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Muscle biopsy also helps set apart many of the recently described autosomal ascendant and autosomal recessive forms of limb girdle 209 210 Moxley and Yurcheshen Present 1 Broad-shouldered Dystrophies in Babyhood: Complications and Treatment Limb girdle Duchenne Becker strapping Faciosc dystrophy dystrophy Myotonic dystrophy dystrophy dystrop Muscle Treatment with No controlled No speci?c treatment; No speci?c analysis; No spe fault prednisone slows or studies of prednisone braces as a replacement for foot drop; braces as regards foot pinch; patients stabilizes muscle treatment; restorative is children on the whole can children by can lifting w soundness; lightweight valuable in past due stages participate in gym in participate in gym in extende long-leg fortifying dogma school braces a maintains ambulation needed in later stages Respiratory Feigned central skill Uncommon until dilatory On the side of congenital cases, Uncommon until overdue Uncomm problems is monitored (in later stages; management then ventilary take care of often is stages; stewardship stages, atelectatic is as with Duchenne needed; the prediction throughout then is as with pneumonitis is dystrophy survival is hugely Duchenne dystrophy hackneyed); colds are modest if the dogged is treated aggressively; if ventilator dependent >4 signs of respiratory weeks; other direction failure exploit, is as after Duchenne nasal=oral ventilation dystrophy should be considered Cardiac Then cardio- Sometimes, unbending Again, tachy- Occasionally, fastidious Uncomm problems myopathy leads to cardiomyopathy develops; arrhythmias or ticker cardiomyopathy congestive heart treatment is the in spite of as prevent a rough out expand on in develops; treatment is flop—afterload- looking for Duchenne dystrophy babyhood forms, and the anyway as an eye to reducing remedy time tread maker treatment Duchenne dystrophy helps; the part of is indicated digoxin is fitful; unaggressive should be monitored for intra- cardiac clots Orthopedic Achilles tendon Uncommon; contractures Talipes deformity Uncommon; Occasio problems contractures rejoin to are much less shared requires treatment contractures are much effusion stretching in premature than in Duchenne with stretching and less banal than in ass effort dev stages, later tendon dystrophy orthotic strengthen; Duchenne dystrophy to tired unfetter surgery often is intermittently surgery conserv necessary; contractures is needed allowance at the hips, knees, in late s elbows, and wrists surgeon generally speaking enlarge on after chaste re the unswerving becomes procedu wheelchair confined; the scap scoliosis repeatedly develops uncomm when patients stop ambulating, and spinal stabilization surgery helps make a case for manipulate of the arms and preserves pulmonary hesitancy Edgy Increased occurrence of Uncommon Theoretical retardation is Uncommon Uncomm arrangement cognitive and simple, peculiarly in cases, th symptoms behavioral problems; congenital cases, and develop of some patients gain strength rare classroom care is occurs i with feel discomfited doses of needed; hearing de?cits with hea methyl-phenidate are routine and may and=or instruct hearing aids; facial leaning, dysarthria, and hearing problems overdo the brand of outlook retardation Psychoanalysis on Husky Dystrophies 211 rdle Fukuyama-type Congenital muscular ar Facioscapulohumeral congenital burly dystrophy: principal Emery–Dreifuss hy dystrophy dystrophy de?ciency of merosin brawny dystrophy ci?c therapy; No speci?c treatment; No speci?c treatment; Unchanged as Fukuyoma- No speci?c treatment; or foot drop; patients should avoid invigorating and specialist order congenital skeletal muscle usually can lifting with arms fully cure are profitable in robust atrophy weak point frequently is ate in gym in extended and abducted; some patients rather mild braces are from time to time compared with cardiac needed problems and does not limit ritual mon until late Uncommon As with Duchenne Anyhow as Fukuyoma- Lenitive other than management dystrophy; patients variety congenital symptoms related to as with again succomb to muscular atrophy cardiac dysfunction ne dystrophy respiratory failure till in adolescence or in pioneer teens nally, acute Uncommon Uncommon Uncommon Recurring cardiac myopathy conduction defects; s; treatment is atrial paralysis, cardiac e as on seize, and abrupt ne dystrophy death are prevalent; pacemaker treatment and remedy group therapy for the sake cardiac emboli often are required mon; Sporadically, knee Contractures develop Contractures, Contractures, first of all tures are much effusion and ill-bred encourage in 70% of patients not later than 3 peculiarly feet in the elbows and mmon than in suffering bring out secondary months of age at the and hips ankles, transpire cock's-crow and ne dystrophy to weakness; ankles, knees, and hips answer fairly to traditional fancy corporeal therapy; measures are effective; surgical unloose of in recent stages some achilles tendon may be surgeons have reported obligatory; some lofty results with patients broaden a exact procedures to stabilize spine syndrome, by reason of the scapula; surgery is which there is no uncommon effective analysis mon Uncommon; in rare Generalized or focal Demented retardation Apt sole to flourish from cases, the infant sally seizures arise in most plain; 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264  |  265  |  266  |  267  |  268  |  269  |  270  |  271  |  272  |  273  |  274  |  275  |  276  |  277  |  278  |  279  |  280  |  281  |  282  |  283  |  284  |  285  |  286  |  287  |  288  |  289  |  290  |  291  |  292  |  293  |  294  |  295  |  296  |  297  |  298  |  299  |  300  |  301  |  302  |  303  |  304  |  305  |  306  |  307  |  308  |  309  |  310  |  311  |  312  |  313  |  314  |  315  |  316  |  317  |  318  |  319  |  320  |  321  |  322  |  323  |  324  |  325  |  326  |  327  |  328  |  329  |  330  |  331  |  332  |  333  |  334  |  335  |  336  |  337  |  338  |  339  |  340  |  341  |  342  |  343  |  344  |  345  |  346  |  347  |  348  |  349  |  350  |  351  |  352  |  353  |  354  |  355  |  356  |  357  |  358  |  359  |  360  |  361  |  362  |  363  |  364  |  365  |  366  |  367  |  368  |  369  |  370  |  371  |  372  |  373  |  374  |  375  |  376  |  377  |  378  |  379  |  380  |  381  |  382  |  383  |  384  |  385  |  386  |  387  |  388  |  389  |  390  |  391  |  392  |  393  |  394  |  395  |  396  |  397  |  398  |  399  |  400  |  401  |  402  |  403  |  404  |  405  |  406  |  407  |  408  |  409  |  410  |  411  |  412  |  413  |  414  |  415  |  416  |  417  |  418  |  419  |  420  |  421  |  422  |  423  |  424  |  425  |  426  |  427  |  428  |  429  |  430  |  431  |  432  |  433  |  434  |  435  |  436  |  437  |  438  |  439  |  440  |  441  |  442  |  443  |  444  |  445  |  446  |  447  |  448  |  449  |  450  |  451  |  452  |  453  |  454  |  455  |  456  |  457  |  458  |  459  |  460  |  461  |  462  |  463  |  464  |  465  |  466  |  467  |  468  |  469  |  470  |  471  |  472  |  473  |  474  |  475  |  476  |  477  |  478  |  479  |  480  |  481  |  482  |  483  |  484  |  485  |  486  |  487  |  488  |  489  |  490  |  491  |  492  |  493  |  494  |  495  |  496  |  497  |  498  |  499  |  500  |  501  |  502  |  503  |  504  |  505  |  506  |  507  |  508  |  509  |  510  |  511  |  512  |  513  |  514  |  515  |  516  |  517  |  518  |  519  |  520  |  521  |  522  |  523  |  524  |  525  |  526  |  527  |  528  |  529  |  530  |  531  |  532  |  533  |  534  |  535  |  536  |  537  |  538  |  539  |  540  |  541  |  542  |  543  |  544  |  545  |  546  |  547  |  548  |  549  |  550  |  551  |  552  |  553  |  554  |  555  |  556  |  557  |  558  |  559  |  560  |  561  |  562  |  563  |  564  |  565  |  566  |  567  |  568  |  569  |  570  |  571  |  572  |  573  |  574  |  575  |  576  |  577  |  578  |  579  |  580  |  581  |  582  |  583  |  584  |  585  |  586  |  587  |  588  |  589  |  590  |  591  |  592  |  593  |  594  |  595  |  596  |  597  |  598  |  599  |  600  |  601  |  602  |  603  |  604  |  605  |  606  |  607  |  608  |  609  |  610  |  611  |  612  |  613  |  614  |  615  |  616  |  617  |  618  |  619  |  620  |  621  |  622  |  623  |  624  |  625  |  626  |  627  |  628  |  629  |  630  |  631  |  632  |  633  |  634  |  635  |  636  |  637  |  638  |  639  |  640  |  641  |  642  |  643  |  644  |  645  |  646  |  647  |  648  |  649  |  650  |  651  |  652  |  653  |  654  |  655  |  656  |  657  |  658  |  659  |  660  |  661  |  662  |  663  |  664  |  665  |  666  |  667  |  668  |  669  |  670  |  671  |  672  |  673  |  674  |  675  |  676  |  677  |  678  |  679  |  680  |  681  |  682  |  683  |  684  |  685  |  686  |  687  |  688  |  689  |  690  |  691  |  692  |  693  |  694  |  695  |  696  |  697  |  698  |  699  |  700  |  701  |  702  |  703  |  704  |  705  |  706  |  707  |  708  |  709  |  710  |  711  |  712  |  713  |  714  |  715  |  716  |  717  |  718  |  719  |  720  |  721  |  722  |  723  |  724  |  725  |  726  |  727  |  728  |  729  |  730  |  731  |  732  |  733  |  734  |  735  |  736  |  737  |  738  |  739  |  740  |  741  |  742  |  743  |  744  |  745  |  746  |  747  |  748  |  749  |  750  |  751  |  752  |  753  |  754  |  755  |  756  |  757  |  758  |  759  |  760  |  761  |  762  |  763  |  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889  |  890  |  891  |  892  |  893  |  894  |  895  |  896  |  897  |  898  |  899  |  900  |  901  |  902  |  903  |  904  |  905  |  906  |  907  |  908  |  909  |  910  |  911  |  912  |  913  |  914  |  915  |  916  |  917  |  918  |  919  |  920  |  921  |  922  |  923  |  924  |  925  |  926  |  927  |  928  |  929  |  930  |  931  |  932  |  933  |  934  |  935  |  936  |  937  |  938  |  939  |  940  |  941  |  942  |  943  |  944  |  945  |  946  |  947  |  948  |  949  |  950  |  951  |  952  |  953  |  954  |  955  |  956  |  957  |  958  |  959  |  960  |  961  |  962  |  963  |  964  |  965  |  966  |  967  |  968  |  969  |  970  |  971  |  972  |  973  |  974  |  975  |  976  |  977  |  978  |  979  |  980  |  981  |  982  |  983  |  984  |  985  |  986  |  987  |  988  |  989  |  990  |  991  |  992  |  993  |  994  |  995  |  996  |  997  |  998  |  999  |  1000  |  1001  |  1002  |  1003  |  1004  |  1005  |  1006  |  1007  |  1008  |  1009  |  1010  |  1011  |  1012 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