05Endocrine, nutritional or metabolic diseases
Metabolic disorders
Inborn errors of metabolism
5C53Inborn errors of energy metabolism
5C53.0Disorders of pyruvate metabolism
5C53.03
category
Pyruvate carboxylase deficiency
Definition 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).
Also indexed as
Ataxia with lactic acidosis type II Pyruvate carboxylase deficiency
Nearby in Disorders of pyruvate metabolism
International Classification of Diseases, Eleventh Revision (ICD-11), World Health Organization (WHO) 2019/2021, https://icd.who.int/browse11. Licensed under Creative Commons Attribution-NoDerivatives 3.0 IGO (CC BY-ND 3.0 IGO).
Release 2026-01.
View this entity in the official WHO ICD-11 browser .
This page reproduces WHO classification content for reference. It is not
medical advice and must not be used to diagnose or treat. For coding
decisions, consult the official release and your national coding guidelines.
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