Ivemark II Syndrome (renal-hepatic-pancreatic dysplasia) is a rare genetic disorder.

Implicated gene: DNAJB11 (DnaJ Heat Shock Protein Family Member B11)


Inheritance: autosomal dominant



(1) pancreatic fibrosis and/or cysts and/or pancreatitis

(2) renal dysplasia with peripheral cortical cysts, primitive collecting ducts, glomerular cysts, and metaplastic cartilage

(3) hepatic dysgenesis with enlarged portal areas and periportal fibrosis

(4) variable situs abnormalities

(5) variable skeletal anomalies

(6) variable anencephaly



(1) renal insufficiency

(2) jaundice

(3) insulin-dependent diabetes

(4) heart failure

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