cmvregistry/ Stop CMV stopcmv.org Brendan B. McGinnis Congenital CMV Foundation CMVfoundation.org

For several years, guidelines from The American College of Obstetricians and Gynecologists (ACOG) have recommended that pregnant women be counseled regarding methods to prevent acquisition of CMV during pregnancy.13 Primarycare providers need to instruct women of childbearing age during preconception and throughout pregnancy to practice good hand hygiene, washing with antimicrobial soap and water for periods of 15 to 20 seconds throughout the day, particularly after changing diapers and coming into contact with any bodily fluid.8 Also, pregnant women should develop the habit of kissing children, including their own, on the forehead rather than on or near the mouth, to minimize contact with the child’s saliva.8 These women also should avoid sharing their drinks or eating utensils with others. In addition, they should regularly disinfect children’s toys and items that are frequently handled by others.8 These simple steps can greatly reduce the risk of CMV transmission to an expectant mother. Conclusion

Regardless of the scientific advances in the field of CMV and the increasing burden of cCMV infections, lack of awareness and lack of screening continue to be the norm. Despite being the leading cause of disability in children, congenital CMV continues to be underrecognized and underdiagnosed.3 Primary-care providers and pediatricians should discuss the importance of prevention strategies with their patients and should themselves be aware of the clinical presentation of CMV in order to identify symptoms that would warrant further evaluation. Primary-care providers have a critical role in CMV and cCMV prevention, diagnosis, and management, particularly as the lack of awareness about this infection persists.6 The involvement of these clinicians is imperative to breaking the silence about congenital CMV. n Ms. Piña is a provider at Pr1me Health & Wellness, Tulsa, Okla. Continues on page 34

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References 1. Griffiths PD. The Stealth Virus. CreateSpace/On-Demand Publishing LLC (Amazon);2012: chap 1, chap 2. 2. Thackeray R, Wright A, Chipman K. Congenital cytomegalovirus reference material: a content analysis of coverage and accuracy. Matern Child Health J. 2014;18(3):584-591. Available at link.springer.com/article/10.1007 %2Fs10995-013-1275-0 3. Swanson EC, Schleiss MR. Congenital cytomegalovirus infection: new prospects for prevention and therapy. Pediatr Clin North Am. 2013;60(2):335-349. Available at ncbi.nlm.nih.gov/pmc/articles/ PMC3807860/ 4. Yinon Y, Farine D, Yudin MH. Screening, diagnosis, and management of cytomegalovirus infection in pregnancy. Obstet Gynecol Surv. 2010;65(11):736-743. 5. Johnson J, Anderson B, Pass RF. Prevention of maternal and congeni-

“All we really know about him is that he likes cookies and has a foot fetish.”

tal cytomegalovirus infection. Clin Obstet Gynecol. 2012;55(2):521-530. Available at ncbi.nlm.nih.gov/pmc/articles/PMC3347968/ 6. Manicklal S, Emery VC, Lazzarotto T, et al. The “silent” global burden of congenital cytomegalovirus. Clin Microbiol Rev. 2013;26(1):86-102. Available at ncbi.nlm.nih.gov/pmc/articles/PMC3553672/ 7. Centers for Disease Control and Prevention. Cytomegalovirus (CMV) and congenital CMV infection. (Page last reviewed and updated June 5, 2013.) Available at cdc.gov/cmv/trends-stats.html 8. Centers for Disease Control and Prevention. Protect your baby. (Page last reviewed and updated June 23, 2014.) Available at cdc.gov/features/ cytomegalovirus/ 9. Gabbe SG, Niebyl JR, Simpson JL, et al. Maternal and perinatal infection - viral. Obstetrics: Normal and Problem Pregnancies. 6th ed. Philadelphia,

“Larry! No!

Pa.: Elsevier Saunders; 2012: chap 50. Available at www.mdconsult. com/books/page.do?eid=4-u1.0-B978-1-4377-1935-2..00050-8-s0250&isbn=978-1-4377-1935-2&type=bookPage&from=content&uniq Id=452463989-2 compensation for human cytomegalovirus-induced pathogenesis and a hypoxia-like condition in placentas with congenital infection. Am J Pathol. 2010;177(3):1298-1310. Available at ncbi.nlm.nih.gov/pmc/ articles/PMC2928963 11. Carlson A, Norwitz ER, Stiller RJ. Cytomegalovirus infection in pregnancy: should all women be screened? Rev Obstet Gynecol. 2010;3(4):172-179. Available at ncbi.nlm.nih.gov/pmc/articles/ PMC3046747/ 12. Nigro G, Adler SP. Cytomegalovirus infections during pregnancy. Curr Opin Obstet Gynecol. 2011;23(2):123-128. 13. The American College of Obstetricians and Gynecologists. ACOG practice bulletin. Perinatal viral and parasitic infections. Number 20, September 2000. Int J Gynaecol Obstet. 2002;76(1):95-107. All electronic documents accessed on November 15, 2014.

“Either the President’s in town or they’re selling zeppoles on Seventh Avenue.”

34 THE CLINICAL ADVISOR • DECEMBER 2014 • www.ClinicalAdvisor.com

10. Maidji E, Nigro G, Tabata T, et al. Antibody treatment promotes

CME CE

n LEARNING OBJECTIVES After completing the activity, the participant should be better able to: • Identify the primary and adjunctive therapies for obstructive sleep apnea • Explain the benefits, risks, and/or contraindications of the various treatments • Evaluate the effectiveness of a chosen treatment • Revise treatment plans as needed based on patient response to therapy n COMPLETE THE POSTTEST: Page 45

FEATURED COURSE

n ADDITIONAL CME/CE CREDIT: Page 66, 71, 77

HME seeks to verify that all scientific research referred to, reported, or used in a CME/CE activity conforms to the generally accepted standards of experimental design, data collection, and analysis. HME is committed to providing its learners with high-quality CME/CE activities that promote improvements in health care and not those of a commercial interest. The faculty reported the following financial relationships with commercial interests whose products or services may be related to the content of this CME activity:

Statement of Need: Obstructive sleep apnea (OSA), which has been associated with increased health-care utilization, is becoming more prevalent with rising obesity rates. OSA has been undertreated or mistreated in primary care, despite the availability of effective therapies. By familiarizing themselves with these treatments, primary-care providers can reduce the burden of OSA for individual patients and the health-care system as a whole.

Faculty Disclosures

Target Audience: This activity has been designed to meet the educational needs of primary-care health-care professionals who will treat persons presenting with symptoms of obstructive sleep apnea. Faculty Susan Collazo, RN, MSN, APN-CNP, thoracic surgery nurse practitioner Northwestern Memorial Hospital, Chicago, Ill. Accreditation Statements Physician Credit: HME is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. Credit Designation: HME designates this enduring material for a maximum of 0.5 AMA PRA Category 1 CreditTM. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Nursing Credit: MER is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. Credit Designation: This CE activity provides 0.5 contact hour of continuing nursing education. MER is a provider of continuing nursing education by the California Board of Registered Nursing, Provider #CEP 12299, for 0.5 contact hour. This activity qualifies for 0.25 pharmacotherapy credit. American Academy of Physician Assistants (AAPA) The AAPA accepts certificates of participation for educational activities certified for AMA PRA Category 1 Credit™ from organizations accredited by ACCME or a recognized state medical society. Physician assistants may receive a maximum of 0.5 hour of Category I credit for completing this program. Disclosure Policy In accordance with the ACCME Standards for Commercial Support, HME requires that individuals in a position to control the content of an educational activity disclose all relevant financial relationships with any commercial interest. HME resolves all conflicts of interest in an effort to ensure independence, objectivity, balance, and scientific rigor in all its educational programs. Furthermore,

Name of faculty

Reported Financial Relationship

Susan Collazo, RN, MSN, APN-CNP

No relevant financial relationships

Staff/Planners’ Disclosures The planners and managers for this program reported the following financial relationships with commercial interests whose products or services may be related to the content of this CME activity: HME planners and managers have no relevant financial relationships to disclose. MER planners and managers have no relevant financial relationships to disclose. Disclosure of Unlabeled Use: This educational activity may contain discussion of published and/or investigational uses of agents that are not indicated by the FDA. HME and MER do not recommend the use of any agent outside of the labeled indications. Please refer to the official prescribing information for each product for discussion of approved indications, contraindications, and warnings. Method of Participation: There are no fees for participating in and receiving CME/CE credit for this activity. During the period of December 10, 2014, through December 9, 2015, participants must: 1) read the learning objectives and faculty disclosures; 2) study the educational activity; 3) complete the posttest and submit it online (clinicians may register at www. myCME.com); and 4) complete the evaluation form online. A statement of credit will be issued only upon receipt of a completed activity evaluation form and a completed posttest with a score of 70% or better. Disclaimer: The content and views presented in this educational activity are those of the authors and do not necessarily reflect those of HME or MER. Any procedures, medications, or other courses of diagnosis or treatment discussed or suggested in this activity should not be used by clinicians without evaluation of their patient’s conditions and possible contraindications on dangers in use, review of any applicable manufacturer’s product information, and comparison with recommendations of other authorities. The information presented in this activity is not meant to serve as a guideline for patient management.

CME CE FEATURED COURSE: SUSAN COLLAZO, RN, MSN, APN-CNP

Obstructive sleep apnea: an overview of treatments With obstructive sleep apnea rates rising in tandem with obesity, primary-care providers should know about available therapeutic options.

he obesity epidemic has dramatically increased the prevalence of obstructive sleep apnea (OSA), which now affects approximately 60% of overweight persons (those with a body mass index, or BMI, ≥25 kg/m2).1 The link between OSA and obesity is further strengthened by findings in which a 10% reduction in body weight was associated with a parallel 26% reduction in apnea-hypopnea index (AHI);2 AHI is derived from the number of apneas and hypopneas during sleep divided by hours of sleep. Not only is the OSA patient population growing, but persons with this sleep disorder, in which airways become narrowed or blocked during sleep so that breathing pauses, have been found to have a higher health-care utilization rate than those without such a diagnosis, particularly in the year of diagnosis.3 With these factors taken together, it may be unrealistic to expect sleep specialists alone to manage this increased caseload of potential OSA patients,4 and researchers have shown that a primary-care treatment model has comparable effectiveness to a sleep-specialist treatment model.5 Primary-care clinicians should therefore become familiar with the various treatment modalities for OSA so that they can appropriately manage each individual patient.

Continuous positive airway pressure (CPAP) is the standard therapy for most cases of OSA.

Patient scenario: Mr. W, a 45 year-old truck driver with a BMI of 35 kg/m2, presents to your clinic with his wife. She reports that Mr. W snores and gasps during sleep—the most sensitive clinical indicators of possible obstructive sleep apnea (OSA). The patient himself notes that he has trouble staying awake while driving. Your physical examination www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • DECEMBER 2014 37

CME CE

FEATURED COURSE: OBSTRUCTIVE SLEEP APNEA

of Mr. W reveals a crowded oropharyngeal airway, a neck size of 18 inches, and hypertension (Table 1). Because you believe that the patient is at high risk for OSA, you send him to a sleep center to undergo polysomnography (sleep study). When you get the results, you look for Mr. W’s AHI, with 5 to 14 breathing pauses per hour indicating mild OSA, 15 to 29 breathing pauses per hour indicating moderate OSA, and 30 or more breathing pauses per hour indicating severe OSA.6 Mr. W’s AHI was severely high at 105, with significant oxygen desaturation (hypoxia) of 75%. Both of these indicators support a diagnosis of OSA. What is the best treatment for Mr. W? OSA should be approached as a chronic disease, requiring a multidisciplinary approach that covers medical, behavioral, and possible surgical options for proper treatment. The main goal of therapy is prevention or alleviation of upper-airway obstruction. Pretreatment patient education

Before any form of OSA therapy is initiated, the clinician should review with the patient the results of the polysomnogram and any other objective testing for OSA and sleepiness that has been performed. The educational program should include discussion of the pathophysiology of OSA, risk factors, and possible health consequences of untreated OSA (Table 2). Treatment options should be discussed in the context of the severity of OSA in that particular patient, possible complications associated with a given treatment, and the importance of treatment compliance in improving health and quality of life. The patient should be given general information on weight loss, smoking cessation, reduction of alcohol intake, medication effects, and sleep position. Videos, including those accessible through online sites such as YouTube.com, as well as handouts and brochures can help the patient learn more about OSA and become less apprehensive about treatment. Positive airway pressure

According to the Adult Obstructive Sleep Apnea Task Force of the American Academy of Sleep Medicine, positive airway pressure (PAP) is the treatment of choice for mild, moderate, or severe OSA and should be offered to all patients.7 Introduced by Sullivan and colleagues in 1981,6,8 PAP provides pneumatic splinting of the upper airway during sleep by delivering pressurized air through a device—usually a face mask or a nasal mask—worn by the patient.8 The device is connected by flexible tubing to a portable PAP machine at the person’s bedside. Apnea, a breathing cessation of at least 10 seconds, is associated with a decrease in blood oxygenation. Hypopnea is a disruption of air flow of at least 30% during sleep or an arousal during sleep. The combination of apnea and hypopnea

TABLE 1. When to suspect obstructive sleep apnea (OSA) Patient complains of daytime sleepiness. Patient’s bed partner reports loud snoring by patient. Patient is obese (BMI ≥30 kg/m2). Physical examination reveals conditions that narrow the upper airway, such as a crowded pharynx with a low-lying uvula and soft palate. Physical examination reveals large tonsils, a retrognathic mandible (overbite), and/or a neck circumference of more than 17 inches in men or more than 16 inches in women. Child patient has enlarged tonsils and/or adenoids. Patient has diabetes or prediabetes, conditions that render the person up to 3 times more likely than those in the general population to have OSA. Patient has a history of congestive heart failure, atrial fibrillation, treatment-refractory hypertension, or pulmonary hypertension. Adapted from Collazo S. Identifying obstructive sleep apnea. The Clinical Advisor. 2014;17(9):36-44.

causes the blood level of carbon dioxide to increase and the blood level of oxygen to decrease, leading to a disruptive pattern of breathing during that period of sleep. AHI and oxygen desaturation levels are used to indicate the severity of obstructive sleep apnea; PAP therapy reduces AHI.7 Mr. W has a severely high AHI with oxygen desaturation. This makes him an ideal candidate for PAP therapy. PAP can be delivered in different modes: continuous (CPAP), bilevel (BPAP), or autotitrating (APAP).9 CPAP delivers a fixed mild positive pressure during inspiration and expiration to maintain airway openness. In patients who cannot tolerate a fixed positive pressure during exhalation, BPAP delivers a higher pressure during inspiration and a lower pressure during exhalation. APAP has variable inspiratory and expiratory pressures as it continuously monitors the patient’s breathing pattern and delivers pressures accordingly. CPAP is the standard treatment option for moderate to severe cases of OSA and also represents a good treatment option for mild sleep apnea.6 A polysomnogram can be administered as a split-night study to allow for initial diagnosis of OSA followed by CPAP titration the same night. CPAP can be delivered through a mask or through nasal pillows, which are plastic inserts placed directly under the nose and held in place by straps that cover less of the face than does the CPAP face mask. Nasal pillows might be the better choice for patients who find the CPAP mask too uncomfortable or unattractive, or who find that their mask leaks air.

