| Renal–hepatic–pancreatic dysplasia | |
|---|---|
| Other names | Ivemark II syndrome, Renohepaticopancreatic dysplasia |
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| Gross 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. It is usually fatal soon after birth.An association with NPHP3 has been described. It was characterized in 1959.
