View source for Medicine:Renal-hepatic-pancreatic dysplasia

{{Infobox medical condition (new)
| synonyms        = Ivemark II syndrome,Renohepaticopancreatic dysplasia
| name            = Renal-hepatic-pancreatic dysplasia
| image           = Ivemark.jpg
| caption         = Gross photo of liver and pancreas showing multiple cysts in the latter in a patient with renal-hepatic-pancreatic dysplasia.
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'''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 [[Biology:NPHP3|NPHP3]] has been described.<ref name="pmid18371931">{{cite journal  |vauthors=Bergmann C, Fliegauf M, Brüchle NO, etal |title=Loss of nephrocystin-3 function can cause embryonic lethality, Meckel-Gruber-like syndrome, situs inversus, and renal-hepatic-pancreatic dysplasia |journal=Am. J. Hum. Genet. |volume=82 |issue=4 |pages=959–970 |date=April 2008 |pmid=18371931 |doi=10.1016/j.ajhg.2008.02.017 |url=http://linkinghub.elsevier.com/retrieve/pii/S0002-9297(08)00209-7 |pmc=2427297}}</ref>

It was characterized in 1959.<ref name="pmid17605805">{{cite journal |vauthors=Vankalakunti M, Gupta K, Kakkar N, Das A |title=Renal-hepatic-pancreatic dysplasia syndrome (Ivemark's syndrome) |journal=Diagn Pathol |volume=2 |issue= |pages=24 |year=2007 |pmid=17605805 |pmc=1919354 |doi=10.1186/1746-1596-2-24 |url=http://www.diagnosticpathology.org/content/2//24}}</ref><ref name="pmid13626573">{{cite journal |vauthors=IVEMARK BI, OLDFELT V, ZETTERSTROM R |title=Familial dysplasia of kidneys, liver and pancreas: a probably genetically determined syndrome |journal=Acta Paediatr |volume=48 |issue=1 |pages=1–11 |date=January 1959 |pmid=13626573 |doi= 10.1111/j.1651-2227.1959.tb16011.x|url=}}</ref>


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[[Category:Congenital disorders]]
[[Category:Autosomal recessive disorders]]
[[Category:Rare diseases]]



{{Sourceattribution|Renal-hepatic-pancreatic dysplasia|1}}