Fatal mitochondrial disease due to combined oxidative phosphorylation defect type 3

Orpha code: 168566OMIM code: 610505

Definition

Combined oxidative phosphorylation deficiency type 3 is an extremely rare clinically heterogenous disorder described in about 5 patients to date. Clinical signs included hypotonia, lactic acidosis, and hepatic insufficiency, with progressive encephalomyopathy or hypertrophic cardiomyopathy.

Disease data
Classification

Disease

Synonyms
Fatal mitochondrial disease due to COXPD3
Śmiertelna choroba mitochondrialna spowodowana COXPD3
ORPHA code
168566
OMIM code
610505
ICD10 code
E88.8
ICD11 code
5C53.23

No additional description.

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