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renohepatopancreatic dysplasia

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).

Synopsis

- 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)

- autosomal recessive polycystic kidney diseases (ARPKD)
- Meckel syndrome (MKS)
- Bardet-Biedl syndrome (BBS)
- RHPD with osseous anomalies (narrow chest, short limbs)

  • Jeune syndrome
  • Saldino-Noonan syndrome
  • Verma-Neumoff syndrome
  • Ellis-van Creveld syndrome

See also

- multivisceral dysplastic syndromes

  • renopancreatic dysplastic syndromes
  • renohepatic dysplastic syndromes
  • hepatopancreatic dysplastic syndromes

References

- 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.