North American Indian childhood cirrhosis (NAIC) is a disease in humans that can affect Ojibway-Cree children in northwestern Quebec, Canada.
[1] The disease is due to an autosomal recessive abnormality of the UTP4 gene, which codes for cirhin,[2] a nucleolar protein.
[4][5] An R565W mutation of UTP4[2][6] leads to partial impairment of cirhin interaction with NOL11.
[6] Initial transient neonatal jaundice advances over time to biliary cirrhosis with severe liver fibrosis.
[1] Eventually, liver failure occurs, and requires liver transplantation.