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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dystrophic hamster has been regarded as the useful model animal for Severe childhood autosomal recessive muscular dystrophy (SCARMD). Although, many studies on Dystrophic hamster have utilized the muscular tissue of the trunk, however no study have been analyzed for the masticatory muscle. For this study, we used a Dystrophic hamster (UM-X7.1 Syrian hamster) to histochemically investigate the effect of muscular dystrophy on the masseter muscle. Large and small regenerated muscle fibers, and necrotic fibers were detected almost in all areas. Opaque fiber, hypertrophic fiber with fiber splitting structure and necrotic fiber filled up by mononuclear phagocytes were recognized. The region, in which the mononuclear phagocytic cells infiltrated, showed strong positivity to acid phosphatase, and lysosome enzyme. There were many muscle fibers with reduced levels of succinate dehydrogenase (SDH) activities in the muscle fiber. Some TUNEL-positive cells were confirmed in both necrotic and non-necrotic areas. It was suggested that a part of TUNEL-positive cells are the cells originated from the connective tissue or immunocytes. In this result, histopathologic changes of the masseter muscle of the UM-X7.1 Syrian hamster was similar to muscle of the body trunk in the past reports. As the result, it was suggested that jaw closing movements may be negatively affected caused by the decline of the masseter muscle twitch. And, the point of view by which apoptosis is the trigger for the muscle fiber collapse were not seen in the Dystrophic hamster masseter muscle. We suggest that apoptosis is a one step in the process of regeneration of muscle fibers.
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PMID:Histopathologic features of masseter muscle in the distrophic hamster (UM-X7.1 Syrian hamster). 1155 88

Severe childhood autosomal recessive muscular dystrophy (SCARMD) is characterized by a severe Duchene muscular dystrophy like phenotype. Most such cases represent alpha or gamma sarcoglycanopathies. Mental subnormality is very uncommon and other central nervous system deficits have not been documented in patients with SCARMD. We report a brother and sister with the SCARMD phenotype, who additionally had static mental subnormality and choreiform movements. Work-up for sarcolgycan genes, dystrophin gene and known causes of mental retardation and chorea was normal.
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PMID:Severe childhood autosomal recessive muscular dystrophy, mental subnormality and chorea. 1711 66

Detailed analysis of muscle biopsy using histological and enzyme histochemical staining techniques forms the basis of diagnosis of muscular dystrophies, while clinical features and family history are important adjuncts in categorising the type of dystrophy. However, in a significant proportion of cases having overlapping clinical and histological features, it is not possible to provide accurate diagnosis. These conditions can be grouped as Limb girdle muscular dystrophy (LGMD), Decker muscular dystrophy (BMD), early onset Duchenne muscular dystrophy (DMD), Congenital muscular dystrophy (CMD), Severe childhood autosomal recessive muscular dystrophy (SCARMD), and SCARMD in girls/manifesting DMD carriers. Immunohistochemical staining procedures demonstrating the presence/absence of dystrophin, adhalin and merosin are found to be of immense value in arriving at a conclusive opinion specifying the type of muscular dystrophy. It is also evident that muscular dystrophy in young girls resembling DMD is not uncommon and that these are mostly cases of SCARMD in girls having adhalinopathy. In addition, a significant proportion of patients (9 in the present series) with clinical and histopathological diagnosis of DMD are likely to be cases of SCARMD in boys after immunohistochemical study of muscle biopsies.
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PMID:Diagnosis of muscular dystrophies : the changing concepts. 2950 74