syndromic visceral malformation renal-hepatic-pancreatic dysplasia DOID:0060259 Ivemark II syndrome Ivemark's syndrome MONDO:0017417 OMIMPS:208540 Orphanet:294415 Renal-hepatic-pancreatic dysplasia is 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 tendancy 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. Renohepaticopancreatic dysplasia SCTID:763891005 UMLS:C2673883 familial cystic renal disease