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POMGNT2-related limb-girdle muscular dystrophy R24

ORPHA:565899· ICD-10 G71.0

Definition

A rare autosomal recessive limb-girdle muscular dystrophy characterized by infantile to adolescent onset of a milder form of limb-girdle muscular dystrophy with or without intellectual disability. Patients present variable proximal limb muscular weakness with calf hypertrophy and elevated serum creatine kinase.

Prevalence
<1 / 1 000 000
Age of onset
Adolescent, Infancy