Abstract
Rationale: Progressive familial intrahepatic cholestasis (PFIC) is a group of genetic diseases affecting the hepatocellular transport and bile formation. In PFIC3 there is a defect in the ABCB4 gene, which leads to impaired biliary phospholipid secretion, damaged hepatocytes and bile ducts, and heterogeneous clinical presentation. Growth impairment is often described in children with cholestatic liver diseases. The aim of our study was to assess the nutritional status in patients with PFIC3.
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