ICD-11 classes
05 Endocrine, nutritional or metabolic diseases
Metabolic disorders
Inborn errors of metabolism
5C53 — Inborn errors of energy metabolism
5C53.2 — Disorders of mitochondrial oxidative phosphorylation

ICD-11 5C53.2 — Disorders of mitochondrial oxidative phosphorylation

An inborn error of metabolism in cellular respiration (oxidative phosphorylation) in the mitochondria, where a series of enzymes catalyze the transfer of electrons to molecular oxygen and the generation of energy-storing ATP

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 5C53.2 contains 9 clarifying diagnoses:

1. 5C53.20 — Mitochondrial DNA depletion syndromes
2. 5C53.21 — Multiple mitochondrial DNA deletion syndromes
3. 5C53.22 — Coenzyme Q10 deficiency
4. 5C53.23 — Mitochondrial protein translation defects
5. 5C53.24 — Leigh syndrome
6. 5C53.25 — Isolated ATP synthase deficiency
7. 8C73.1 — Neuropathy, ataxia, and retinitis pigmentosa
8. 5C53.2Y — Other specified disorders of mitochondrial oxidative phosphorylation
9. 5C53.2Z — Disorders of mitochondrial oxidative phosphorylation, unspecified