Congenital cataract-progressive muscular hypotonia-hearing loss-developmental delay syndrome

Orpha code: 330054OMIM code: 613076

Definition

Congenital cataract-progressive muscular hypotonia-hearing loss-developmental delay syndrome is a rare, genetic, mitochondrial myopathy disorder characterized by congenital cataract, progressive muscular hypotonia that particularly affects the lower limbs, reduced deep tendon reflexes, sensorineural hearing loss, global development delay and lactic acidosis. Muscle biopsy reveals reduced complex I, II and IV respiratory chain activity.

Disease data
Classification

Disease

Synonyms
Congenital cataract-progressive muscular hypotonia-deafness-developmental delay syndrome
Zaćma wrodzona - postępująca hipotonia mięśni - głuchota - opóźnienie rozwoju
ORPHA code
330054
OMIM code
613076
ICD10 code
G71.3
ICD11 code
-

No additional description.

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