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

TABLE 2. Checklist of key components of OSA patient-education discussion Review with the patient his or her polysomnogram results and other objective findings related to OSA. Explain the pathophysiology of OSA. Discuss the possible correlation between the person’s individual risk factors and his/her clinical symptoms.

treatment in persons who have mild to severe OSA, with high patient acceptance and compliance.11 Users of EPAP may have difficulty breathing since they need to sleep with an adhesive to the nose and experience increased resistance during expiration. Patients with any nasal problems are not candidates for EPAP therapy. Further research is needed before EPAP therapy can be recommended for use in the pediatric population. Positional therapy

Point out the possible consequences of untreated OSA. Describe the treatment options. Clarify the anticipated outcomes of various treatments. Outline the possible complications of treatment. Ask the patient to share any concerns and questions. Adapted from Epstein LJ, Kristo D, Strollo PJ, et al.; Adult Obstructive Sleep Apnea Task Force of the American Academy of Sleep Medicine. Clinical guideline for the evaluation, management and long-term care of obstructive sleep apnea in adults. J Clin Sleep Med. 2009;5(3):263-276. Available at aasmnet.org/Resources/clinicalguidelines/OSA_Adults.pdf

Patient complaints of congestion, rhinorrhea, sneezing, or other nasal symptoms due to CPAP therapy are common.10 Humidification of the CPAP air has been shown to reduce the frequency of nasal symptoms in some persons with OSA.10 The patient can also be prescribed nasal saline or nasal decongestants including nasal steroids. Persons with persistent nasal symptoms during PAP therapy should be referred to a sleep specialist or to a specialized respiratory therapist for reevaluation of the PAP setting and mask fittings. Other side effects of PAP therapy include skin irritation as well as mask allergies, particularly for persons with a latex allergy. In addition, portability of the CPAP machine may be a concern. For example, a patient such as Mr. W, who drives a truck for a living, might find it difficult to transport and operate a CPAP machine while on the road. Because compliance with this effective form of primary therapy is essential, and the therapy is long-term, providers should make it a priority to address any patient concerns regarding CPAP during an office visit shortly after the treatment is prescribed and take measures to prevent or minimize any side effects (Table 3). Nasal expiratory positive airway pressure (EPAP) is an OSA treatment that utilizes the patient’s own breathing to create positive airway pressure and prevent obstructed breathing. This treatment employs a device consisting of a small valve attached externally to each nostril with hypoallergenic adhesive. Nasal EPAP has been shown to provide significant reductions in AHI and improve subjective daytime sleepiness compared with sham

For many patients, sleep apnea is worse when lying on the back. For example, Oksenberg and colleagues found in their study of 100 consecutive adult patients with OSA that the supine position was associated with increased frequency of apneas and hypopneas during the rapid eye movement (REM) stage of sleep, although duration of these episodes did not increase.12 Gravity, airway anatomy, airway critical closing pressures, and effects on upper-airway dilator muscle function all come into play when a person is in the supine position and can aggravate OSA. In normal sleep, parasympathetic activity is enhanced and the muscle tone of the upper airway is decreased. A healthy person can maintain a patent airway and adequate airflow during sleep. A patient with OSA has a smaller, more collapsible upper airway during sleep, resulting in apneic or hypopneic events and often causing snoring from upper-airway tissue vibration.13 Positional therapy involves the use of assistive devices to help a patient avoid the supine position during sleep. Studies noting the efficacy of positional therapy are lacking or have not been consistent. Sleeping on one’s side is often advised to assist in decreasing the risk of OSA.6 Other simple sleeping strategies include the following: • Have the person sew a small pocket to the back of the pajamas and place a tennis ball or other small ball into it, to keep the person off of his or her back during sleep. • Have the person use a special pillow that helps to stretch the neck; this may reduce snoring and improve sleep for people with mild sleep apnea. • Have the person sleep in an upright position; this may improve oxygen levels in overweight people with sleep apnea. Elevating the head of the bed may help.6 Weight management

Weight loss can significantly reduce OSA symptoms and should be recommended for all overweight persons with OSA, as illustrated by results reported by Foster and fellow members of the Sleep AHEAD Research Group, part of the Look AHEAD Research Group for the study of intensive lifestyle interventions for overweight persons with diabetes.14 In the randomized trial

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CME CE

FEATURED COURSE: OBSTRUCTIVE SLEEP APNEA

Three times as many weight-loss participants had total remission of OSA, and severe OSA was only half as prevalent in the weight-loss group. conducted by Foster’s team, 264 adults (mean age 61.2 years) with type 2 diabetes, mean BMI 36.7 kg/m2, and mean AHI 23.2 events per hour were assigned to a behavioral weight-loss program or to 3 group sessions related to effective diabetes management. At 1 year, after losing significantly more weight than the diabetes-management group (10.8 kg vs. 0.6 kg), the weight-loss group demonstrated an adjusted decrease in AHI of 9.7 events per hour relative to the diabetes-management group. In addition, 3 times as many weight-loss participants experienced total remission of OSA, and severe OSA was only half as prevalent in the weight-loss group as in the diabetesmanagement group. The investigators noted that persons who lost 10 kg or more had the greatest reductions in AHI. Weight loss should occur in conjunction with primary treatment of OSA. Once substantial weight loss is achieved, a follow-up polysomnogram is indicated to assess for residual

OSA, especially in patients who had low oxygen saturation levels and high supine AHI before weight loss.15 Clinicians should be aware that within the global epidemic of childhood and adolescent obesity, up to 60% of obese children have OSA.16 Early recognition and treatment of OSA in obese youths are likely to lead to a reduction in the cardiometabolic burden of these patients.16 In addition to following healthy eating habits, children and adolescents generally should engage in at least 60 minutes of moderately intense physical exercise per day to prevent obesity or maintain weight.16 Almost half of all obese children with OSA have adenotonsillar hypertrophy; as a result, the American Board of Pediatrics recommends adenotonsillectomy as the first step in OSA management.16 This surgery leads to improvement of obstructive symptoms in 80% of cases in otherwise normal children with OSA, but is less likely to be successful in morbidly obese children.16

TABLE 3.Troubleshooting side effects of CPAP and oral-appliance treatments for OSA Treatment

Side effects

Solution

Continuous positive airway pressure (CPAP)

Mask allergies

• Ask patient about latex allergy.

Skin irritation

• Have patient try nasal pillows. • Discuss with sleep specialist.

Dry mouth

• Blend heated humidifier into CPAP machine. • Have patient use chin strap to keep mouth closed. • Have patient use a different type of mask. • Discuss with sleep specialist.

Nasal congestion Sinusitis Epistaxis (nosebleed)

• Blend heated humidifier into CPAP machine. • Ensure patient’s CPAP mask is well-fitted. • Have patient use nasal saline at bedtime. • Discuss possible use of steroid nasal spray with sleep specialist.

Aerophagia (air swallowing)

• Decrease CPAP pressure (discuss with sleep specialist). • Have patient lower head of bed or flatten sleeping position. • Have patient try taking a proton pump inhibitor.

No improvement in symptoms

• Have patient keep sleep diary to document: —— tolerance —— side effects (e.g., nasal symptoms) —— daytime symptoms (e.g., sleepiness) —— nocturnal awakenings • Encourage CPAP compliance and discuss with sleep specialist.

Excessive salivation Dry mouth Tooth and jaw discomfort Temporomandibular joint (TMJ) problems Temporary bite changes Tongue pain Gagging

• Adjust oral appliance. • Discuss with dental sleep specialist. • Monitor children for dental-skeletal changes. • Investigate other treatment options, such as CPAP, if side effect continues.

Oral appliances

Compiled by Susan Collazo, RN, MSN, APN-CNP

40 THE CLINICAL ADVISOR • DECEMBER 2014 • www.ClinicalAdvisor.com

No pharmacologic therapy has proven to prevent or overcome upper-airway obstruction enough to be considered a primary treatment for OSA. Bariatric surgery was described as “a definitive treatment for obstructive sleep apnea” by the authors of a systematic review involving 69 studies and 13,900 patients.17 Undertaken by Sarkhosh and associates to determine which of the available bariatric procedures were the most effective in the treatment of OSA, the review indicated that more than 75% of patients had at least some improvement in OSA, with biliopancreatic diversion being the most successful bariatric procedure in improving or resolving OSA and laparoscopic adjustable gastric banding being the least successful. Clinical practice guidelines for bariatric surgery cosponsored by the American Association of Clinical Endocrinologists, The Obesity Society, and the American Society for Metabolic and Bariatric Surgery list OSA as a severe obesity-related comorbidity, with the recommendation being that a patient with OSA and with a BMI ≥35 kg/m 2 be offered bariatric surgery.18 The guideline authors caution, however, that OSA is associated with increased risk for all-cause mortality and in bariatric surgery patients, with adverse outcomes. Bariatric surgery is rarely recommended for children unless the child is morbidly obese (BMI >40 kg/m2, or has a BMI of 35-40 kg/ m2 with coexisting diseases.16 Other nonsurgical approaches should be exhausted before bariatric surgery is considered for the treatment of OSA in children. Cigarette smoking and other behaviors

Because cigarette smoking is an independent risk factor for snoring and may be associated with OSA, smoking cessation is an essential part of OSA therapy.19 Smoking is known to increase inflammation and fluid retention in the upper airway, which can further aggravate OSA. Persons with OSA also should be advised to avoid alcohol, sleeping pills, and other sedatives, as these substances can relax throat muscles and contribute to airway collapse during sleep. Oral appliances

Some patients may be candidates for customized nighttime oral or dental appliances to reduce snoring and apnea. These devices can enlarge the upper airway and/or decrease airway collapsibility, thus improving OSA.20 Mandibular advancement devices (MADs) move the lower jaw forward slightly, which tightens the soft tissue and muscles of the upper airway to prevent obstruction of the airway during sleep (Figure 1). Evidence has shown CPAP to reduce AHI and arousal index (number of arousals per hour during sleep) scores and increase

oxygen saturation to a greater degree than do MADs.21 In 2013 the American College of Physicians (ACP) recommended CPAP as initial therapy for persons with a diagnosis of OSA, but also recommended MADs as an alternate therapy to patients who prefer MADs over CPAP or who experience adverse effects with CPAP.21 In the extensive literature review that formed the basis of the ACP recommendations, the most commonly reported adverse effects with MADs were tooth loosening, dental crown damage, and temporomandibular joint (TMJ) pain. No long-term consequences were reported. According to practice parameters established by the American Academy of Sleep Medicine, oral appliances for the treatment of snoring or OSA should be fitted and checked regularly by qualified dental personnel.22 (Dental sleep medicine specialists can be located through the American Academy of Dental Sleep Medicine website: aadsm.org.) Device efficacy should be assessed by means of follow-up polysomnography or an attended cardiorespiratory sleep study.22 Such testing also may be needed if OSA symptoms worsen or recur. Oral appliance therapy reportedly relieves apnea in 20% to 70% of patients, and reduces AHI to normal in 50% to 60% of patients.23 These devices have been reported to be well-tolerated by most patients, but may cause frequent yet generally minor and temporary side effects, including but not limited to excessive salivation, dry mouth, gum irritation, tooth pain, TMJ pain, myofascial pain, and bite changes.20 Pharmacologic therapy

Numerous pharmacotherapeutic approaches have been investigated for the treatment of OSA but none have proven to prevent or overcome upper-airway obstruction enough to justify this approach as a primary treatment for OSA.1,7 Thus, pharmacologic treatment is considered adjunctive rather than primary therapy. An example is the use of pharmacologic therapy for residual symptoms of excessive daytime sleepiness that persists despite the patient’s use of CPAP at night.24 Before prescribing any form of adjunctive pharmacotherapy for OSA, the clinician should determine whether the patient has indeed been compliant with primary therapy. The clinician also should assess the patient for additional factors that can contribute to poor sleep before considering pharmacotherapy, including having one or more of the following:7,24 • ill-fitting PAP mask • poor sleep hygiene • depression

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FEATURED COURSE: OBSTRUCTIVE SLEEP APNEA

CME CE

FIGURE 1. Mandibular advancement device moves lower jaw forward.

FIGURE 2. Removal of enlarged tonsils can improve OSA symptoms.

• thyroid disease • other sleep disorders (e.g., restless leg syndrome, narcolepsy). Only two drugs are FDA-approved for the treatment of residual daytime somnolence: modafinil24,25 and armodafinil.26 Both of these wakefulness-promoting agents are oral medications that have properties similar to amphetamines and other sympathomimetic agents, but their mechanisms of action are unknown. Modafinil and armodafinil should be used in conjunction with PAP therapy.7 In addition to considering the patient’s cardiovascular health and other medical conditions that might preclude the use of modafinil or armodafinil,25,26 providers should also know that these medications may affect the user’s judgment and cognitive abilities. Persons taking these medications should be cautioned not to drive and not to engage in other potentially dangerous activities until they can determine how the drug affects them.25,26 Both agents may be habit-forming as well. Clinicians should inform female patients of childbearing age that modafinil and armodafinil may also reduce the effectiveness of hormonal contraceptives.

but also may increase episode duration.7,27 In addition, the use of supplemental oxygen used alone can worsen nocturnal hypercapnia in individuals who have comorbid respiratory disease.7

Supplemental oxygen

Supplemental oxygen is not recommended as a primary treatment for OSA, but may have an adjunctive role in treating hypoxemia.7,27 This treatment can help overcome oxygen desaturation and may reduce the frequency of apnea episodes,

Structural surgery

Craniofacial and upper-airway structure, including the presence of mandibular or maxillary dysmorphisms, can contribute significantly to the development of OSA.28 In one study of 142 men without OSA (mean age 47 years; mean BMI 29 kg/m2) and 62 men with OSA (mean age 47 years; mean BMI 32 kg/m2), relative narrowness in the horizontal dimension of the maxilla was found to be the most important cephalometric measure in predicting AHI severity.28 In children, enlarged tonsils and adenoids can cause abnormal growth patterns of the lower face and jaw, predisposing these youths to OSA. In the recent Childhood Adenotonsillectomy Trial (CHAT), 464 children aged 5 to 9 years with OSA were randomized to early adenotonsillectomy or to watchful waiting.29 At 7 months, the surgery group exhibited significantly greater improvements than did the watchful-waiting group in behavioral, quality-of-life, and polysomnographic findings and significantly greater reductions in symptoms and AHI. This improvement was attributed to lymphoid tissue regression, airway growth, and routine medical care. Adenotonsillectomy did not, however, significantly improve attention or executive function as measured by neuropsychologic testing.

