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Autosomal recessive polycystic kidney disease (ARPKD). (A) Baby with distended abdomen due to voluminous kidneys that lead to respiratory problems. (B) Abdominal situs of a perinatally demised ARPKD patient with symmetrically enlarged kidneys that maintain their reniform configuration. (C) Cross section of an ARPKD kidney with cortical extension of fusiform and cylindrical spaces arranged radially throughout the renal parenchyma from medulla to cortex. (D,E) Microscopically, fusiform dilations of renal collecting ducts and distal tubuli lined by columnar or cuboidal epithelium. These dilated collecting ducts run perpendicular to the renal capsule. (F) Obligatory hepatobiliary changes in ARPKD known as ductal plate malformation characterized by dysgenesis of the hepatic portal triad with hyperplastic biliary ducts and congenital hepatic fibrosis. (G–I) Renal ultrasound of young children with ARPKD demonstrating enlarged echogenic kidneys with fusiform dilations of collecting ducts and distal tubules arranged radially throughout the renal parenchyma from medulla to cortex.
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