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A Hypertensive Child With Irritability and a Rash

A16-month-old previously healthy boy is evaluated for an unusual constellation of symptoms. Approximately 22 months ago, he developed symptoms consistent with an upper respiratory tract infection including cough, runny nose, and congestion. One week after the onset of his upper respiratory tract infection symptoms, he developed swelling and intermittent purple discoloration of his fingertips. His mother reports the subsequent onset of irritability, poor energy, decreased appetite, excessive sweating, and ‘‘spots’’ on his forearms and hands. These symptoms have not been treated with medications thus far. Further questioning notes that the child has not been having fevers, his weight has been stable (despite decreased eating), stools have been firm, and he has been reluctant to ambulate as usual. A physical examination in his pediatrician’s office a few weeks ago had been unremarkable except for tachycardia. A chest radiograph was obtained and read as normal. A complete blood count was unremarkable, and comprehensive serum chemistry evaluation noted an aspartate aminotransferase (AST) concentration of 84 U/L and an alanine aminotransferase (ALT) concentration of 166 U/L. A course of amoxicillin was prescribed, and consultation with cardiology was arranged. The child was evaluated by a cardiologist, and an echocardiogram and electrocardiogramwere normal. Concerned that the child’s symptoms may represent a process more worrisome than the common cold, the pediatrician recommended consultation with a pediatric rheumatologist. At this point, 3 diagnoses were considered: juvenile rheumatoid arthritis, dermatomyositis, and cytomegalovirus infection. Further testing included a second complete blood count and comprehensive metabolic panel with the addition of an erythrocyte sedimentation rate, antinuclear antibody test, C-reactive protein quantitation, Epstei nBarr virus/cytomegalovirus serology, and serum aldolase. The only noted abnormalities on this battery of tests were an AST of 90 U/L (reference range, 24Y47 U/L) and an ALT of 190 U/L (normal reference range, 13Y48 U/L). The boy is now at a tertiary pediatric hospital for complete abdominal ultrasonography (US) and for comprehensive medical assessment and recommendations. His blood pressure (BP) is remarkable at 140/112 mm Hg and accompanied by a heart rate of 119 beats/min. He does not appear in distress but is refusing to walk and is continuously rubbing his notably swollen hands together. He is hospitalized for treatment of grade II hypertension. Four-limb BPs are obtained and noted to be as follows: right upper extremity, 119/86 mm Hg; right lower extremity, 137/58 mm Hg; left upper extremity, 140/112 mm Hg; and left lower extremity, 129/92 mm Hg. Labetalol 0.2 mg/kg was administered intravenously with a systolic BP goal of 110 mm Hg. Further testing notes proteinuria and hematuria. Serum AST and ALT have risen to 333 and 567 U/L, respectively. The abdominal US does not discover any anatomic abnormalities. The pediatric team plans further testing to include bilateral renal US with Doppler flow assessment, complement concentrations, serum lactate dehydrogenase concentration, antineutrophil cytoplasmic antibody testing, anti-DNAse B measurement,thyroidstudies,serumrenin and aldosterone levels, and urine vanillylmandelic acid/homovanillic acid measurement. The diagnosis, however, is made after an astute clinician suggests the addition of a previously unconsidered test.