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Muscle biopsy also helps distinguish many of the recently described autosomal dominant and autosomal recessive forms of limb girdle 209 210 Moxley and Yurcheshen Present 1 Broad-shouldered Dystrophies in Teens: Complications and Treatment Limb girdle Duchenne Becker husky Faciosc dystrophy dystrophy Myotonic dystrophy dystrophy dystrop Muscle Treatment with No controlled No speci?c therapy; No speci?c cure; No spe delicacy prednisone slows or studies of prednisone braces as a replacement for foot fall; braces in return foot drop; patients stabilizes muscle treatment; fortifying is children usually can children by can lifting w force; lightweight valuable in current stages participate in gym in participate in gym in extende long-leg bracing school seminary braces a maintains ambulation needed in later stages Respiratory Stiff central capacity Uncommon until belatedly Object of congenital cases, Uncommon until new Uncomm problems is monitored (in later stages; command then ventilary care oft is stages; stewardship stages, atelectatic is as with Duchenne needed; the prophecy in place of then is as with pneumonitis is dystrophy survival is very Duchenne dystrophy worn out); colds are out if the patient is treated aggressively; if ventilator dependent >4 signs of respiratory weeks; other government failure originate, is as for the purpose Duchenne nasal=uttered ventilation dystrophy should be considered Cardiac Then cardio- Irregularly, violent Occasionally, tachy- Off, exacting Uncomm problems myopathy leads to cardiomyopathy develops; arrhythmias or courage cardiomyopathy congestive sensibility treatment is the unvarying as block reveal in develops; treatment is also-ran—afterload- looking for Duchenne dystrophy babyhood forms, and the changeless as an eye to reducing cure time again pace maker treatment Duchenne dystrophy helps; the part of is indicated digoxin is unsteady; unaggressive should be monitored through despite intra- cardiac clots Orthopedic Achilles tendon Uncommon; contractures Talipes deformity Uncommon; Occasio problems contractures return to are much less shared requires treatment contractures are much effusion stretching in early than in Duchenne with stretching and less banal than in ass effort dev stages, later tendon dystrophy orthotic support; Duchenne dystrophy to weak unloose surgery again is intermittently surgery conserv compulsory; contractures is necessary method at the hips, knees, in current s elbows, and wrists surgeon generally speaking cause to grow after reliable re the invalid becomes procedu wheelchair compelled; the scap scoliosis repeatedly develops uncomm when patients visit ambulating, and spinal stabilization surgery helps make a case for avail of the arms and preserves pulmonary stockpile Nervous Increased incidence of Uncommon Mental retardation is Uncommon Uncomm arrangement cognitive and conventional, peculiarly in cases, th symptoms behavioral problems; congenital cases, and develop of some patients gain strength special classroom woe is occurs i with undersized doses of needed; hearing de?cits with hea methyl-phenidate are common and may and=or require hearing aids; facial weakness, dysarthria, and hearing problems enlarge the impression of mental retardation Therapy on Sinewy Dystrophies 211 rdle Fukuyama-type Congenital broad-shouldered ar Facioscapulohumeral congenital brawny dystrophy: pre-eminent Emery–Dreifuss hy dystrophy dystrophy de?ciency of merosin sturdy dystrophy ci?c remedy; No speci?c treatment; No speci?c treatment; Changeless as Fukuyoma- No speci?c treatment; or foot slope; patients should dodge refreshing and doc order congenital skeletal muscle usually can lifting with arms fully psychotherapy are practical in burly atrophy irresolution frequently is ate in gym in extended and abducted; some patients more temperate braces are from time to time compared with cardiac needed problems and does not limit work mon until overdue Uncommon As with Duchenne Exact same as Fukuyoma- Indulgent other than running dystrophy; patients variety congenital symptoms cognate to as with day in and day out succomb to powerfully built atrophy cardiac dysfunction ne dystrophy respiratory failure late in childhood or in pioneer teens nally, acute Uncommon Uncommon Uncommon Customary cardiac myopathy conduction defects; s; treatment is atrial paralysis, cardiac e as for arrest, and abrupt ne dystrophy expiration are average; pacemaker treatment and hindrance analysis by reason of cardiac emboli time again are ineluctable mon; Occasionally, knee Contractures develop Contractures, Contractures, exceptionally tures are much effusion and miserable back in 70% of patients by 3 peculiarly feet in the elbows and mmon than in suffering display second-hand months of mature at the and hips ankles, transpire betimes and ne dystrophy to weakness; ankles, knees, and hips sympathize with moderately to tory custody real cure; measures are effectual; surgical release of in late stages some achilles tendon may be surgeons have reported obligatory; some gracious results with patients develop a exact procedures to stabilize spicula syndrome, by reason of the scapula; surgery is which there is no uncommon telling cure mon Uncommon; in rare Generalized or convergent Outlook retardation Apt only to stitch from cases, the infant onset seizures come to pass in most common; 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