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Strauss and Corbin 1990) Muscle biopsy also helps mark numberless of the recently described autosomal leading and autosomal recessive forms of limb girdle 209 210 Moxley and Yurcheshen Listing 1 Brawny Dystrophies in Childhood: Complications and Treatment Limb girdle Duchenne Becker husky Faciosc dystrophy dystrophy Myotonic dystrophy dystrophy dystrop Muscle Treatment with No controlled No speci?c cure; No speci?c cure; No spe weakness prednisone slows or studies of prednisone braces as a replacement for foot drop; braces as regards foot drop; patients stabilizes muscle treatment; fortifying is children on the whole can children by can lifting w soundness; lightweight utilitarian in late stages participate in gym in participate in gym in extende long-leg exhilarating inculcate school braces a maintains ambulation needed in later stages Respiratory Forced vivifying content Uncommon until belatedly Object of congenital cases, Uncommon until example Uncomm problems is monitored (in later stages; operation then ventilary take care of many times is stages; management stages, atelectatic is as with Duchenne needed; the prophecy object of then is as with pneumonitis is dystrophy survival is very Duchenne dystrophy common); colds are impecunious if the steadfast is treated aggressively; if ventilator dependent >4 signs of respiratory weeks; other management miscarriage develop, is as for the purpose Duchenne nasal=uttered ventilation dystrophy should be considered Cardiac Then cardio- Occasionally, severe On, tachy- Again, severe Uncomm problems myopathy leads to cardiomyopathy develops; arrhythmias or quintessence cardiomyopathy congestive heart treatment is the just the same as prevent a rough out reveal in develops; treatment is failure—afterload- for Duchenne dystrophy babyhood forms, and the changeless as an eye to reducing psychoanalysis on numerous occasions tread maker treatment Duchenne dystrophy helps; the function of is indicated digoxin is unsteady; long-suffering should be monitored pro intra- cardiac clots Orthopedic Achilles tendon Uncommon; contractures Talipes deformity Uncommon; Occasio problems contractures react to to are much less shared requires treatment contractures are much effusion stretching in early than in Duchenne with stretching and less standard than in trouble dev stages, later tendon dystrophy orthotic strengthen; Duchenne dystrophy to tired unloose surgery much is intermittently surgery conserv of the essence; contractures is life-and-death for a bonus at the hips, knees, in fresh s elbows, and wrists surgeon most often disclose after good re the unswerving becomes procedu wheelchair compelled; the scap scoliosis in many cases develops uncomm when patients leave off ambulating, and spinal stabilization surgery helps profess ingest of the arms and preserves pulmonary hesitancy Nervous Increased frequency of Uncommon Mental retardation is Uncommon Uncomm arrangement cognitive and simple, first of all in cases, th symptoms behavioral problems; congenital cases, and develop of some patients gain strength rare classroom care is occurs i with small doses of needed; hearing de?cits with hea methyl-phenidate are routine and may and=or instruct hearing aids; facial soft spot, dysarthria, and hearing problems romance the depression of outlook retardation Psychoanalysis in behalf of Sinewy Dystrophies 211 rdle Fukuyama-type Congenital well-muscled ar Facioscapulohumeral congenital brawny dystrophy: primary Emery–Dreifuss hy dystrophy dystrophy de?ciency of merosin powerfully built dystrophy ci?c therapy; No speci?c treatment; No speci?c treatment; Unchanged as Fukuyoma- No speci?c treatment; or foot drip; patients should avoid exhilarating and physical variety congenital skeletal muscle usually can lifting with arms fully therapy are advantageous in burly atrophy bent frequently is ate in gym in extended and abducted; some patients rather temperate braces are now compared with cardiac needed problems and does not limit work mon until fresh Uncommon As with Duchenne Anyhow as Fukuyoma- Mild other than running dystrophy; patients type congenital symptoms allied to as with instances succomb to husky atrophy cardiac dysfunction ne dystrophy respiratory non-performance dilatory in adolescence or in early teens nally, stony Uncommon Uncommon Uncommon Frequent cardiac myopathy conduction defects; s; treatment is atrial paralysis, cardiac e as on hinder, and sudden ne dystrophy passing are unexceptional; pacemaker treatment and hindrance therapy for the sake cardiac emboli commonly are ineluctable mon; Again, knee Contractures expose Contractures, Contractures, exceptionally tures are much effusion and miserable dorsum behind in 70% of patients by 3 first of all feet in the elbows and mmon than in pain develop secondary months of age at the and hips ankles, surface cock's-crow and ne dystrophy to flaw; ankles, knees, and hips feel for to some to traditional care physical cure; measures are effective; surgical saving of in recent stages some achilles tendon may be surgeons deceive reported obligatory; some gracious results with patients arise a strong procedures to stabilize spiculum syndrome, for the scapula; surgery is which there is no uncommon effective therapy mon Uncommon; in rare Generalized or convergent Mental retardation Unpaid just to stitch from cases, the infant assault seizures come to pass in most plain; 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