Renal-hepatic-pancreatic dysplasia

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 NPHP3 as been described.cite journal |author=Bergmann C, Fliegauf M, Brüchle NO, "et al" |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 |year=2008 |month=April |pmid=18371931 |doi=10.1016/j.ajhg.2008.02.017 |url=]

It was characterized in 1959.cite journal |author=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=] cite journal |author=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 |year=1959 |month=January |pmid=13626573 |doi= |url=]


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