Wednesday 29 June 2005
Definition: Renohepatopancreatic dysplasia (RHPD) is caracterized by the association of several visceral dysplasia involving the kidney (bilateral non-obstructive multicystic renal dysplasia or BNOMRD) , the liver (ductal plate malformation or DPM; previously congenital hepatic fibrosis) and the pancreas (pancreatic acinar dysplasia or PAD).
bilateral non-obstructive multicystic renal dysplasia or BNOMRD
hepatic ductal plate malformation (DPM) (ex-congenital hepatic fibrosis)
pancreatic acinar dysplasia (PAD)
Etiology (renohepatopancreatic dysplastic syndromes)
- Jeune syndrome
- Saldino-Noonan syndrome
- Verma-Neumoff syndrome
- Ellis-van Creveld syndrome
multivisceral dysplastic syndromes
- renopancreatic dysplastic syndromes
- renohepatic dysplastic syndromes
- hepatopancreatic dysplastic syndromes
Ivemark, B. I.; Oldfelt, V.; Zetterstrom, R. :
Familial dysplasia of kidneys, liver and pancreas: a probably genetically determined syndrome. Acta Paediat. Scand. 48: 1-11, 1959.