Renal–hepatic–pancreatic dysplasia

Renal–hepatic–pancreatic dysplasia
Other namesIvemark II syndrome, Renohepaticopancreatic dysplasia
Photo of liver and pancreas showing multiple cysts in the latter in a patient with renal–hepatic–pancreatic dysplasia

Renal–hepatic–pancreatic dysplasia is an autosomal recessive congenital disorder characterized by pancreatic fibrosis, renal dysplasia and hepatic dysgenesis. An association with NPHP3 has been described. It was characterized in 1959.

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