Last update 01 Nov 2024

Renal-Hepatic-Pancreatic Dysplasia

Basic Info

Synonyms
Ivemark II syndrome, RENAL-HEPATIC-PANCREATIC DYSPLASIA, Renal hepatic pancreatic dysplasia
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Introduction
A rare genetic developmental defect during embryogenesis syndrome with 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.

Analysis

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