A case of bilateral renal hypoplasia and neonatal hemochromatosis.

Abstract

Neonatal hemochromatosis is a severe, often fatal multiorgan disorder of iron metabolism. We report a case of neonatal hemochromatosis associated with bilateral renal hypoplasia. The patient also had congenital intestinal atresia and meconium peritonitis. The radical operation was performed for congenital intestinal atresia at one-day-old. After surgery, oliguria occurred and renal disorder progressed to chronic renal failure. Subsequently, liver dysfunction and hyperferritinemia appeared. Bilateral hypoplastic kidney was diagnosed by CT scan, and MRI demonstrated abnormal iron deposition in the liver, pancreas and renal cortex at 2-month-old, indicating hemochromatosis. The hemochromatosis slowly improved by withdrawal of blood and oral vitamin E. Peritoneal dialysis was initiated for chronic renal failure at 10-month-old. We conclude that this is a very rare case of renal hypoplasia complicated with neonatal hemochromatosis.