Duchenne's muscular dystrophy

Duchenne's muscular dystrophy

Etymology: Guillaume B.A. Duchenne

an abnormal congenital condition characterized by progressive symmetric wasting of the leg and pelvic muscles. This disease predominantly affects males and accounts for 50% of all muscular dystrophy diseases. It is an X-linked recessive disease that appears insidiously between 3 and 5 years of age and spreads from the leg and pelvic muscles to the involuntary muscles. Associated muscle weakness produces a waddling gait and pronounced lordosis. Muscles rapidly deteriorate, and calf muscles become firm and enlarged as a result of fatty deposits. Affected children experience contractures, have difficulty climbing stairs, often stumble and fall, and display winged scapulae when they raise their arms. Such persons are usually confined to wheelchairs by 12 years of age, and progressive weakening of cardiac muscle causes tachycardia and pulmonary problems. The patients affected may also have cardiac murmurs, faint heart sounds, and chest pain and may suffer arrhythmias or infections that produce overt heart failure. Such complications, especially in the later stages of this disease, can cause sudden death. Duchenne's muscular dystrophy usually causes death within 10 to 15 years of symptom onset. There is no successful treatment of the disease. Orthopedic appliances, exercise, physical therapy, and surgery to correct contractures can help preserve mobility. Nursing care involves psychologic support of the patient and family and encouragement of the patient to prevent long periods of bed rest and inactivity and ensure maximum physical activity. Splints, braces, grab bars, and overhead slings help the patient exercise. A wheelchair helps preserve mobility. Other devices that can increase comfort and help prevent footdrop include footboards, high-topped sneakers, and foot cradles. Also called pseudohypertrophic muscular dystrophy.

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Duchenne's muscular dystrophy,

n an X-linked recessive condition pres-ent at birth in which the muscles of the pelvis and legs waste away in a symmetric fashion. Appears in children between three to five years of age, with death occurring 10 to 15 years after the onset of symtoms. Also called
pseudohypertrophic muscular dystrophy.

Duchenne's muscular dystrophy.