Fibrillary glomerulopathy in a 10-year-old female

A 10-year-old African-American female was evaluated for asymptomatic protei nuria. Initial investigation did not reveal the etiology of her renal disea se. She subsequently underwent a percutaneous renal biopsy, which was consi stent with fibrillary glomerulopathy. This condition is rare in children an d is identified histologically by a solid, randomly arranged, amyloid-like fibrillar structure with a diameter of about 18-22 nm. It is a diagnosis of exclusion, the clinical presentation of which can be quite varied. Childre n usually present with nephrotic syndrome. There is no effective therapy fo r this condition. Therapy with steroids, cytotoxic agents, and plasmapheres is does not confer any real benefit in stabilizing or improving renal funct ion. However, angiotensin converting enzyme inhibitors can decrease the pro teinuria. End-stage renal disease is known to occur in 50% of patients with in 4 years of diagnosis. The nephrotic-range proteinuria in our patient sig nificantly decreased during, a 4-year follow-up without any therapy, while maintaining normal renal function.