Week 1 Answers


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Week 1 Answers

  1. 1. SEPT QUIZ 1 CRITIQUEQuestion 1An 11-year-old boy presents for evaluation of recurrent oral ulcers, joint pain, and weight loss for 3months.Of the following, the MOST likely explanation for these findings isA Crohn diseaseB cyclic neutropeniaC hand-foot-and-mouth diseaseD herpanginaE herpetic gingivostomatitisCritique AThe clinical triad of recurrent oral ulcers (aphthae), joint pain, and weight loss exhibited by theboy described in the vignette suggests a diagnosis of Crohn disease. Extraintestinalmanifestations occur in 25% to 35% of patients who have inflammatory bowel disease. Almostany system can be involved, but the skin and mucosa, joints, liver, eye, and bone are affectedmost often.Oral aphthae occur in as many as 20% of patients who have Crohn disease. They are painfululcers or erosions on the lips, gingivae, tongue, palate, or buccal mucosa (Item C221A).Individual lesions last 7 to 10 days and may occur in crops at irregular intervals. Aphthaeparallel disease activity, but may precede the appearance of intestinal symptoms. Oral ulcersalso are a feature of cyclic neutropenia, a rare disorder in which periodic fluctuations incirculating neutrophil numbers are associated with fever, pharyngitis, and lymphadenopathy.Symptoms occur at regular intervals and are associated with a nadir in bone marrow functionand consequent neutropenia.A number of viral infections produce oral ulcers or erosions, but they may be distinguishedfrom Crohn disease because they are self-limited, may have distinctive extraoral findings, andare not associated with chronic weight loss or joint pain. Hand-foot-and-mouth diseaseproduces shallow ulcers that involve the soft palate, uvula, tonsillar pillars, and tongue (ItemC221B). Most affected patients also exhibit erythematous papules or oval vesicles that haveerythematous borders and are located on the palms (Item C221C), soles, and digits. Small (1-to 2-mm) vesicles and ulcers that involve the posterior oropharynx, including the tonsillarpillars, soft palate, and uvula, are characteristic of herpangina (Item C221D). Children whohave herpetic gingivostomatitis exhibit fever, irritability, and refusal to eat. Vesicles and ulcersare observed on the anterior soft palate, tongue, and gingivae. The gingivae are edematous,friable, and inflamed. Vesicles and erosions also may appear on the lips and chin (ItemC221E).Question 2You are following an 11-year-old girl who has Crohn disease involving the stomach, ileum, andcolon. Her maintenance medications are mesalamine and 6-mercaptopurine. Over the pastyear, she has received four courses of corticosteroid treatment, but continues to haveintermittent abdominal pain and diarrhea. Upon review of her growth curve, you note that herheight has been the same over the past 12 months. You suspect that the combination of Crohndisease and corticosteroid therapy has resulted in growth arrest. You discuss your concernswith her gastroenterologist.Of the following, the MOST appropriate medication to control this patients disease and reduceher dependence on corticosteroids is
  2. 2. A cyclophosphamideB infliximabC mycophenolate mofetilD tacrolimusE ThalidomideCritique BCrohn disease (CD) and ulcerative colitis (UC) are serious illnesses mediated by the immunesystem that cause intestinal inflammation. The inflammation in UC is limited to the mucosallayer of the large bowel. Thus, affected patients most commonly present with pain associatedwith defecation, diarrhea, and rectal bleeding. In contrast, the inflammation in CD istransmural (involving the entire thickness of the bowel) and can involve any region of thegastrointestinal tract (most commonly the ileum and cecum). Therefore, patients who have CDpresent with a wide variety of symptoms, including diarrhea, rectal bleeding, anorexia, growthfailure, anemia, abdominal pain, and perianal disease. The diagnosis of inflammatory boweldisease is established by contrast radiography, upper endoscopy, and colonoscopy.Therapy for inflammatory bowel disease involves a combination of medical and nutritionaltherapy. The administration of a liquid diet (elemental or polymeric formula) for 6 to 8 weeks,combined with the cessation of eating during this period, may induce a remission in childrenwho have CD, but this approach is not effective in UC. In addition, it is difficult to maintainsuch a diet