Autosomal recessive polycystic kidney disease. Definition

Medical Definition: autosomal recessive polycystic kidney disease

One in 40,000 babies have autosomal recessive polycystic kidney disease, associated in all cases, with some form of liver, especially dilated bile ducts and, especially, periportal fibrosis (congenital hepatic fibrosis). When renal involvement is early, what happens very often, have oligohydramnios and, therefore, respiratory distress is common in pulmonary fibrosis. Clinical expression is variable, if present in the neonatal period leads rapidly to ESRD, but its evolution is slower, from the clinical point of view, dominated by secondary alterations to liver damage. Histologically, it presents a significant increase in kidney size (10 to 12 times greater), with multiple small cysts, cortical and spinal cord, with a radial disposition towards the medulla, suggesting that cysts are fully dilated glomeruli.

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