Renal-hepatic-pancreatic dysplasia

A rare, genetic, developmental defect during embryogenesis syndrome characterized by the triad of pancreatic fibrosis (and cysts, with a reduction of parenchymal tissue), renal dysplasia (with peripheral cortical cysts, primitive collecting ducts, glomerular cysts and metaplastic cartilage) and hepatic dysgenesis (enlarged portal areas containing numerous elongated binary profiles with a tendency to perilobular fibrosis). Situs abnormalities, skeletal anomalies and anencephaly have also been associated. Patients that survive the neonatal period present renal insufficiency, chronic jaundice and insulin-dependant diabetes.

Also known as: Ivemark II syndrome, Renohepaticopancreatic dysplasia

0 clinical trials for this condition and its sub-types, 0 tagged with Renal-hepatic-pancreatic dysplasia itself.