ICD-11 classes
13 Diseases of the digestive system
Diseases of liver
5C90 — Metabolic or transporter liver disease
5C90.0 — Liver diseases due to urea cycle defects
5C50.A2 — Argininaemia
ICD-11 5C50.A2 — Argininaemia
Arginase deficiency is a rare autosomal recessive amino acid metabolism disorder characterised clinically by variable degrees of hyperammonemia, developing from about 3 years of age, and leading to progressive loss of developmental milestones and spasticity in the absence of treatment.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: