Isolated neonatal sclerosing cholangitis

Isolated neonatal sclerosing cholangitis is a rare, genetic, biliary tract disease characterized by severe neonatal-onset cholangiopathy with patent bile ducts and absence of ichthyosiform skin lesions. Patients present with jaundice, acholic stools, hepatosplenomegaly and high serum gamma-glutamyltransferase activity. Liver histology shows portal fibrosis, ductular proliferation, hepatocellular metallothionein deposits, and intralobular bile-pigment accumulations. Some patients may also have renal disease.

Also known as: NSC, sclerosing cholangitis, neonatal

3 clinical trials for this condition and its sub-types, 0 tagged with Isolated neonatal sclerosing cholangitis itself.