renal-hepatic-pancreatic dysplasia

A physical disorder characterized by pancreatic fibrosis, renal dysplasia and hepatic dysgenesis; it is usual fatal soon after birth.

Signs and symptoms

• Stage 5 chronic kidney disease
• Renal insufficiency
• Cirrhosis
• Hyperechogenic pancreas
• Femoral bowing
• Enlarged cisterna magna
• Malformation of the hepatic ductal plate
• Oligohydramnios
• Cystic renal dysplasia
• Polycystic kidney dysplasia

Also known as: Ivemark's syndrome