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hepatic ARPKD
MIM.263200
Sunday 9 May 2004
Synopsis
anomalies of hepatic organogenesis (hepatic dysplasia)
- ductal plate malformation (DPM)
- portal fibrosis (congenital hepatic fibrosis)
Associations
phosphomannose isomerase deficiency (10963387)
hereditary hemorrhagic telangiectasia (9500780)
References
Strayer DS, Kissane JM. Dysplasia of the kidneys, liver, and pancreas: report of a variant of Ivemark’s syndrome. Hum Pathol. 1979 Mar;10(2):228-34. PMID: 422192
