ICD-11 classes
05 Endocrine, nutritional or metabolic diseases
Metabolic disorders
Inborn errors of metabolism
5C53 — Inborn errors of energy metabolism
5C53.0 — Disorders of pyruvate metabolism
5C53.03 — Pyruvate carboxylase deficiency

ICD-11 5C53.03 — Pyruvate carboxylase deficiency

This is a deficiency in the enzyme of the ligase class that catalyzes the (depending on the species) irreversible carboxylation of pyruvate to form oxaloacetate (OAA).

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.