42 THE CLINICAL ADVISOR • DECEMBER 2014 • www.ClinicalAdvisor.com

Individuals with OSA and anatomic obstruction, such as tonsillar hypertrophy that obstructs a pharyngeal airway, should be considered candidates for surgery when PAP therapy or oral appliances are ineffective or are declined. Numerous surgical approaches exist: • Nasal surgery, including turbinectomy (removal of nasal turbinates) or correction of nasal septal deviation; polypectomy; rhinoplasty • Tonsillectomy and/or adenoidectomy (Figure 2) • Uvulopalatopharyngoplasty (UPPP)—reduction or removal of the uvula, the soft palate, the tonsils, the adenoids, and/or the pharynx. (Laser-assisted uvulopalatoplasty [LAUP] is not routinely recommended in OSA as this procedure does not generally normalize AHI and has not resulted in significant improvements in secondary outcomes.30) • Radiofrequency ablation to reduce targeted tissue • Tracheostomy—the most consistently effective surgical intervention for OSA yet also the last resort: It alters the patient’s appearance and places the patient at risk for aspiration, pneumonia, and vocal cord paralysis. Benefits include statistically significant decreases in AHI, hypopneas, and mortality.31 • Maxillomandibular advancement (MMA) is the most effective surgical treatment for OSA excluding tracheostomy.32 However, this procedure leads to facial changes that may be unacceptable to patients with bimaxillary protrusion, a common feature of Asian faces. Liao et al. found that a modified MMA procedure consisting of osteotomies and postsurgical orthodontics was effective in treating OSA without affecting facial appearance or dental occlusion in Asians.32

Outcomes assessment

Regardless of the form of OSA treatment chosen, a proper outcomes assessment should be performed following initiation of therapy (Table 4). Outcome indicators include resolution of daytime somnolence, improved scores on objective testing such as on the Epworth Sleepiness Scale, patient reports of feeling more alert and more awake during the day, adherence to prescribed therapy, weight loss, and avoidance of factors that exacerbate OSA such as smoking and alcohol use. The patient’s bed partner can confirm whether the patient has had less snoring, less restlessness, and fewer nighttime awakenings. Persons undergoing treatment for OSA who continue to experience excessive daytime somnolence or other residual symptoms should be referred to a sleep specialist. Conclusion

OSA treatment is aimed at alleviating airway obstruction through numerous approaches including PAP, positional therapy, weight management, smoking cessation and other behavioral changes, oral appliances, and surgical procedures. Each OSA patient should be followed closely to ensure adherence to primary therapy. The primary-care provider is an integral part of a multidisciplinary approach of OSA treatment and effective outcomes management. n Ms. Collazo, who specializes in thoracic surgery at Northwestern Memorial Hospital in Chicago, is experienced in pulmonary medicine and internal medicine. References 1. Schwartz AR, Susheel PP, Laffan AM, et al. Obesity and obstructive sleep apnea: pathogenic mechanisms and therapeutic approaches. Proc Am Thorac Soc. 2008;5(2):185-192. Available at atsjournals.org/doi/pdf/10.1513/

TABLE 4. OSA treatment-outcome indicators

pats.200708-137MG 2. Peppard PE, Young T, Palta M, et al. Longitudinal study of moderate

Resolution of daytime sleepiness

weight change and sleep-disordered breathing. JAMA. 2000;284(23):3015– 3021. Available at jama.jamanetwork.com/article.aspx?articleid=193382

Improvements on Epworth Sleepiness Scale and/or other objective tests

3. Diaz K, Faverio P, Hospenthal A, et al. Obstructive sleep apnea is

Patient self-reports of increased alertness/wakefulness during day

associated with higher healthcare utilization in elderly patients. Ann Thorac Med. 2014;9(2):92-98. Available at thoracicmedicine.org/temp/ AnnThoracMed9292-2442842_064708.pdf

Bed-partner reports of less snoring, less restlessness, and fewer nighttime awakenings by patient

4. Fleishman SA. Response to article on: Primary care vs specialist sleep center management of OSA and daytime sleepiness and quality of life.

Adherence to therapy

JAMA. 2013;310(1):97. Available at jama.jamanetwork.com/article.aspx?artic leid=1707712&resultClick=3

Weight loss

5. Chai-Coetzer CI, Antic NA, Rowland LS, et al. Primary care vs specialist

Avoidance of factors exacerbating OSA, such as smoking and alcohol

sleep center mananagement of obstructive sleep apnea and daytime sleepiness and quality of life. A randomized trial. JAMA. 2013;309(10):997-1004. Available

www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • DECEMBER 2014 43

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FEATURED COURSE: OBSTRUCTIVE SLEEP APNEA

at jama.jamanetwork.com/article.aspx?articleid=1667091&resultClick=3

20. Ferguson KA, Cartwright R, Rogers R, Schmidt-Nowara W. Oral

6. American Academy of Sleep Medicine. Obstructive sleep apnea (fact

appliances for snoring and obstructive sleep apnea: a review. Sleep. 2006;

sheet). 2008. Available at aasmnet.org/resources/factsheets/sleepapnea.pdf

29(2):244-262. Available at aasmnet.org/resources/practiceparameters/

7. Epstein LJ, Kristo D, Strollo PJ, et al.; Adult Obstructive Sleep Apnea

review_oralapplianceosa.pdf

Task Force of the American Academy of Sleep Medicine. Clinical guideline

21. Qaseem A, Holty J-EC, Owens DK, et al.; Clinical Guidelines

for the evaluation, management and long-term care of obstructive sleep

Committee of the American College of Physicians. Management of

apnea in adults. J Clin Sleep Med. 2009;5(3):263-276. Available at aasmnet.

obstructive sleep apnea in adults: a clinical practice guideline from the

org/Resources/clinicalguidelines/OSA_Adults.pdf

American College of Physicians. Ann Intern Med. 2013;159(7):471–483.

8. Sullivan CE, Berthon-Jones M, Issa FG, Eves L. Reversal of obstructive

Available at annals.org/article.aspx?articleid=1742606&resultClick=3

sleep apnoea by continuous positive airway pressure applied through the

22. Kushida CA, Morgenthaler TI, Littner MR, et al.; American Academy

nares. Lancet. 1981;317(8225):862-865.

of Sleep. Practice parameters for the treatment of snoring and obstruc-

9. Kryger MH, Malhotra A. Management of obstructive sleep apnea in

tive sleep apnea with oral appliances: an update for 2005. Sleep.

adults. UpToDate. Available at uptodate.com/contents/management-of-

2006;29(2):240-243. Available at aasmnet.org/resources/practiceparam-

obstructive-sleep-apnea-in-adults; topic updated October 15, 2014.

eters/pp_update_oralapplicance.pdf

10. Ryan S, Doherty LS, Nolan GM, McNicholas WT. Effects of heated

23. Padma A, Ramakrishnan N, Narayanan V. Management of obstructive

humidification and topical steroids on compliance, nasal symptoms, and

sleep apnea: a dental perspective. Indian J Dent Res. 2007;18(4):201-209.

quality of life in patients with obstructive sleep apnea syndrome using nasal

24. Morgenthaler TI, Kapen S, Lee-Chiong T, et al. Practice parameters for

continuous positive airway pressure. J Clin Sleep Med. 2009;5(5):422-427.

the medical therapy of obstructive sleep apnea. Sleep. 2006;29(8):1031-

Available at ncbi.nlm.nih.gov/pmc/articles/PMC2762712/#__ffn_sectitle

1035. Available at aasmnet.org/Resources/PracticeParameters/PP_

11. Berry RB, Kryger MH, Massie CA. A novel nasal expiratory positive

MedicalTherapyOSA.pdf

airway pressure device for the treatment of obstructive sleep apnea: a

25. National Institutes of Health. MedlinePlus: Modafinil. Page last

randomized controlled trial. Sleep. 2011;34(4):479-485. Available at ncbi.

reviewed November 20, 2012. Available at nlm.nih.gov/medlineplus/dru-

nlm.nih.gov/pmc/articles/PMC3065258/

ginfo/meds/a602016.html

12. Oksenberg A, Arons E, Nasser K, et al. REM-related obstructive sleep

26. National Institutes of Health. MedlinePlus: Armodafinil. Page last

apnea: The effect of body position. J Clin Sleep Med. 2010;6(4):343–348.

reviewed November 20, 2012. Available at nlm.nih.gov/medlineplus/dru-

Available at ncbi.nlm.nih.gov/pmc/articles/PMC2919664/

ginfo/meds/a602016.html

13. Mehra R. Sleep apnea ABCs: airway, breathing, circulation. Cleve Clin J

27. Victor LD. Treatment of obstructive sleep apnea in primary care. Am Fam

Med. 2014;81(8):479-489. Available at ccjm.org/content/81/8/479.full

Physician. 2004;69(3):561-569. Available at aafp.org/afp/2004/0201/p561.html

14. Foster GD, Borradaile KE, Sanders MH, et al.; Sleep AHEAD Research

28. Dempsey JA, Skatrud JB, Jacques AJ, et al. Anatomic determinants of

Group of the Look AHEAD Research Group. A randomized study on the

sleep-disordered breathing across the spectrum of clinical and nonclinical

effect of weight loss on obstructive sleep apnea among obese patients with

male subjects. Chest. 2002;122(3):840-851. Available at journal.publica-

type 2 diabetes: the Sleep AHEAD study. Arch. Intern. Med. 2009;169(17):1619-

tions.chestnet.org/data/Journals/CHEST/21982/840.pdf

1626. Available at archinte.jamanetwork.com/article.aspx?articleid=224770

29. Marcus CL, Moore RH, Rosen CL, et al.; Childhood Adenotonsillectomy

15. Bae EK, Lee YJ, Yun CH, Heo Y. Effects of surgical weight loss for treating

Trial (CHAT). A randomized trial of adenotonsillectomy for childhood sleep

obstructive sleep apnea. Sleep Breath. 2014 (July 16; Epub ahead of print).

apnea. N Engl J Med. 2013;368(25):2366-2376. Available at nejm.org/doi/

16. Narang I, Mathew JL. Childhood obesity and obstructive sleep apnea. J

full/10.1056/NEJMoa1215881

Nutr Metab. 2012;2012:134202. Available at ncbi.nlm.nih.gov/pmc/articles/

30. Aurora RN, Casey KR, Kristo D, et al. Practice parameters for the

PMC3432382/

surgical modifications of the upper airway for obstructive sleep apnea in

17. Sarkhosh K, Switzer NJ, El-Hadi M, et al. The impact of bariatric surgery on

adults. Sleep. 2010;33(10):108-1413. Available at aasmnet.org/Resources/

obstructive sleep apnea: a systematic review. Obes Surg. 2013;23(3):414-423.

PracticeParameters/PP_SurgicalModificationsOSA.pdf

18. Mechanick JI, Youdim A, Jones DB, et al. Clinical practice guide-

31. Camacho M, Certal V, Brietzke SE, et al. Tracheostomy as treatment

lines for the perioperative nutritional, metabolic, and nonsurgical sup-

for adult obstructive sleep apnea: a systematic review and meta-analysis.

port of the bariatric surgery patient—2013 update: cosponsored by

Laryngoscope. 2014;124(3): 803-811.

American Association of Clinical Endocrinologists, The Obesity Society,

32. Liao YF, Chiu YT, Lin CH, et al. Modified maxillomandibular advance-

and American Society for Metabolic & Bariatric Surgery. Endocr Pract.

ment for obstructive sleep apnoea: towards a better outcome for Asians.

2013;19(2):337-372. Available at guideline.gov/content.aspx?id=47785

Int J Oral Maxillofac Surg. 2014 (October 8; Epub ahead of print).

19. Lin YN, Li QY, Zhang XJ. Interaction between smoking and obstructive sleep apnea: not just participants. Chin Med J (Engl). 2012;125(17):3150-3156.

All electronic documents accessed on November 15, 2014.

44 THE CLINICAL ADVISOR • DECEMBER 2014 • www.ClinicalAdvisor.com

CME CE

POSTTEST Expiration date: December 9, 2015

FEATURED COURSE: Obstructive sleep apnea CREDITS: 0.5

For more credit information, please turn to p. 36.

1. Mr. P, aged 54 years, suffers from chronic sinusitis as well as from obstructive sleep apnea (OSA). With an apneahypopnea index (AHI) of 24, Mr. P is considered to have moderate OSA, yet his symptoms are mild. His regular routine of running and strength-training keeps him at a trim 175 lb, and he feels awake and alert during the day but has frequent nocturnal awakenings and he snores “like a locomotive,” according to his wife. He has been using continuous positive airway pressure (CPAP) therapy for his sleep apnea, but dislikes the “trapped” feeling he says he gets when wearing the mask. He inquires about the nasal expiratory positive airway pressure (EPAP) device. How can you best address this patient’s inquiry? a. Advise the patient against the use of EPAP as it has not been found to reduce AHI. b. Switch the patient to EPAP as soon as possible because it is more effective than CPAP. c. Explain to the patient that the near-constant nasal congestion he suffers as part of his chronic sinusitis makes him a poor candidate for EPAP. d. Caution the patient that the EPAP device is likely to cause a strong skin reaction where it is secured to the nose. 2. Mr. R, a 62-year-old smoker with a body mass index (BMI) of 35 kg/m2 , recently learned he has OSA in addition to a temporomandibular joint (TMJ) disorder. He is tolerating bilevel positive airway pressure (BPAP), yet he still feels more fatigued during the day than he thinks is reasonable. He also has some myofascial discomfort due to the TMJ but is hesitant to use any pain-relieving agents due to his overuse of prescription painkillers 5 years ago. He knows weight loss will enhance the effectiveness of his PAP therapy and will make him feel better overall, and he has even joined a gym, but has not yet seen any results. In addition to offering Mr. R more information on weightmanagement methods, what adjunct strategy might you suggest that could relieve his daytime fatigue sooner?

a. Have the patient use a mandibular advancement device. b. Prescribe modafinil to counteract the patient’s daytime somnolence. c. Recommend that the patient try sleeping in the supine position to take pressure off of his jaw and to leave his mouth more relaxed for BPAP. d. Advise the patient to quit smoking. 3. Ms. A, aged 45 years, recently switched from a CPAP mask to nasal pillows after consulting with a home respiratory therapist. Ms. A tolerated this form of treatment better than she did the mask, but due to her vasomotor rhinitis, she has been awakening every day with nasal stuffiness or epistaxis. She typically exercises each morning to maintain her BMI of 20 kg/m2 , and these nasal symptoms have been disrupting that schedule. Which course of action is most appropriate? a. Have the patient use a nasal saline spray every night before applying the nasal pillows. b. Advise the patient to use the nasal pillows only every other night instead of every night. c. Explain to the patient that she is going to have to go back to using the CPAP mask instead of the nasal pillows. d. Have the patient stop using the nasal pillows and prescribe supplemental oxygen for her instead. 4. Your OSA patient has lost 45 lb following bariatric surgery 6 months ago, and she expects to lose at least 35 lb more. She reports being much more energetic, alert, and happier now, and asks to be taken off of CPAP therapy. How should you respond? a. Agree to wean the patient off of CPAP therapy since she most likely does not have OSA. b. Tell the patient to stop CPAP therapy and see if OSA symptoms return. c. Make no determinations about the patient’s treatment until her weight has stabilized in about 1 more year. d. Order a polysomnogram for the patient.

TO TAKE THE POSTTEST please go to: myCME.com/Dec14CAfeature

www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • DECEMBER 2014 45

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 DECEMBER 2014

The Consultant Files Yellow lights, digoxin toxicity. . . . . . . 52 Not all clinicians are aware of this classic sign of digoxin toxicity.

Your Comments Think broadly for abdominal pain. . . . 52 A provider offers her own take on how she would have handled a patient with hepatitis C, fatty pancreas, diabetes, and chronic abdominal pain.

More thoughts on craniosacral therapy, from the founder’s son. . . . . . . 53 John Matthew Upledger responds to our article on the practice pioneered by his father.

We would like to hear from you! Please send your clinical questions, pearls, and stories that can inform and improve daily practice for your colleagues to editor@ClinicalAdvisor.com. We will publish all appropriate submissions here in Advisor Forum.

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 editor@ 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.

THE CONSULTANT FILES The Clinical Advisor asked our Advisor Forum consultants what interesting cases they have encountered themselves.

YELLOW LIGHTS, DIGOXIN TOXICITY Contributed by Kim Zuber, PA-C, who oversees patients in 7 dialysis centers for Metropolitan Nephrology Associates, Clinton, Md. We had an older female with chronic kidney disease who started complaining about seeing yellow lights and being short of breath. She had a history of heart failure and was treated with digoxin. When we examined the patient, she was hypotensive and bradycardic. We sent her straight over to the emergency room, where her digoxin level was found to be 5 ng/mm (therapeutic goal: 0.8 to 2.0 ng/mm). She was admitted to the intensive-care unit and treated with digoxin immune fab to bring her level back to normal. While we all knew that digoxin must be renally dosed, only one of us knew the “classic” vision abnormalities seen with digoxin toxicity—purple or yellow lights. Now we all know it. (194-1)

YOUR COMMENTS THINK BROADLY FOR ABDOMINAL PAIN In the reader’s question, “Pain in a person with hepatitis C and a fatty pancreas” (Advisor Forum item 192-3, October 2014;

OUR CONSULTANTS

Philip R. Cohen, MD,

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.

Abimbola Farinde, PhD, PharmD,

is a professor at Columbia Southern University in Orange Beach, Ala.

Abby A. Jacobson, PA-C,

Debra August King, PhD, PA,

is a physician assistant at Delaware Valley Dermatology Group in Wilmington, Del.

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

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

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11/21/14 11:22 AM

available at ClinicalAdvisor.com/pain-in-a-person-withhepatitis-c-and-a-fatty-pancreas/article/374261, accessed on November 15, 2014), the expert’s answer was given as possibly caused by pancreatitis or cirrhosis. However, being that the patient’s liver function tests and lipase levels were normal, this is highly unlikely. Neither fatty liver, fatty pancreas, nor cirrhosis in and of themselves cause abdominal pain regardless of etiology, and pancreatitis is by definition inflammatory—evidenced by elevated lipase. With any type of chronic abdominal pain, think broadly. No other history or associated symptoms were given, but in this diabetic patient, one must consider gastroparesis, chronic gastritis (e.g., Helicobacter pylori), peptic ulcer disease, biliary dysfunction, and ischemia. The expert suggested endoscopic ultrasound, but this would be no more revealing as to the cause of pain than simple abdominal ultrasound (looking for gallstones, mass lesion, thrombosis). If the cause isn’t better defined by a more complete history (timing, provocative factors, associated symptoms, social/diet history, review of symptoms), I would give this patient a strict anti-reflux diet (good for any gastrointestinal symptoms), put him on a daily proton pump inhibitor (which, with varices, he should be taking anyway), add sucralfate 3 times a day (making sure to precaution for constipation, which can trigger encephalopathy), check a stool antigen for H. pylori, and order a HIDA scan looking for biliary dyskinesia (extremely common in end-stage liver disease). If results are all normal, I’d proceed with a gastric-emptying study, looking for gastroparesis.—ANNE WALSH, PA-C, MMSc, Los Angeles (194-2)

MORE THOUGHTS ON CRANIOSACRAL THERAPY, FROM THE FOUNDER’S SON John Matthew Upledger, CEO of Upledger Institute International and son of craniosacral therapy pioneer John E. Upledger, DO,

Mary Newberry, CNM, MSN,

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

responds to the Alternative Meds Update on craniosacral therapy by Sherril Sego, FNP-C, DNP (October 2014; available at ClinicalAdvisor.com/craniosacral-therapy-may-be-helpful-but-notcurative/article/374869, accessed on November 15, 2014): We thank Sherril Sego for helping to spread the word about Upledger CranioSacral Therapy (CST) and want to share some information to clarify a few points in her article. There are varying schools of thought that developed out of William G. Sutherland’s initial work. Much of the early research on Sutherland’s findings was performed by osteopathic physician Dr. John E. Upledger. Dr. Upledger was hired by Michigan State University to prove or disprove the work of Dr. Sutherland. Based on this research, Dr. Upledger developed a treatment modality, which he named “CranioSacral therapy” in order to differentiate his researchbased techniques from cranial osteopathy. Upledger CST focuses on the whole body and the interactions within it. CST incorporates evaluation and gentle mobilization of the cranium, dura mater, dural tube, and sacrum, as well as the continuity of this anatomical system with the fascia of the entire body. An important feature of CST is the attention given to the body’s fascial system since the dura mater is the core of this membranous system. Restrictive patterns within the fascia translate their forces throughout the body. The production, reabsorption, and subsequent draining of the cerebrospinal fluid (CSF) into the venous portion of the cardiovascular system produce a subtle movement of the cranial bones called the CranioSacral Rhythm (CSR). The CSR is also reflected throughout the body via the delicate stimulation of the motor cortex during the production phase. CST uses the palpation of the CSR via the bones of the cranium and throughout the body to detect and release restrictions in the membranous system that could potentially

Claire O’Connell, MPH, PA-C,

Katherine Pereira, DNP, FNP,

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

is assistant professor at Duke University School of Nursing, Durham, N.C.

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.

www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • DECEMBER 2014 53

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Advisor Forum

Dr. Sego responds: As health-care providers, we can all agree that exploring the actual clinical significance of CST for the average primary-care provider is the ultimate goal of this discussion. In describing the actual technique of CST, it appears that the subtleties of the practice are a set of finely tuned skills that would require intensive training and experience. Having read the literature, I do understand the subtle difference between mobilization, or freeing up, and manipulation, and agree that this is a fine point of clarification but not likely to be one of focus for the general practitioner. The tenet that the skilled practitioner must not only be able to physically detect this phenomenon of the body, but then discern the physiologic significance for the rest of the body, is clear. As with many alternative medical practices however, there is nearly as much literature refuting this as there is supporting it, and inter-rater reliability of this skill has not always been upheld. Ultimately, the decision is usually made by the patient. Unfortunately, this is often not dictated by the potential for benefit of the practice, but by the financial aspect of whether or not health insurance covers the therapy. In my own experience, this is a real and frustrating point. Some insurance companies specify that CST is considered “physical therapy” and cover the practice only if it is performed by a licensed physical therapist. In summary, the age-old premise put forth by Hippocrates of “do no harm” would most likely be upheld by CST. It is a practice that, at worst, would fail to meet the goals of the patient and the practitioner. But in the real world of restricted resources and evidence-based practice, CST may not be the best investment for return. (194-4) n

“Maybe we need different types of clubs for different types of situations.”

“You don’t need to conduct me so hard—I’m right here.”

“I have over a hundred words for ‘grits.’ ”

cause sensory, motor, or neurologic dysfunctions. As such, CST is intended to facilitate the body’s ability to self-correct. The fibromyalgia study cited [in the article] demonstrated the effectiveness of managing the symptoms of fibromyalgia over time. When contrasted with [drug] treatment and associated side effects, CST offered a gentle and viable alternative. CST is recommended for all ages—newborns to geriatrics— when performed by a highly skilled practitioner trained in the various specialties. There are few contraindications to CST, but they should be noted: CST should not be done in individuals with acute stroke, acute cerebral hemorrhage, aneurysm, or any acute cerebral vascular condition with an active bleed. CST should not be done in anyone with an epidural leak, recent skull fracture, or acute traumatic brain injury, or in certain cases of brain herniations, tumors, or conditions in which changes in intracranial fluid pressure could conceivably cause a problem.—JOHN MATTHEW UPLEDGER, CEO, Upledger Institute International, Palm Beach Gardens, Fla. (194-3)

54 THE CLINICAL ADVISOR • DECEMBER 2014 • www.ClinicalAdvisor.com

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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 ClinicalAdvisor.com/DermDx.

Itchy rash on eyelids, chest, back, and hands A patient, aged 40 years, presented with complaints of a mildly itchy rash on his eyelids, chest, back, and hands that had been present for the past 3 months. He reported feeling weak over the past month and that the weakness was getting progressively worse. CAN YOU DIAGNOSE THIS CONDITION?

• Dermatomyositis • Lupus erythematosus • Scleroderma • Morphea ● See the full case at ClinicalAdvisor.com/DermDx_Dec2014A

Desquamating full-body rash in HIV-positive patient A 50-year-old man with HIV/AIDS and cytomegalovirus colitis presented with a full-body desquamating skin rash that had developed several weeks earlier. He had been an inpatient for more than a month with treatment-resistant diarrhea and failure to thrive. CAN YOU DIAGNOSE THIS CONDITION?

• Stevens-Johnson syndrome • Kwashiorkor • Pemphigus vulgaris • Bullous pemphigoid ● See the full case at ClinicalAdvisor.com/DermDx_Dec2014B

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

Photosensitive blisters on the hands

Warty papules on the chest, ears

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LEGAL ADVISOR CASE

Misdiagnosis leads to fatal emergency

BY ANN W. LATNER, JD

Ms. C was a 37-year-old nurse practitioner working in an urgent-care clinic. The clinic was staffed by 2 physicians and 3 nurse practitioners, as well as office staff and several assistants. At any given time, there were at least 2 clinicians in the office—a physician and a nurse practitioner—and each saw a share of the patients. The clinic was spacious, clean, and modern and was equipped to provide laboratory testing, electrocardiograms, and x-rays. In the summer, the clinic tended to get patients with ailments such as sports injuries, broken bones, and allergies. In colder weather, patients often came in with cold, cough, and flu complaints. On occasion, the clinic would get a patient who had a true emergency and needed immediate attention at the hospital. In those cases, the physician or nurse practitioner would refer the patient immediately to the local hospital’s emergency department. On a particularly busy evening in the clinic, Ms. C was working with only 1 other clinician, Dr. P, to see to a large group of patients. One of Ms. C’s patients that evening was Mr. D, a 23-year-old graduate student, who

A young man dies when an emergent condition is mistaken for a nonemergent one.

Mr. D, a 23-year-old graduate student, complained of fever, chest pains, and cough.

60 THE CLINICAL ADVISOR • DECEMBER 2014 • www.ClinicalAdvisor.com

complained of fever, chest pains, and cough. His temperature was 101°F. He said that he had been unwell for the last couple of days. Ms. C performed a brief examination and gave Mr. D a diagnosis of bronchitis, providing him with a prescription for an antibiotic. Ms. C told Mr. D to get some rest, start taking the antibiotic, and come back in to the clinic if he did not feel better in a couple of days. The next morning, when he did not show up at school, some friends went to check on Mr. D and found him dead in his bed. In the weeks that followed, medical examiners identified Mr. D’s cause of death as myocarditis. Ms. C was unaware of what had happened to her patient until several months later when she was notified that the clinic had been sued because of the untimely death of Mr. D and that the plaintiffs (Mr. D’s family) were alleging his 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.

LEGAL ADVISOR

Mr. D’s cause of death was myocarditis, a condition that would have been identified if an electrocardiogram had been obtained. Ms. C was called to testify. On direct examination by her own attorney, she described her brief interaction with Mr. D. Ms. C stated that she did not suspect myocarditis because of the patient’s age and his other symptoms, particularly the cough. However, on cross-examination, Ms. C was forced to admit that Mr. D had complained of chest pains and that typically, when a patient makes such a complaint, an electrocardiogram is ordered. The plaintiffs’ expert testified that once the patient had complained of chest pains, Ms. C was negligent when she did not order the electrocardiogram, and this fell below the standard of care required. The jury returned a verdict for the plaintiffs and ordered a $4.8 million award to Mr. D’s family.

the circumstances that led to the alleged malpractice. This is almost always established by a qualified expert medical witness who testifies about the appropriate medical standard of care under the circumstances and how the clinician’s deviation from that standard played a role in the patient’s injuries. In this case, the expert testified that when a patient presents with chest pains as a complaint, the standard of care requires the administration of an electrocardiogram or immediate referral to a hospital emergency department that could treat a possible emergency cardiac condition. Protecting yourself

On the surface, it is somewhat understandable that Ms. C would mistake myocarditis for bronchitis. The youth of the patient, combined with the fact that coughing can cause chest pain, could point to a more likely diagnosis of bronchitis. However, assumptions like those can cause tragic mistakes. Chest pain should always be treated seriously—whether the patient is a man, a woman, old, or young. An electrocardiogram is a simple, cost-effective diagnostic tool and should always be ordered for any patient who complains of chest pain. If your medical facility does not have the capability to administer electrocardiograms, patients with chest pain should immediately be referred to the nearest hospital emergency department. n Ms. Latner, a former criminal defense attorney, is a freelance medical writer in Port Washington, N.Y.

