Autosomal Recessive Polycystic Kidney Disease

• Etiology: microcystic disease occurring in the distal convoluted tubules and collecting ducts
• US: bilateral enlarged / echogenic / nonfunctioning kidneys, may also have hepatic fibrosis and intra + extrahepatic biliary dilation with there being an inverse relationship between hepatic + renal involvement, individual renal microcyts may be difficult to resolve
• Clinical: may present with oligohydramnios, pulmonary hypoplasia, respiratory failure

Cases of Autosomal Recessive Polycystic Kidney Disease