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The answer is b. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy, or PFAPA syndrome is a relatively common cause of recurrent fever in children, but its true prevalence is unknown. Although the exact cause has yet to be determined, this syndrome results in stereotypic attacks of fever lasting 4 to 5 days recurring on a fairly regular interval of approximately 4 to 5 weeks. Although not contained in the acronym, abdominal pain accompanies febrile episodes in up to 60% of patients. Children with PFAPA are completely healthy between attacks and display normal growth and development. During attacks, inflammatory markers such as the ESR and CRP are typically elevated, but return to normal between attacks. Children who remain ill between febrile episodes, have atypical growth and/or development, or persistently elevated inflammatory markers are more likely to have a more serious cause of their recurrent fevers. In general, the prognosis of children with PFAPA is good with nearly 40% either entering spontaneous remission or reduction in frequency and severity of attacks within 2 to 5 years of symptom onset.

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The answer is a. There are several treatment options for PFAPA. A single dose of oral prednisone or prednisolone (0.5-2 mg/kg) has been shown to abort attacks if given at the first sign of fever in >90% of PFAPA patients. However, almost 20% of patients who use prednisone to manage their attacks suffer an increase in the frequency and severity of attacks. Cimetidine and colchicine have also been used to prevent PFAPA episodes with success in 33% of patients. The medication must be given every day and missing a single dose can result in an attack. Adenotonsillectomy has been gaining popularity in the management of this condition. To date, close to 100 patients with PFAPA have undergone tonsillectomy reported in the literature, which has resulted in complete cessation of attacks in virtually 100% patients within 12 months after the procedure. There have been no
reported adverse events. Infliximab and methotrexate have not been used for treatment of PFAPA.

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The answer is c. Juvenile fibromyalgia syndrome (jFMS) is an increasingly recognized condition. Patients with jFMS complain of a combination of the following six symptoms: fatigue, disordered and nonrestorative sleep, chronic headaches, nonspecific gastrointestinal complaints, widespread musculoskeletal pain above and below the waist, and chronic anxiety or tension. In addition to these somatic complaints, these patients have exquisite tenderness at characteristic musculoskeletal tender points on physical examination. Several medications have recently been marketed as treatment for adult fibromyalgia, including pregabalin and duloxetine. At the time of publication, there have been no prospective studies examining the safety or efficacy of these medications in the treatment of jFMS. Experts in jFMS recommend a multidisciplinary treatment plan including intensive physical therapy, cognitive behavioral therapy, and improved sleep hygiene. Adjunctive use of amitriptyline (<75 mg) in combination with a multidisciplinary care plan may also be beneficial. Longitudinal studies on patients with jFMS are few so prognosis cannot be stated with accuracy. However, successful participation in and completion of a multidisciplinary care plan usually results in sustained return of function but pain may persist for years.

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The answer is a. This patient’s signs and symptoms are suggestive of vasculitis, specifically Takayasu’s arteritis (TA). TA is a large-vessel vasculitis causing stenotic and rarely aneurismal changes in the aorta and its branches. The thoracic aorta and its branches are commonly involved but the abdominal aorta and renal arteries may be more frequently affected in children. Children with TA may present with constitutional symptoms such as fatigue and malaise or hypertension. In addition, children are less likely than adults to complain of carotidynia (pain over course of internal carotid arteries) and claudication. Recently proposed classification criteria for childhood TA require demonstration of angiographic abnormalities of the aorta or its primary branches using magnetic resonance, computed tomography, or conventional angiography plus at least one of the four following features: