성인에서 발생한 Glomerulocystic Kidney Disease 1예

Abstract

Glomeuloncystic kidney disease(GCKD) is an uncommon type of renal cystic disease characterized by dilatation of Bowman's space with variable atrophy of glomerular tuft. GCKD is not widely recognized as a condition that affect adults, because most of the patients were neonate or infants with other associated congenital abnormalities in other organs. We experineced a case of GCKD in a 40-year-old woman who was admitted because of seizure attack and severe reanal failure. Abdominal ultrasonography and computed tomography revealed numerous small cysts confined to the renal cortex and numerous small cysts confined to the renal cortex and renal biopsy specimen showed cystic dilatation of Bowman's space with atrophic glomerular tuft. She also had a family histordy on cystic renal disease. A GCKD can be considered as a cause of renal dysfunction in adult patients having enlarged kidneys.