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
DB93.21 — Idiopathic copper-associated cirrhosis

ICD-11 DB93.21 — Idiopathic copper-associated cirrhosis

Idiopathic copper-associated cirrhosis is a rare copper-overload liver disease characterised by a rapidly progressive liver cirrhosis from the first few years of life leading to hepatic insufficiency and harbouring a specific pathological aspect: pericellular fibrosis, inflammatory infiltration, hepatocyte necrosis, absence of steatosis, poor regeneration and histochemical copper staining.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.