Combined oxidative phosphorylation defect type 21

Orpha code: 420733OMIM code: 615918

Definition

Combined oxidative phosphorylation defect type 21 is a rare mitochondrial disease characterized by axial hypotonia with limb hypertonia, developmental delay, hyperlactatemia, central nervous system anomalies visible on magnetic resonance imaging (e.g. corpus callosum hypoplasia, lesions of the globus pallidus) and multiple deficiency of the mitochondrial respiratory chain complexes in muscle tissue, but not in fibroblasts or liver.

Disease data
Classification

Disease

Synonyms
COXPD21
COXPD21
ORPHA code
420733
OMIM code
615918
ICD10 code
E88.8
ICD11 code
-

No additional description.

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