Legal background

One of the elements of proving medical malpractice or professional negligence is demonstrating that the practitioner’s actions deviated from the accepted standard of care. Most medical malpractice cases hinge on this. The medical standard of care is typically defined as the level and type of care that a reasonably competent and skilled health-care professional, with a similar background and in the same medical community, would have provided under

death was the result of Ms. C’s negligent treatment. The defense attorney who was representing the clinic explained to Ms. C that this was a medical malpractice case, which is really the tort of professional negligence. In such a case, the plaintiff would have to prove all of the following 5 elements to be successful: 1) a duty was owed to the patient; 2) that duty was breached; 3) the breach caused an injury; 4) the practitioner deviated from the accepted standard of care; and 5) that damage occurred because of it. Settlement negotiations failed, and the case went to trial. At trial, the plaintiffs introduced expert testimony establishing that Mr. D’s cause of death was myocarditis, a condition that would have been identified if an electrocardiogram had been obtained. The plaintiffs also established that the clinic had an electrocardiogram machine and could have easily administered the test.

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Ortho Dx

In partnership with TheJopa.org

Journal of Orthopedics for Physician Assistants

READ CLINICAL DESCRIPTIONS, view images, and then make the diagnosis. See how you compare with your peers in our online polling feature. Visit ClinicalAdvisor.com/OrthoDx to test your diagnostic skills in orthopedics.

Sharp knee pain without injury A patient, aged 56 years, presented with severe acute pain in the right knee without injury. The patient reported getting out of his car 2 days earlier and feeling a sudden, sharp pain. Since then, he had been unable to extend or put weight on his knee. WHAT IS THE MOST LIKELY CAUSE OF THIS PATIENT’S KNEE PAIN?

• Knee osteoarthritis • Meniscus tear • Medial collateral ligament (MCL) tear • Loose body ● See the full case at ClinicalAdvisor.com/OrthoDx_Dec2014A

Weakness, loss of sensation A 48-year-old male presented with complaints of radiculopathy, muscle weakness, and a loss of sensation in the left lower extremity. To help determine the cause of the problem, magnetic resonance imaging (MRI) was ordered. BASED ON THE MRI IMAGES, WHAT ARE THE MOST LIKELY PHYSICAL EXAM FINDINGS?

• Tibialis anterior weakness and loss of sensation at the medial ankle • Gastrocnemius weakness and loss of sensation at the plantar aspect of the foot • Extensor hallucis longus weakness and loss of sensation at the dorsum of the foot • Hip flexion weakness and loss of sensation at the anterior thigh ● See the full case at ClinicalAdvisor.com/OrthoDx_Dec2014b

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CME CE

n LEARNING OBJECTIVES After completing the activity, the participant should be better able to:

For Dermatology Clinic

• Examine various skin pathologies • Apply increased knowledge of dermatologic conditions • Formulate diagnostic procedures and protocols for specific dermatologic conditions

For Dermatologic Look-Alikes

• Differentiate between varying dermatologic conditions and their diagnostic manifestations • Demonstrate proficiency in identifying and treating two distinct skin ailments

DERMATOLOGY COURSES

n COMPLETE THE POSTTEST: Page 77 n ADDITIONAL CME/CE CREDIT: Page 36, 71

This activity is provided by Haymarket Medical Education (HME) for physician credit. This activity is co-provided by Medical Education Resources (MER) for nursing contact hours. Release Date: December 10, 2014 Expiration Date: December 9, 2015 Estimated time to complete the educational activity: 30 minutes Statement of Need: Undertraining in dermatology for primary-care providers is a common phenomenon. Thus, primary-care clinicians need additional educational outlets devoted to the diagnosis and treatment of specific dermatologic conditions. For clinicians out of training, CME becomes the most accessible route. Target Audience: This activity has been designed to meet the educational needs of primary-care health-care professionals who treat patients with various dermatologic conditions. Faculty Padma Chitnavis, BS, medical student, Virginia Commonwealth University School of Medicine, Richmond Julia R. Nunley, MD, professor and program director of dermatology, Virginia Commonwealth University School of Medicine, Richmond Audrey Chan, MD, pediatric dermatology fellow, Texas Children’s Hospital, Houston Andrew S. Fischer, BS, medical student, Baylor College of Medicine, Houston Christopher B. Rizk, BS, medical student, Baylor College of Medicine, Houston Adam Rees, MD, dermatologist, Los Angeles Accreditation Statements Physician Credit: HME is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians. Credit Designation: HME designates this enduring material for a maximum of 0.5 AMA PRA Category 1 CreditTM. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Nursing Credit: MER is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. Credit Designation: This CE activity provides 0.5 contact hour of continuing nursing education. MER is a provider of continuing nursing education by the California Board of Registered Nursing, Provider #CEP 12299, for 0.5 contact hour. This activity qualifies for 0.13 pharmacotherapy credit. American Academy of Physician Assistants (AAPA) The AAPA accepts certificates of participation for educational activities certified for AMA PRA Category 1 Credit™ from organizations accredited by ACCME or a recognized state medical society. Physician assistants may receive a maximum of 0.5 hour of Category I credit for completing this program. Disclosure Policy In accordance with the ACCME Standards for Commercial Support, HME requires that individuals in a position to control the content of an educational activity disclose all relevant financial relationships with any commercial interest. HME resolves all conflicts of interest to ensure independence, objectivity, balance, and scientific rigor in all its educational programs.

standards of experimental design, data collection, and analysis. HME is committed to providing its learners with high-quality CME/CE activities that promote improvements in health care and not those of a commercial interest. The faculty reported the following financial relationships with commercial interests whose products or services may be related to the content of this CME activity: Faculty Disclosures

Name of faculty

Reported Financial Relationship

Padma Chitnavis, BS

No relevant financial relationships

Julia R. Nunley, MD

No relevant financial relationships

Audrey Chan, MD

No relevant financial relationships

Andrew S. Fischer, BS

No relevant financial relationships

Christopher B. Rizk, BS

No relevant financial relationships

Adam Rees, MD

No relevant financial relationships

Staff/Planners’ Disclosures The planners and managers for this program reported the following financial relationships with commercial interests whose products or services may be related to the content of this CME activity: HME planners and managers have no relevant financial relationships to disclose. MER planners and managers have no relevant financial relationships to disclose. Disclosure of Unlabeled Use: This educational activity may contain discussion of published and/or investigational uses of agents that are not indicated by the FDA. HME and MER do not recommend the use of any agent outside of the labeled indications. Please refer to the official prescribing information for each product for discussion of approved indications, contraindications, and warnings. Method of Participation: There are no fees for participating in and receiving CME/CE credit for this activity. During the period of December 10, 2014, through December 9, 2015, participants must: 1) read the learning objectives and faculty disclosures; 2) study the educational activity; 3) complete the posttest and submit it online (clinicians may register at www.myCME.com); and 4) complete the evaluation form online. A statement of credit will be issued only upon receipt of a completed activity evaluation form and a completed posttest with a score of 70% or better. Disclaimer: The content and views presented in this educational activity are those of the authors and do not necessarily reflect those of HME or MER. Any procedures, medications, or other courses of diagnosis or treatment discussed or suggested in this activity should not be used by clinicians without evaluation of their patient’s conditions and possible contraindications on dangers in use, review of any applicable manufacturer’s product information, and comparison with recommendations of other authorities. The information presented in this activity is not meant to serve as a guideline for patient management.

Furthermore, HME seeks to verify that all scientific research referred to, reported, or used in a CME/CE activity conforms to the generally accepted

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Dermatology Clinic

CME CE DERMATOLOGY COURSES

CASE #1

Purple plaque on foot after swim in river PADMA CHITNAVIS, BS; AND JULIA R. NUNLEY, MD

A previously healthy 45-year-old white woman was transferred from an external health-care facility with vomiting, abdominal pain, and painful lesions on her foot that had failed to improve despite 24 hours of broad-spectrum antibiotics; doxycycline had been started just before the transfer. On arrival, the patient was experiencing occasional chills and was found to be febrile, mildly tachycardic, and tachypneic. Examination revealed massive edema with multiple large, hemorrhagic, and violaceous bullae on the dorsal aspect of her right foot. She reported that these lesions began approximately 1 day after cutting her foot on a rock while swimming in a river in coastal Virginia. What is your diagnosis? Turn to page 68.

CASE #2

Papules on girl with Down syndrome AUDREY CHAN, MD

A 14-year-old girl came into the clinic with an eruption of skincolored to red-brown papules on her neck and chest that had been present for months. The lesions were usually asymptomatic, but the girl did complain of occasional pruritus. She denied tenderness to palpation. A review of systems was negative for fever or chills. The patient’s medical history was significant for Down syndrome but negative for diabetes. She was not taking any medications. Her social history was noncontributory. The physical examination was notable for many 6- to 7-mm firm papules on the anterior neck and chest that varied from skin-colored to red-brown in color. What is your diagnosis? Turn to page 69. www.ClinicalAdvisor.com • THE CLINICAL ADVISOR • DECEMBER 2014 67

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CME CE

CASE #1

Dermatology Clinic

Vibrio vulnificus infection

Monitoring the patient in the intensive care unit was initially considered, but her hemodynamic status normalized rapidly with intravenous volume expansion. A marked neutrophilic leukocytosis with left shift, mildly elevated creatinine, mild thrombocytopenia, and metabolic acidosis were among the early laboratory abnormalities noted. Gram stain of the bullous fluid from the patient’s foot was not diagnostic. However, the presence of fluffy bilateral infiltrates on chest radiograph supported the suspected diagnosis of vibriosis, which results from infection by a member of the Vibrio species. Doxycycline was continued and intravenous (IV) ceftazidime was initiated. Over the next 24 hours, the patient’s erythema and edema stabilized, but the hemorrhagic bullae progressed to necrosis, requiring wound debridement. Approximately 48 hours after admission, cultures from the patient’s wound were determined to be positive for Vibrio vulnificus. Endemic to brackish waters, such as coastal and estuarine environments, Vibrio vulnificus is a free-living, opportunistic, halophilic pathogen that thrives in warm waters, preferably those at a temperature that is greater than 18° C (64° F), with a salinity between 15 and 25 parts per 1,000.1 For comparison, the average salinity of the open ocean is 35 parts per 1,000, whereas the salinity of the Dead Sea, the Great Lakes, and common city water are approximately 330 parts, 10 parts, and less than 1 part per 1,000, respectively. Of the 3 biotypes of Vibrio vulnificus (numbered 1 to 3) that are known to exist, biotype 1 is responsible for the majority of infections.1 Infections tend to be seasonal, peaking during the summer and fall months, with 85% of cases occurring between May and October.2 The majority of cases reported in the United States are from states along the Gulf and Atlantic coasts (33% and 34%, respectively, in 2011); however, cases have also been reported to occur on the Pacific coast.3 Of the 853 cases of vibriosis reported in the United States in 2011, those due specifically to Vibrio vulnificus infection had the highest rates of hospitalization and mortality.3 The illnesses ascribed to Vibrio vulnificus infection range from mild gastroenteritis to wound infections and lifethreatening septicemia,4 and conditions vary according

to the site of the original infection and state of the host.5 Ingestion of raw or undercooked oysters is responsible for the majority of cases of gastroenteritis or primary sepsis.1 Cutaneous infections occur primarily when open wounds are exposed to saltwater, often following recreational activities, including swimming or boating.5 Individuals whose occupations repeatedly expose them to raw shellfish and oysters (e.g., fishermen, cooks) are also at risk for soft-tissue infections and should always wear gloves and utilize other safety precautions. Whereas most Vibrio vulnificus infections related to recreational activities typically occur on the lower extremities, those related to occupational exposure are more likely to occur on the upper extremities.5 Skin lesions, however, are not unique to primary cutaneous inoculation; patients with septicemia can also develop hemorrhagic bullae.1 In the worst-case scenario of cutaneous infection with Vibrio vulnificus, lesions can progress to necrotizing fasciitis. The virulence of Vibrio vulnificus is attributed to its ability to produce and release numerous highly destructive cytotoxins.4 These cytotoxins have been found to facilitate destruction of the fascia, increase vascular permeability, and contribute directly to hemolysis and pulmonary injury.4,6 Associated toxins include cytolysin, hemolysin, protease, lipase, hyaluronidase, mucinase, deoxyribonuclease, and sulfatase.4,6 Healthy individuals are less likely to develop cutaneous infection following casual exposure and, if infected, are more likely to have a less virulent course. In comparison, individuals with chronic underlying diseases are at a much greater risk for infection and typically have a more aggressive course, often with a less than optimal outcome.1 In particular, chronic corticosteroid use or the presence of diabetes mellitus, immunodeficiency, hematologic and iron-storage disorders, malignancies, gouty arthritis, chronic renal failure, or chronic liver disease, especially with cirrhosis, confers a higher risk for infection and worse prognosis.5 Optimal treatment requires early intervention upon early suspicion with appropriate antimicrobial therapy and wound care.1 The preferred antibiotic regimen is a combination of 100 mg of IV doxycycline every 12 hours and 2 g of ceftazidime every 8 hours, with alternative regimens including either cefotaxime or ciprofloxacin.1 Emergent debridement, including fasciotomy in extreme cases and amputation in life-threatening cases, has been shown to significantly reduce mortality and length of hospital stay and should be included in the treatment protocol.4 Conditions to be considered in patients with bullous hemorrhagic lesions can be subdivided into 3 major groups: infections, including a variety of bacteria and viruses,

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especially herpes infections; autoimmune disorders, such as bullous pemphigoid, pemphigus vulgaris, and pyoderma gangrenosum; and other etiologies including purpura fulminans, contact dermatitis, and drug reaction.7 Other bacterial infections causing skin lesions in an acutely ill patient include Neisseria meningitidis, Pseudomonas aeruginosa, and species of Aeromonas, Clostridium, Staphylococcus and Streptococcus.7 However, in the setting of an exposure to brackish waters or ingestion of raw clams or oysters, especially in a patient with chronic liver disease or immunosuppression, one must always consider Vibrio vulnificus. A diagnosis of Vibrio vulnificus infection can be confirmed by isolation of the organism or by identification of the characteristic fluffy, bilateral pulmonary infiltrates on chest radiograph. In this patient, early suspicion by the clinical team days before confirmation allowed for a positive outcome. She continued to improve during her hospital stay and was discharged with an additional 2-week course of oral doxycycline. One year later, the patient is fully functional, although the affected foot shows some minor discoloration and still swells after long periods of standing. Mr. Chitnavis is a fourth-year medical student in the Virginia Commonwealth University School of Medicine in Richmond, where Dr. Nunley is professor and program director in the Department of Dermatology. References 1. Horseman MA, Surani S. A comprehensive review of Vibrio vulnificus: an important cause of severe sepsis and skin and soft-tissue infection. Int J Infect Dis. 2011;15(3):e157-e166. Available at ijidonline.com/article/ S1201-9712(10)02538-5/fulltext 2. Jones EH, Feldman KA, Palmer A, et al. Vibrio infections and surveillance in Maryland, 2002-2008. Public Health Rep. 2013;128(6):537-545. 3. Centers for Disease Control and Prevention. National Enteric Disease Surveillance: COVIS [Cholera and Other Vibrio Illness Surveillance] Annual Summary, 2011. Available at cdc.gov/ncezid/dfwed/PDFs/covis-annualreport-2011-508c.pdf 4. Tsai YH, Huang TJ, Hsu RW, et al. Necrotizing soft-tissue infections and primary sepsis caused by Vibrio vulnificus and Vibrio cholerae non-O1. J Trauma. 2009;66(3):899-905. 5. Bross MH, Soch K, Morales R, Mitchell RB. Vibrio vulnificus infection: diagnosis and treatment. Am Fam Physician. 2007;76(4):539-544. Available at www.aafp.org/afp/2007/0815/p539.html 6. Park JW, Ma SN, Song ES, et al. Pulmonary damage by Vibrio vulnificus cytolysin. Infect Immun. 1996;64(7):2873-2876. Available at ncbi.nlm.nih.gov/ pmc/articles/PMC174159

7. Norton SA. Section 29: Bacterial disease. Chapter 183: Miscellaneous bacterial infections with cutaneous manifestations. In: Goldsmith LA, Katz SI, Gilchrest BA, et al., eds. Fitzpatrick’s Dermatology in General Medicine. 8th ed. New York, N.Y.: McGraw-Hill; 2012. All electronic documents accessed on November 15, 2014.

