Dysferlin-related limb-girdle muscular dystrophy R2

Orpha code: 268OMIM code: 253601

Definition

A subtype of autosomal recessive limb-girdle muscular dystrophy characterized by an onset in late adolescence or early adulthood of slowly progressive, proximal weakness and atrophy of shoulder and pelvic girdle muscles. Cardiac and respiratory muscles are not involved. Hypertrophy of the calf muscles and highly elevated serum creatine kinase levels are frequently observed.

Disease data
Classification

Disease

Synonyms
Autosomal recessive limb-girdle muscular dystrophy type 2B
Dystrofia obręczowo-kończynowa z powodu niedoboru dysferliny
LGMD2B
Dysferlin-related LGMD R2
LGMD due to dysferlin deficiency
LGMD type 2B
LGMD2B
Limb-girdle muscular dystrophy due to dysferlin deficiency
Limb-girdle muscular dystrophy type 2B
ORPHA code
268
OMIM code
253601
ICD10 code
G71.0
ICD11 code
8C70.41

No additional description.

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