TMEM70-related mitochondrial encephalo-cardio-myopathy

Orpha code: 1194OMIM code: 614052

Definition

Mitochondrial encephalo-cardio-myopathy due to <i>TMEM70</i> mutation is characterized by early neonatal onset of hypotonia, hypetrophic cardiomyopathy and apneic spells within hours after birth accompanied by lactic acidosis, hyperammonemia and 3-methylglutaconic aciduria.

Disease data
Classification

Disease

Synonyms
Mitochondrial encephalo-cardio-myopathy due to F1Fo ATPase deficiency
Mitochondrialna encefalo-kardio-miopatia z powodu niedoboru F1Fo ATP-azy
Mitochondrialna encefalo-kardio-miopatia z powodu niedoboru kompleksu V mitochondrialnego łańcucha oddechowego
Mitochondrialna encefalo-kardio-miopatia z powodu niedoboru syntazy ATP
Mitochondrial encephalo-cardio-myopathy due to isolated ATP synthase deficiency
Mitochondrial encephalo-cardio-myopathy due to isolated mitochondrial respiratory chain complex V deficiency
ORPHA code
1194
OMIM code
614052
ICD10 code
G71.3
ICD11 code
-

No additional description.

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