CASE #2

Eruptive syringomas

Syringomas, of which eruptive syringomas are one of the rare clinical variants, are benign adnexal neoplasms. Although syringomas were historically thought to have an eccrine lineage, it is now thought that these neoplasms can be derived from either apocrine or eccrine glands1 Syringomas are believed to be more common in women; however, this may simply be because women are more likely than men to seek care for these lesions.1 Japanese women may be affected disproportionately.2 Syringomas occur in 18% of adults with Down syndrome, which is approximately 30 times the frequency seen in the normal population.2 Familial cases have been reported, but an inheritance pattern or underlying gene mutation has not been established.2 The pathogenesis of syringomas is unknown. In the case of eruptive syringomas, some hypothesize that the condition represents a proliferative process of inflamed normal eccrine glands, based on the observation that numerous lesions have appeared after the waxing of pubic areas.2 Clinically, syringomas present as small papules that are 1 to 3 mm in diameter.2 The color varies; skin-colored, yellow, brown, and pink papules have been described.2 Solitary lesions are rarely seen, as most syringomas occur in multiples. They most commonly appear on the periorbital areas, especially the eyelids, and upper cheeks.1,2 However, syringomas may also involve the upper trunk, more often on the ventral surface, or genital skin.1 Clinical variants of syringomas are rare and include eruptive syringomas in which multiple lesions appear suddenly.2 Historically, eruptive syringomas have also been referred to as eruptive hidradenomas of Jacquet and Darier.3 This entity is rare, with fewer than 100 cases reported in the

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CME CE

Dermatology Clinic

literature.4 The onset is usually during young adulthood, in the second and third decades of life.3 In a retrospective review of 27 patients with eruptive syringomas, Soler-Carillo et al. noted that females were more commonly affected than males, and white patients were predominantly affected. However, ethnic variation is difficult to interpret, because this study was reported from a hospital in Barcelona, Spain, which involves a population that is presumably predominantly white.4 In this cohort, the mean age of diagnosis was 29 years, with only 33% of patients presenting before age 15 years.4 In Soler-Carillo’s review, it was noted that in prepubertal patients, eruptive syringomas favored the neck and anterior trunk, followed by areas of apocrine localization, such as the axillae and pubis.4 Eruptive syringomas are usually round to oval in shape, slightly elevated, firm, faintly rose-colored, and several millimeters in size.3 Individual lesions of this clinical variant have been known to coalesce into larger plaques. Eruptive syringomas may align with their long axes parallel to the lines of cleavage.3 This type of syringoma is seen most frequently on the neck, chest, axillae, upper arms, and periumbilical area.2 The lesions may also be found on the extremities, including the palms and soles.1 Crops of lesions occur for 2 to 3 years and tend to persist indefinitely.3 In rare cases, eruptive syringomas regress in adulthood, but this seems to be the exception rather than the rule.4 Eruptive syringomas can be associated with Nicolau-Balus syndrome, which is characterized by syringomas, milia, and atrophoderma vermiculata.4 There are reports of eruptive syringomas developing in patients with Down syndrome.4 Associations with neoplasms and diabetes mellitus have been reported, but because of the rarity of eruptive syringomas, it is difficult to determine any pathogenic relationship.4 A few familial cases of eruptive syringomas have also been reported.4 Other clinical variants of syringomas, which are all rare, include the following: those limited to the scalp, associated with alopecia; those with a unilateral linear or nevoid distribution; those limited to the vulva or penis; those limited to the distal extremities; and those resembling lichen planus or milia.2 Calcifications may occur in syringomas and may be mistaken for subepidermal calcified nodules.2 Plaque-type syringomas, which could resemble microcystic adnexal carcinoma,2 and acral syringomas are also rare clinical variants. Acral syringomas are considered to be of an eccrine lineage, because these lesions occur on the palms and soles, which exclusively contain eccrine glands.1 Patients with Down syndrome often present with eyelid syringoma.4 Eyelid syringomas are also frequently seen in Marfan and Ehlers-Danlos syndrome.4 The diagnosis of syringomas is often made clinically. The

diagnosis of eruptive syringomas is a clinical one that is made when numerous syringomas present with sudden onset. When in question, a diagnosis of syringomas can be confirmed with a skin biopsy. On scanning magnification, syringomas are small and circumscribed, and they are usually confined to the superficial dermis as is expected in most benign lesions.1 Histologic examination reveals dilated cystic spaces.2 As expected, since syringomas are most commonly thought to be of eccrine lineage, these cystic spaces are lined by two layers of cuboidal cells with pale or pinkish cytoplasm.1,2 Epithelial strands of similar cells are often seen in the dermis as well.2 Depending on the way the sample is sectioned during processing, some of these cystic spaces appear to have comma-like tails, leading to descriptions such as “tadpoles in the dermis” or “paisley-tie pattern.”1,2 There is often a dense, fibrous stroma.2 A histologic variant of syringomas with cells that have an abundant clear cytoplasm due to the accumulation of glycogen has been described.2 Clear-cell syringomas are only a histologic variant, as they are clinically indistinguishable from syringomas. Clear-cell syringomas are associated with diabetes mellitus.1 Identical to the staining of the intraglandular eccrine duct, syringomas stain for keratins 5, 6, 14, 6, 16, 19, and 77 on the inner cell layer, and K5 and K4 on the outer cell layer.2 Treatment is difficult with syringomas, and not very effective. Anecdotally, syringomas respond well to light electrodesiccation or shave removal.2 To improve the cosmetic appearance of larger lesions, syringomas may be surgically excised.2 There are also case reports of syringomas being treated effectively with carbon-dioxide laser by the pinhole method or fractional thermolysis.2 Other reported therapies include trichloroacetic acid or cryotherapy.1 A biopsy confirmed the diagnosis of the patient in this case. Our patient was not distressed by the cosmetic appearance of her lesions, so treatment was not pursued. n Dr. Chan is a pediatric dermatology fellow at Texas Children’s Hospital in Houston. References 1. Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. 3rd ed. St. Louis, Mo.: Mosby Elsevier; 2011: Chap.111. 2. James WD, Berger T, Elston D. Andrews’ Diseases of the Skin. 11th ed. Philadelphia, Pa.: Elsevier; 2011:579-580. 3. Kuttner BJ, Kaplan DL, Rothstein MS. Eruptive pruritic papules. Eruptive syringomas (eruptive hidradenomas of Jacquet and Darier). Arch Dermatol. 1989;125(7):985, 988. 4. Soler-Carrillo J, Estrach T, Mascaró JM. Eruptive syringoma: 27 new cases and review of the literature. J Eur Acad Dermatol Venereol. 2001;15(3):242-246.

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Dermatologic Look-Alikes

CME CE DERMATOLOGY COURSES

Hypopigmented lesions ANDREW S. FISCHER, BS; CHRISTOPHER B. RIZK, BS; AND ADAM REES, MD

CASE #1

CASE #2

A 6-year-old boy presented with his parents for evaluation of hypopigmented lesions on his abdomen and extremities. The hypopigmented patches were asymptomatic, and the pigmentary pattern had been noted at birth. The child’s father and paternal grandmother had similar skin findings. The boy also had a white forelock. His medical history was unremarkable. He had met all of his developmental milestones and was up to date with all routine vaccinations. His parents were concerned that he might be teased in school about his skin and hair.

A woman, aged 48 years, presented with a complaint of 10 years of depigmentation on her face and body. The areas of depigmentation were completely asymptomatic, but she was embarrassed by her appearance. Her medical history was significant for Hashimoto thyroiditis, for which she was on medication. Her family history was unremarkable; her parents, siblings, and children did not have a similar pigmentary disorder. The patient was a nonsmoker and did not use any drugs or alcohol. She had undergone no prior treatment for this problem.

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CASE #1

Dermatologic Look-Alikes

Piebaldism

Piebaldism is a rare autosomaldominant disorder characterized by the congenital absence of melanocytes in the skin and hair due to a mutation of the c-kit gene, which affects the differentiation and migration of melanoblasts from the neural crest. Affected individuals typically present at birth with a white forelock and stable, persistent depigmentation of the skin that primarily affects the frontal scalp, forehead, ventral trunk, and extremities. It is a permanent, albeit benign, skin condition, and treatment can be challenging. Piebaldism is estimated to affect approximately 1 in 20,000 to 40,000 men and women of all races, although the true prevalence is unknown.1,2 Individuals typically present at birth with a stable and persistent depigmentation of the hair and skin. The hallmark of piebaldism is a triangular white forelock, which is present in 80% to 90% of cases.1,3 In

The hallmark of piebaldism is a triangular white forelock, which is present in 80% to 90% of cases. some cases, it may be the only manifestation, but its absence does not exclude the diagnosis. Underlying the forelock may be an amelanotic triangular or diamond-shaped patch that may extend to include the root of the nose and medial third of the eyebrows and eyelashes in severe cases.1,4 The leukoderma of piebaldism is distinctive. Depigmented macules may be rectangular, rhomboid, or irregular in shape and are well-circumscribed and milk-white in color.4,5 The macules are characteristically symmetrically distributed over the central anterior trunk, extending to the flanks, mid-extremities, central forehead, and mid-frontal portion of scalp (with the forelock).1 Lesions typically spare the posterior midline, hands, feet, and periorificial areas. Normally

pigmented or hyperpigmented macules or patches may be found within or at the border of the depigmented macules. Piebaldism is inherited in an autosomal-dominant fashion. Most cases are linked to mutations in the KIT proto-oncogene, c-kit, on chromosome 4q12, which encodes a transmembrane cell-surface receptor of the tyrosine kinase family on the surface of melanocytes.6-11 This cell-surface receptor binds steel factor, an embryonic growth factor. To date, 14 pointmutations, 9 deletions, 2 nucleotide splice mutations, and 3 insertions of the c-kit gene, which present with a range of phenotypes, have been reported in the literature.6 Histopathologic evaluation of the hypopigmented macules reveals considerably reduced or absent melanocytes, whereas the hyperpigmented macules have a normal number of melanocytes but an increased number of melanosomes. The significant or complete loss of melanocytes in the amelanotic lesions is caused by improper migration of melanocytes from the neural crest in the embryo.1,4 The diagnosis of piebaldism is well-established based on the presence of stable, depigmented macules since birth in the typical distribution, a white forelock in the frontal region, and the presence of depigmented macules in other family members. However, the differential diagnosis includes other conditions that present with depigmentation of the hair and skin (e.g., vitiligo) and other congenital conditions of hypopigmentation (e.g., albinism, nevus depigmentosus, and the hypomelanosis of tuberous sclerosis).12-18 Piebaldism is distinguished from vitiligo by the presence of lesions since birth, hyperpigmented macules within and at the border of the depigmented areas, and the static course of the condition. Vitiligo may be present at birth, but usually is acquired later in life. It follows an unstable course, and typically involves the acral and periorifical areas. Piebaldism spares the acral and periorificial areas. Some syndromes may present with piebald-like hypopigmentation of the skin and hair, but also with additional anomalies and no association with c-kit mutations. Waardenburg syndrome is an autosomal-dominant disorder characterized by a congenital white forelock, lateral displacement of the medial canthi, a hypertrophic nasal root, partial or total heterochromia of the iris, and sensorineural deafness.19 Ziprkowski-Margolis syndrome, now called the albinism-deafness syndrome (ADFN), is a rare X-linked recessive disorder that presents with hypomelanosis, hyperpigmented macules, deafness, mutism, and heterochromic iridis.20 Woolf syndrome is an autosomal-recessive disorder that presents with piebaldism and congenital deafness.21 Tietz syndrome is an autosomal-dominant condition with

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congenital generalized depigmentation and sensorineural deafness associated with a mutation in the microphthalmiaassociated transcription factor (MITF) gene.22 Treatment of piebaldism is challenging. Sunscreen is recommended to avoid burns and reduce carcinogenesis. Tanning agents such as dihydroxyacetone (DHA) may be used as a temporary pigmenting agent along with makeup to camouflage affected areas.23 The depigmented skin is considered to be generally unresponsive to medical treatment or phototherapy. Numerous surgical techniques have had varying results, including split-thickness grafting, minigrafting, transplant of in vitro cultured epidermis and suction-blister epidermal grafting, and a combination of dermabrasion and grafting of pigmented skin into the depigmented areas.24,25 Culture and transfer of autologous melanocytes or of melanocytes and keratinocytes has been described as safe and effective.26,27 This typically requires multiple procedures, and phototherapy may be effective if used afterward.1 In this case, the patient and family were educated about the disease. Because the skin findings were cosmetically acceptable to the family, no intervention was required.

CASE #2

Vitiligo

Vitiligo is a common skin disease characterized by acquired, circumscribed depigmentation of the skin and hair. Loss of epidermal melanocytes is believed to result from autoimmune destruction. The etiology of vitiligo is multifactorial and likely polygenic. The course of the disease is unpredictable, but often progressive. Vitiligo is the most common disorder that results in depigmentation, affecting 0.5% to 2% of the world’s population.28 There is no predilection for sex, age, race, or skin type.29,30 Onset may be at any time from shortly after birth to adulthood, although it is rarely seen in infancy or old age. Peak onset is between ages 10 and 30 years, with approximately half of patients presenting by age 20 years.29,31 Many patients attribute the disease onset to a physical or emotional injury, sunburn, illness, or pregnancy. However, there is little evidence to support this, with the exception of the Koebner phenomenon.28

Histopathologic evaluation of the completely depigmented epidermis of affected skin in patients with vitiligo shows an absence of functional melanocytes and melanin, which is generally agreed to be a result of their destruction.28 Hypopigmented areas on the peripheries of expanding lesions demonstrate few melanocytes with some melanosomes. Furthermore, the outer hyperpigmented borders contain prominent melanocytes with many melanosomes. The observation of vacuolated keratinocytes and extracellular granular material in the skin that appears normal in

Vitiligo is the most common disorder that results in depigmentation, affecting 0.5% to 2% of the world’s population. patients with rapidly progressive disease suggests that vitiligo affects not only melanocytes but the entire epidermal melanin unit.32 The etiology of vitiligo is multifactorial, including both genetic and nongenetic factors. Although there is no Mendelian inheritance pattern, there is often a positive family history, and it has been reported in monozygotic twins.33-35 Some studies suggest a polygenic mechanism,36 which include genes that are also associated with autoimmune disease. This strengthens the hypothesis for the autoimmune destruction of melanocytes. Other theories to explain the destruction of melanocytes include the neurogenic hypothesis and the self-destruct hypothesis, whereby melanocytes are destroyed through interaction with nerve cells or by toxic metabolites, respectively.29 Patients typically present with 1 or more depigmented macules or patches with a normal or hyperpigmented convex border. The lesions are well-demarcated and may be round, oval, irregular, or linear in shape. Lesions vary in size and tend to enlarge centrifugally at an unpredictable rate over time. Distribution is often symmetric and lesions can appear on any body site, including mucous membranes. Lesions are most commonly found on the face, the upper part of the chest, axillae, dorsal aspects of the hand, and the inguinal and anogenital region. There is a predilection for the skin around orifices, including the eyes, nose, mouth, ears, nipples, umbilicus, penis, vulva, and anus. Lesions also appear at areas of physical trauma (the Koebner phenomenon), and so, they may be present on the elbows and knees. Involvement of the palms, soles, lips, and oral mucosa

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Dermatologic Look-Alikes

may be subtle in light-skinned individuals and may not be evident without a Wood lamp skin examination.28,29,31 Lesions are often asymptomatic but may be pruritic. White hairs may be present in the affected regions. Vitiligo of the scalp may present as one or several localized patches of white hair, scattered white hairs, or total depigmentation. In general, up to 30% of patients may experience some spontaneous repigmentation of the skin.29 Depigmentation of the retinal pigment epithelium and choroids may occur in patients with vitiligo. Uveitis is the most important ocular abnormality associated with vitiligo. Involvement of the melanocytes in the membranous labyrinth of the inner ear may manifest as hearing impairment. Destruction of the leptomeningeal melanocytes may result in aseptic meningitis. Vogt-Koyanogi-Harada (VKH) syndrome is characterized by vitiligo with uveitis, hearing loss, tinnitus, meningismus, and poliosis.37-39

Repigmentation is the cornerstone of treatment; it is based on the stimulation of melanocytic reserves in the hair follicles. Occasionally, patients with vitiligo may present with unusual variants. Vitiligo ponctuĂŠ or confetti-type vitiligo is characterized by several tiny, discrete hypomelanotic macules, which may be superimposed on hyperpigmented macules. Vitiligo macules that have an erythematous border are referred to as inflammatory vitiligo. Vitiligo that develops in an area already affected by post-inflammatory hyperpigmentation is referred to as blue vitiligo. Also described are trichrome, quadrichrome, and pentachrome vitiligo, which refer to additional colorations. Trichrome vitiligo has an additional uniform tan hypopigmented zone between normal and depigmented skin. The hypopigmented macules progress to full depigmentation. Quadrichrome vitiligo features a fourth, dark-brown pigment, which surrounds hair follicles. Pentachrome vitiligo has a fifth shade and presents with the addition of blue-gray hyperpigmented macules.28,40 Vitiligo is generally classified into either the segmental form, which is rare, or the nonsegmental form, which is common. Segmental vitiligo is characterized by lesions that are restricted to a given body segment and do not cross the midline; it is generally stable after rapid onset and occurs more frequently in children than adults. The nonsegmental form of vitiligo is characterized by widely

distributed patches; it is progressive and may be associated with the Koebner phenomenon and autoimmune disease. It is possible for the segmental and nonsegmental forms to coexist, but these cases are rare and the two forms are generally regarded as distinct, with separate pathogeneses.31,40 Vitiligo may also be classified as localized, generalized, or universal, based on the pattern and extent of involvement.28 The generalized type is most common. Most patients have no associated conditions. However, there have been reports of vitiligo associated with autoimmune diseases.28,29,31,41-44 These conditions include thyroid disease (Hashimoto thyroiditis and Graves disease), pernicious anemia, systemic lupus erythematosus, rheumatoid arthritis, insulin-dependent diabetes, alopecia areata, Addison disease, myasthenia gravis, and autoimmune polyglandular syndrome. The diagnosis can be established on the basis of acquired, well-demarcated, depigmented lesions on the skin, which tend to enlarge centrifugally over time. The lesions are accentuated on skin examination with a Wood lamp. Differential diagnosis includes other causes of acquired hypopigmentation, such as post-inflammatory hypopigmentation, tinea versicolor, pityriasis alba, leprosy, sarcoidosis, and leukoderma that is induced by chemicals or drugs or that is associated with melanoma or scleroderma. 28,29,41 Vitiligo can be easily distinguished from piebaldism because piebaldism typically presents at birth with a single white forelock and amelanocytic lesions distributed on the head and anterior trunk, without periorificial involvement. Piebaldism may also feature hyperpigmented macules within amelanotic areas and is typically stable over time, in contrast to the progressive nature of vitiligo. Treatment options for vitiligo include medical, photo-, and surgical therapy. Repigmentation is the cornerstone of treatment; it is based on the stimulation of melanocytic reserves in the hair follicles. Adults with localized disease and children can be treated for 6 to 12 months with topical corticosteroids, ultraviolet B (UVB) phototherapy, or phototherapy that combines psoralen with long-wave ultraviolet radiation (PUVA) phototherapy. Narrow-band (311-nm) UVB is most effective and safe for generalized vitiligo and can be used for up to 24 months. Immunomodulators, such as tacrolimus and pimecrolimus, may be used as alternatives to steroids.31 Segmental vitiligo and vitiligo of the lips and digits are often treated with autologous transplantation. Patients with extensive disease or those who do not respond to repigmentation may opt for depigmentation therapy with bleaching creams, such as monobenzyl ether of hydroquinone or the Q-switched ruby laser, to achieve complete

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depigmentation. All patients should be advised to wear sunscreen, because vitiligo lesions are ultrasensitive to UV light and decreased tanning of the uninvolved skin will reduce the contrast with hypopigmented areas. In addition, makeup and tanning lotions that contain dihydroxyacetone (DHA) may help to camouflage affected areas temporarily.31 In this case, the patient was treated with tacrolimus ointment. UVB phototherapy was initially recommended but the patient was unable to come in for the thrice-weekly treatments. She was educated regarding the use of camouflaging makeup and with that, she feels less self-conscious. After 3 months of tacrolimus ointment, the patient only had partial repigmentation. She continues to use camouflaging makeup and occasionally gets a “spray tan,” which also provides an acceptable—albeit temporary—cosmetic result. n

1995;56(1):58-66. Available at ncbi.nlm.nih.gov/pmc/articles/PMC1801299 11. Sánchez-Martin M, Pérez-Losada J, Rodríguez-Garcia A, et al. Deletion of the SLUG (SNAI2) gene results in human piebaldism. Am J Med Genet A. 2003;122A(2):125-132. 12. Light-related diseases and disorders of pigmentation. In: Habif TP. Clinical Dermatology: A Color Guide to Diagnosis and Therapy. 5th ed. Philadelphia, Pa.: Mosby Elsevier; 2010: Chap. 19, 764-769. 13. Mahakrishnan A, Srinivasan MS. Piebaldness with Hirschsprung’s disease. Arch Dermatol. 1980;116(10):1102. 14. Angelo C, Cianchini G, Grosso MG, et al. Association of piebaldism and neurofibromatosis type 1 in a girl. Pediatr Dermatol. 2001;18(6):490-493. 15. Köklü S, Ertuğrul D, Onat AM, et al. Piebaldism associated with congenital dyserythropoietic anemia type II (HEMPAS). Am J Hematol. 2002;69(3):210-213. Available at onlinelibrary.wiley.com/doi/10.1002/ ajh.10055/abstract 16. Costa LD, Fixler J, Berets O, et al. Piebaldism in diamond-blackfan

Mr. Fischer and Mr. Rizk are medical students at Baylor College of Medicine in Houston. Dr. Rees is a dermatologist in Los Angeles.

anaemia: a new phenotype? Br J Haematol. 2002;119(2):572. 17. Kiwan RA, Mutasim DF. Grover disease (transient acantholytic dermatosis) and piebaldism. Cutis. 2002;69(6):451-453.

References

18. Spritz R. Letter: Misdiagnosis of ‘‘neurofibromatosis’’ in patients with

1. Ortonne JP, Passeron T. Vitiligo and other disorders of hypopigmen-

piebaldism. Dermatol Online J. 2011;17(11):13. Available at escholarship.org/

tation. In: Bolognia JL, Jorizzo JL, Schaffer JV, eds. Dermatology. 3rd ed.

uc/item/96r0v4fr

Philadelphia, Pa.: Elsevier Saunders; 2012: Chap. 66, 1032-1033.

19. Waardenburg PJ. A new syndrome combining developmental anoma-

2. Agarwal S, Ojha A. Piebaldism: A brief report and review of the litera-

lies of the eyelids, eyebrows and nose root with pigmentary defects of

ture. Indian Dermatol Online J. 2012;3(2):144-147. Available at idoj.in/text.

the iris and head hair and with congenital deafness. Am J Hum Genet.

asp?2012/3/2/144/96722

1951;3(3):195-253. Available at ncbi.nlm.nih.gov/pmc/articles/PMC1716407

3. Ward KA, Moss C, Sanders DS. Human piebaldism: Relationship

20. Ziprkowski L, Krakowski A, Adam A, et al. Partial albinism and

between phenotype and site of KIT gene mutation. Br J Dermatol.

deaf-mutism due to a recessive sex-linked gene. Arch Dermatol.

1995;132(6):929-935.

1962;86:530-539.

4. Thomas I, Kihiczak GG, Fox MD, et al. Piebaldism: An update. Int J

21. Woolf CM. Albinism among Indians in Arizona and New Mexico. Am

Dermatol. 2004;43(10):716-719.

J Hum Genet. 1965;17:23-35.Available at ncbi.nlm.nih.gov/pmc/articles/

5. Fukai K, Hamada T, Ishii M, et al. Acquired pigmented macules in human

PMC1932584

piebald lesions. Ultrastructure of melanocytes in hypomelanotic skin. Acta

22. Smith SD, Kelley PM, Kenyon JB, Hoover D. Tietz syndrome

Derm Venereol. 1989;69(6):524-527.

(hypopigmentation/deafness) caused by mutations of MITF. J Med Genet.

6. Richards KA, Fukai K, Oiso N, Paller AS. A novel KIT mutation results

2000;37(6):446-448. Available at jmg.bmj.com/content/37/6/446.long

in piebaldism with progressive depigmentation. J Am Acad Dermatol.

23. Suga Y, Ikejima A, Matsuba S, Ogawa H. Medical pearl: DHA applica-

2001;44(2):288-292.

tion for camouflaging segmental vitiligo and piebald lesions. J Am Acad

7. Giebel LB, Spritz RA. Mutation of the KIT (mast/stem cell growth factor

Dermatol. 2002;47(3):436-438.

receptor) protooncogene in human piebaldism. Proc Natl Acad Sci U S A.

24. Falabella R, Barona M, Escobar C, et al. Surgical combination therapy

1991;88(19):8696-8699. Available at pnas.org/content/88/19/8696.long

for vitiligo and piebaldism. Dermatol Surg. 1995;21(10):852-857.

8. Spritz RA. Molecular basis of human piebaldism. J Invest Dermatol.

25. Njoo MD, Nieuweboer-Krobotova L, Westerhof W. Repigmentation

1994;103(5 suppl):137S-140S.

of leucodermic defects in piebaldism by dermabrasion and thin split-

9. Fleischman RA, Saltman DL, Stastny V, Zneimer S. Deletion of the c-KIT

thickness skin grafting in combination with minigrafting. Br J Dermatol.

protooncogene in the human developmental defect piebald trait. Proc

1998;139(5):829-833.

Natl Acad Sci U S A. 1991;88(23):10885-10889. Available at pnas.org/con-

26. Neves DR, Régis Júnior JR, Oliveira PJ, et al. Melanocyte transplant in

tent/88/23/10885.long

piebaldism: Case report. An Bras Dermatol. 2010;85(3):384-388. Available

10. Ezoe K, Holmes SA, Ho L, et al. Novel mutations and deletions of the

at ref.scielo.org/qtmqb4

KIT (steel factor receptor) gene in human piebaldism. Am J Hum Genet.

Continues on page 76

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27. Van Geel N, Wallaeys E, Goh BK, et al. Long-term results of noncul-

36. Spritz RA. The genetics of generalized vitiligo and associated autoim-

tured epidermal cellular grafting in vitiligo, halo naevi, piebaldism and nae-

mune diseases. Pigment Cell Res. 2007;20(4):271-278. Available at onlineli-

vus depigmentosus. Br J Dermatol. 2010;163 (6):1186-1193.

brary.wiley.com/doi/10.1111/j.1600-0749.2007.00384.x/full

28. Ortonne JP, Passeron T. Vitiligo and other disorders of hypopigmen-

37. Vogt A. Frühzeitiges Ergrauen der Zilien und Bemerkungen uber

tation. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. 3rd ed.

den sogenannten plötzlichen Eintritt dieser Veranderung. Klin Monatsbl

Philadelphia, Pa.: Elsevier Saunders; 2012: Chap. 66, 1023-1030.