indefinitely. For this reason, medications are essential in the long-termmanagement of this condition. Most commonly, corticosteroids are used to induce remission inmoderate-to-severe CD or UC. Salicylates (eg, mesalamine) are useful in maintainingremission in UC, but are less effective in CD. Immunomodulators (6-mercaptopurine,azathioprine, methotrexate) are used to maintain remission in patients who have UC and failsalicylate therapy and in those who have CD. Patients who have CD and fail salicylate andimmunomodulator therapy, such as the girl described in the vignette, benefit from the additionof infliximab, an antibody to tumor necrosis factor-alpha. Infliximab has been approved by theUnited States Food and Drug Administration for use in children who have CD. Although thismedication is highly effective treatment, patients have an increased risk of opportunisticinfections (especially tuberculosis) and lymphoma. Therefore, a physician and patientconsidering infliximab need to weigh the risks and benefits carefully. Tacrolimus andthalidomide sometimes are used in the treatment of CD if patients failed or are intolerant toinfliximab. Cyclophosphamide and mycophenolate rarely are used to treat inflammatory boweldiseaseQuestion 3A 16-year-old girl comes to your office complaining of irregular menstrual periods. She hadmenarche at 11 years of age and experiences menstrual periods every 2 weeks to 3 months.She has noticed increased acne, facial hair growth, and a 20-lb weight gain over the past year.Of the following, the MOST likely diagnosis is
  3. 3. A Cushing syndromeB hypothyroidismC Noonan syndromeD ovarian tumorE polycystic ovary syndromeCritique ESigns of androgen excess (especially hirsutism and acne) combined witholigomenorrhea or anovulatory bleeding, as described for the adolescent girlin the vignette, should alert the clinician to the possibility of polycystic ovarysyndrome (PCOS). PCOS is the most common endocrinopathy inpremenopausal women, but its clinical criteria for diagnosis, pathophysiology,and treatment remain controversial. Definite or probable criteria for diagnosisinclude laboratory or clinical hyperandrogenism, menstrual dysfunction, andexclusion of congenital adrenal hyperplasia. Other criteria often used tosupport a diagnosis include insulin resistance, perimenarcheal onset, elevatedluteinizing hormone-to-follicle-stimulating hormone ratio, andultrasonographic abnormalities. Abnormalities observed in patients who havePCOS occur in four key areas: 1) increase in luteinizing hormone secretion, 2)increase in adrenal androgen production, 3) increase in body mass, and 4)onset of adult patterns of insulin resistance. Some girls have a transientperiod of hyperandrogenism during the first 3 years after menarche; othershave persistent PCOS symptoms. Patients who have PCOS are at increasedrisk for diabetes mellitus, obesity, insulin resistance, infertility, and impairedquality of life.Cushing syndrome is less likely in the absence of other stigmata of thesyndrome, including weakness, spontaneous ecchymoses, large purple striae(Item C73A), hypokalemia, and osteoporosis. A rapid onset of hirsutism withvirilization is characteristic of ovarian tumors. Hypothyroidism can causemenstrual irregularities and weight gain, but is not associated with androgenexcess. Patients who have Noonan syndrome (Item C73B) may present withdelayed puberty and associated amenorrhea, but they do not exhibitandrogen excess and weight gain.Question 4A 14-year-old boy requests treatment for his acne. He is using no medicationsand has no known drug allergies. Physical examination of the face reveals a fewsmall inflammatory papules and numerous blackheads and whiteheads ; there isno scarring. No acne lesions are present on the chest and back.Of the following, the MOST appropriate treatment isA benzoyl peroxide topicallyB benzoyl peroxide topically and tetracycline orallyC benzoyl peroxide topically and tretinoin topicallyD clindamycin topicallyE tretinoin topicallyCritique CAcne affects 85% or more of adolescents and is the skin disorder most oftentreated by physicians. Multiple factors contribute to acne, including bacteria(that induce an inflammatory response, leading to erythematous papules andpustules (Item C97A), androgens (that cause increased sebum production),and abnormal follicular keratinization (that causes blockage within pores,resulting in blackheads and whiteheads (Item C97B). The treatment of acne