ICD-11 classes
13 Diseases of the digestive system
Diseases of liver
DB93 — Hepatic fibrosis or cirrhosis
DB93.2 — Certain specified fibrosis or cirrhosis of liver
DB93.20 — Hereditary North American Indian childhood cirrhosis

ICD-11 DB93.20 — Hereditary North American Indian childhood cirrhosis

Hereditary North American Indian childhood cirrhosis is a severe autosomal recessive intrahepatic cholestasis that has only been described in aboriginal children from northwestern Quebec. Manifesting first as transient neonatal jaundice, the disease evolves into periportal fibrosis and cirrhosis during a period ranging from childhood to adolescence.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.