Abstract
A 14-year-old boy with obesity was referred to our center for evaluation of elevated blood pressures. His past medical history was notable for prematurity. Gestational age was 31.5 weeks, and neonatal issues included respiratory distress, hyperbilirubinemia, and grade I intraventricular hemorrhage. He did not have umbilical lines. He also had a history of tonsillectomy, adenoidectomy, and myringotomy tubes. His family history was relevant for his mother having longstanding hypertension and proteinuria, and his maternal grandmother died in her sixth decade of life secondary to cardiac amyloidosis and multiple myeloma. She presented with end-stage kidney disease (ESKD) a few years earlier and did not have a kidney biopsy. Physical examination was unremarkable except for a blood pressure of 154/103 and obesity. On random urinalysis, he had 2+ proteinuria and no microhematuria. His laboratory studies showed normal kidney function with serum creatinine of 0.6 mg/dl, normal electrolytes, mild hypoalbuminemia (albumin 3.5 g/dl) and anemia (hemoglobin 11.3 g/dl), Fig. 1 Kidney biopsy findings. a Light microscopy shows striking glomerulomegaly and lobularity of glomerular tufts, marked mesangial hypercellularity, and markedly increased mesangial matrix [hematoxylin and eosin (H&E) stain, 200×]. b Periodic acid-Schiff (PAS) stain shows increased amounts of PAS-positive material in the capillary loops with irregular thickening (400×). c, d Scattered tubular atrophy, moderate patchy interstitial fibrosis, mild patchy interstitial inflammation, and globally sclerotic glomeruli seen on H&E and trichrome stains, respectively (200×). e Electron microscopy (EM) shows extensive mesangial and subendothelial lightly electron-dense, granular deposits and partial effacement of foot processes. f Fibrillary appearance of deposits (small randomly oriented fibrils) on higher magnification. Note: Immunofluorescence was negative for IgA, IgG, IgM, C3, and C1q, immunohistochemistry was negative for kappa and lambda light chains, and Congo Red staining was negative The answers to these questions can be found at http://dx.doi.org/10.1007/ s00467-015-3128-0.
Published Version
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