Duchenne muscular dystrophy

Duchenne muscular dystrophy

a specific form of muscular dystrophy that is inherited as a sex-linked recessive trait and thus confined to young males and to females with turners syndrome. One third of all cases are estimated to be new mutational events.

See: dystrophin.

It is characterised by degeneration and necrosis of skeletal muscle fibres, that are replaced by fat and fibrous tissue.

symptoms include muscle weakness and in some forms, the appearance of muscle enlargement (pseudo-hypertrophy). Advanced cases can include weakness of the respiratory muscles (compromising breathing) and cardiomyopathy.

Inheritance: sex-linked recessive.

Incidence: 1 in 4000 male births.

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