Augenheilk. 1906;44:228-242.

29. Section 13: Pigmentary disorders. In: Wolff K, Johnson RA, Saavedra

38. Harada E. On the acute diffuse choroiditis. Acta Soc Ophthalmol Jpn.

AP, eds. Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology. 7th ed.

1926;30:356-378.

New York, N.Y.: McGraw-Hill Educational; 2013. 284-290.

39. Koyanagi Y. Dysakusis, Alopecia und Poliosis bei schwerer Uveitis nicht

30. Taïeb A, Picardo M. Clinical practice: Vitiligo. N Engl J Med.

traumatischen Ursprungs. Klin Monatsbl Augenheilk. 1929;82:194-211.

2009;360(2):160-169.

40. Yaghoobi R, Omidian M, Bagherani N. Vitiligo: a review of the pub-

31. Light-related diseases and disorders of pigmentation. In: Habif TP.

lished work. J Dermatol. 2011;38(5):419-431.

Clinical Dermatology: A Color Guide to Diagnosis and Therapy. Philadelphia,

41. Birlea SA, Fain PR, Spritz RA. A Romanian population isolate with high

Pa.: Mosby Elsevier; 2010: Chap. 19, 764.

frequency of vitiligo and associated autoimmune diseases. Arch Dermatol.

32. Moellmann G, Klein-Angerer S, Scollay DA, et al. Extracellular

2008;144(3):310-316.

granular material and degeneration of keratinocytes in the nor-

42. Daneshpazhooh M, Mostofizadeh GM, Behjati J, et al. Anti-thyroid per-

mally pigmented epidermis of patients with vitiligo. J Invest Dermatol.

oxidase antibody and vitiligo: a controlled study. BMC Dermatol. 2006;6:3.

1982;79(5):321-330.

Available at biomedcentral.com/1471-5945/6/3

33. Galadari I, Bener A, Hadi S, Lestringant GG. Clinical and immunologi-

43. Alkhateeb A, Fain PR, Thody A, et al. Epidemiology of vitiligo and

cal studies in vitiligo in the United Arab Emirates. Allerg Immunol (Paris).

associated autoimmune diseases in Caucasian probands and their families.

1997;29(10):297-299.

Pigment Cell Res. 2003;16(3):208–214.

34. Lerner AB. On the etiology of vitiligo and gray hair. Am J Med.

44. Bloch MH, Sowers JR. Vitiligo and polyglandular autoimmune endocri-

1971;51(2):141-147.

nopathy. Cutis. 1985;36(5):417-419.

35. Mohr J. Vitiligo in a pair of monovular twins. Acta Genet Stat Med. 1951;2(3):252-255.

All electronic documents accessed on November 15, 2014.

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CME CE

POSTTEST

Expiration date: December 9, 2015

DERMATOLOGY COURSES CREDITS: 0.5

For more credit information, please turn to p. 66

Dermatology Clinic

Dermatologic Look-Alikes

page 67

page 71

Case #1: Vibrio vulnificus infection

Case #1: Piebaldism

1. A 40-year-old man who works as a cook in a local seafood restaurant presents with gastroenteritis and violaceous bullae and purpura. He is hemodynamically stable. You suspect a case of cutaneous vibriosis. Which of the following antibiotics would be the best treatment choice? a. Doxycycline b. Gentamicin c. Ciprofloxacin d. Cefotaxime

1. How is piebaldism inherited? a. It is not inherited. b. Autosomal dominant, due to mutation in the c-kit gene c. Autosomal recessive, due to mutation in the TGM1 gene d. X-linked recessive, due to mutation in the gene encoding steroid sulfatase

2. Which of the following radiographic findings are typically seen in cases of systemic Vibrio vulnificus? a. Lobar consolidation b. Unilateral or bilateral hilar enlargement c. Multiple, ill-defined, patchy areas of consolidation d. Bilateral, fluffy pulmonary infiltrates Case # 2: Eruptive syringomas 3. A patient presents with numerous firm, rose-colored papules on the anterior trunk with acute onset. You suspect a diagnosis of syringomas but are unsure. What would be the next best step for diagnosis? a. Skin biopsy b. Viral culture c. Bacterial culture d. Fungal culture 4. Upon confirmation of a diagnosis of eruptive syringomas in the above patient, what should be your next step? a. Inform the patient that the lesions will resolve in 1 to 2 weeks. b. Refer the patient to surgery for excision. c. Inform the patient that these lesions tend to persist for life, although there are rare reports of resolution in adulthood, and no treatment is very effective. d. Inform the patient that these lesions are malignant, and refer them to oncology.

2. What is present in most patients with piebaldism? a. Depigmented patches on distal extremities b. Mental retardation c. Dysmorphic facial features d. A white forelock Case #2: Vitiligo 3. A female patient with vitiligo of the neck and forearms is very distressed, because she is getting married in 8 months and is embarrassed that the vitiligo is noticeable in her sleeveless wedding dress. Which is the most effective treatment to pursue for this patient? a. Narrow-band ultraviolet-B phototherapy b. Topical clobetasol c. Topical dihydroxyacetone d. Psoralen with ultraviolet-A phototherapy 4. Which of the following diseases is most commonly associated with vitiligo? a. HIV/AIDS b. Congestive heart failure c. Hashimoto thyroiditis d. Hodgkin lymphoma

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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.

Fluoride

An ionic form of the chemical element fluorine, fluoride exists naturally on earth and is well-known for its ability to help prevent dental caries. The U.S. Public Health Service began fluoridating public water supplies in 1951,1 and, as of 2006, nearly 70% of Americans received fluoride through these sources.2 Hailed as one of the top 10 accomplishments in public health in the 20th century, this practice has saved billions of dollars in health-related costs and has prevented immeasurable pain and disability.3

Background Researchers became interested in fluoride’s role in dental health when they noticed that certain areas of the United States had fewer cases of severe tooth decay than others. Groundwater in regions that were rich in fluoride had high amounts of the ion, whereas groundwater in areas with low concentrations had negligible amounts of fluoride. This discovery led to public-health policies that added fluoride to drinking water, a practice that has been estimated to reduce dental caries in both children and adults by up to 40%.4 During the latter half of the 20th century, researchers also discovered the role fluoride plays in the development of strong bones.

Science The basic mechanism of action by which fluoride prevents cavities in teeth and promotes strong bones is by enhancement of the remineralization that occurs naturally and constantly

in these tissues.5 When the pH in the mouth falls below 5.5, tiny craters can begin to form on the tooth surface. If left untreated, these areas eventually become a true cavity. Fluoride ions in saliva bond with the ambient hydroxyapatite from the dissolved enamel, speed remineralization, and prevent further erosion of the enamel.5 Dental caries are not affected by the ingestion of fluoride until after tooth eruption. A similar mechanism strengthens bone matrix. Bone mass is a constant balance between the activity of osteoblasts, which build new bone, and osteoclasts, which tear away old bone. Supporting the use of fluoride for osteoporosis, early studies documented increased bone mineral density of the lumbar spine, one of the areas most susceptible to fracture, following fluoride treatment at certain doses.6 However, later data increasingly showed that too much fluoride led to bone that was more brittle and increased nonvertebral fractures.7 Since the utility of fluoride for dental health was established, commercial products began to appear and dominate the oralhealth marketplace. Multiple studies now verify the safety

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ALTERNATIVE MEDS UPDATE and efficacy of the routine use of fluoridated toothpastes and mouthwashes. A recent metaanalysis found that topical fluoride was as effective as fluoridated water in the prevention of dental caries.8 Since the late 1970s, a trend toward fewer caries has been documented, even in areas that do not fluoridate public water supplies.9

(7 Suppl):1-4. Available at nature.com/bdj/journal/v199/n7s/ full/4812863a.html 2. Centers for Disease Control and Prevention. Community water fluoridation: 2010 water fluoridation statistics. Available at cdc.gov/fluoridation/statistics/2010stats.htm 3. Centers for Disease Control and Prevention. Achievements in public health, 1900-1999: Fluoridation of drinking water to prevent dental caries. MMWR. 1999;48(41):933-940. Available

Safety, interactions, side effects

at cdc.gov/mmwr/preview/mmwrhtml/mm4841a1.htm 4. Griffin SO, Regnier E, Griffin PM, Huntley V.

In developed countries around the world, at least a small amount of fluoride occurs naturally in groundwater and food.10 The National Institutes of Health and the Academy of Nutrition and Dietetics have issued recommendations for the minimum daily intake of f luoride from all sources.10,11 Fluoride levels of community water in the United States are set at 0.7 mg/L, which results in the need to defluorinate in some areas that are rich in ground fluoride.1,12 The maximum amount of elemental fluoride that is safe for daily ingestion is 10 mg.13 Excessive, chronic ingestion of fluoride can increase bone fragility, discolor tooth enamel, and cause severe gastric distress.10 The only specific contraindication against additions of fluoride to the diet is for postmenopausal women with known osteoporosis.

Effectiveness of fluoride in preventing caries in adults. J Dent Res. 2007;86(5):410-415.

Flouride levels vary by region; this is reflected by an area’s groundwater.

A recent meta-analysis found that topical fluoride was as effective as fluoridated water in the prevention of dental caries.

Cost, how supplied, dose

5. Cury JA, Tenuta LM. How to maintain a cariostatic fluoride concentration in the oral environment. Adv Dent Res. 2008;20(1):13-16. 6. Vestergaard P, Jorgensen NR, Schwarz P, Mosekilde L. Effects of treatment with fluoride on bone mineral density and fracture risk—a meta-analysis. Osteoporos Int. 2008;19(3):257-268. 7. Gutteridge DH, Stewart GO, Prince RL, et al. A randomized trial of sodium fluoride (60 mg) +/- estrogen in postmenopausal osteoporotic vertebral fractures: increased vertebral fractures and peripheral bone loss with sodium fluoride; concurrent estrogen prevents peripheral loss, but not vertebral fractures. Osteoporos Int. 2002;13(2):158-170. 8. Pizzo G, Piscopo MR, Pizzo I, Giuliana G. Community water fluoridation and caries prevention: a critical review. Clin Oral Investig. 2007;11(3):189-193. 9. Hellwig E, Lennon AM. Systemic versus topical fluoride. Caries Res. 2004;38(3):258-262. Available at karger.com/ Article/FullText/77764

Fluoride is supplied mainly through community water supplies and topical fluoride products, which are usually in the form of toothpaste or mouthwash and costs from $3 to $7 per unit.

Summary

10. Hamrick I, Counts SH. Vitamin and mineral supplements. Prim Care. 2008;35(4):729-747. 11. Palmer C, Wolfe SH; American Dietetic Association. Position of the American Dietetic Association: the impact of fluoride on health. J Am Diet Assoc. 2005;105(10):1620-1628. Available at andjrnl.org/article/S0002-8223(05)01391-X/fulltext 12. U.S. Department of Health and Human Services. Proposed HHS recommendation for fluoride concentration in drinking water for prevention of dental caries. Available at federalregister.gov/a/2011-637. 13. U.S. Institute of Medicine Standing Committee on the Scientific Evaluation of Dietary Reference Intakes. Dietary Reference Intakes for Calcium, Phosphorus, Magnesium, Vitamin D, and Fluoride. Washington, D.C.: National Academies Press;

References

1997. Available at nap.edu/openbook.php?record_id=5776

1. Mullen J; European Association for Paediatric Dentistry. History of water fluoridation. Br Dent J. 2005;199

All electronic documents accessed on November 15, 2014.

The efficacy of fluoride in the prevention of dental caries is well-established. Cultural development has potentially eliminated the need for fluoridation of public water supplies. However, the efficacy of topical products is only as good as compliance with their use. n

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COMMENTARY Paula Dunn Tropello, EdD, FNP-BC, dean of the Evelyn L. Spiro School of Nursing at Wagner College in Staten Island, N.Y., specializes in global and disaster nursing.

Don’t fumble the patient hand-off The basic goal of good communication in health care has been to ensure safety, continuity of care, and efficiency of care; communication between providers is a key element of that effort. However, with the implementation of electronic health records (EHRs) in recent years, the opportunity for actual face-to-face communication between clinicians to inform each other of a patient’s status has diminished. Managed care and the need to see more patients in a shorter period of time has contributed greatly to providers’ use of the EHR as the sole means of obtaining patient information prior to the patient encounter. One often finds that the patient needs to be asked the same

The travel history of a man with Ebola had not been obtained by the clinician making the diagnosis.

questions that already had been asked on intake in order to verify the reason for the visit. This takes up valuable time that might be better used to get details that would enhance the visit and make for a better patient outcome. Mnemonics such as SBAR (Situation, Background, Assessment, Recommendation) that guide hand-off procedures in many inpatient facilities are adaptable for primary- or acute-care situations (Reisenberg LA, Leitzsch J, Little BW. Systematic review of handoff mnemonics literature. American Journal of Medical Quality. 2009;24[3]:196-204). Although technology might have weakened the idea that verbal patient hand-offs from one clinician to another are important, such exchanges remain key to patient care, as evidenced by the recent Ebola case at Texas Health Presbyterian Hospital Dallas. When the patient from West Africa did not receive the diagnosis of Ebola in a timely fashion, he had the potential to infect many others with whom he had contact. The EHR was the source of information and the patient’s travel history had not been obtained by the provider making the diagnosis. We cannot be sure whether a more timely diagnosis would have made a difference in the man’s survival, but we do know that it has been a teaching moment for many practitioners and for the health-care system. If the clinician who did the intake had simply stated to the next provider that this person had just

arrived from West Africa, where there is an Ebola outbreak, a better outcome could have been achieved initially at least. There also would have been more trust in the health-care system in general, and this hospital in particular might have had the opportunity to alert the Centers for Disease Control and Prevention (CDC) before the situation became emergent. What can nurse practitioners (NPs) and physician assistants (PAs) learn from this sentinel event? The legal implications of the EHR have great impact on the provider when this record is the only source used to obtain background information and updates on a patient. A crucial piece of information delivered during a verbal hand-off may be small but if not conveyed, the consequences could be devastating, as with the index Ebola patient. In patient care, all forms of communication are equally valuable, and none should be left out simply to stay on schedule or to bring in more money. Documentation, report, and hand-off, as well as the EHR, all improve patient care and continuity of care so that the system works better for all of us. NPs and PAs should stand strong in communicating with all members of the health-care team rather than remaining in isolation with the computer, accessing information and possibly missing a sentinel ingredient that would enhance patient care and ultimately safety and outcomes. n

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