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https://observatorio.fm.usp.br/handle/OPI/8997
Title: | Clinical aspects of patients with sarcoglycanopathies under steroids therapy |
Authors: | ALBUQUERQUE, Marco A. V.; ABATH-NETO, Osorio; MAXIMINO, Jessica R.; CHADI, Gerson; ZANOTELI, Edmar; REED, Umbertina C. |
Citation: | ARQUIVOS DE NEURO-PSIQUIATRIA, v.72, n.10, p.768-772, 2014 |
Abstract: | Patients with sarcoglycanopathies, which comprise four subtypes of autosomal recessive limb-girdle muscular dystrophies, usually present with progressive weakness leading to early loss of ambulation and premature death, and no effective treatment is currently available. Objective: To present clinical aspects and outcomes of six children with sarcoglycanopathies treated with steroids for at least one year. Method: Patient files were retrospectively analyzed for steroid use. Results: Stabilization of muscle strength was noted in one patient, a slight improvement in two, and a slight worsening in three. In addition, variable responses of forced vital capacity and cardiac function were observed. Conclusions: No overt clinical improvement was observed in patients with sarcoglycanopathies under steroid therapy. Prospective controlled studies including a larger number of patients are necessary to determine the effects of steroids for sarcoglycanopathies. |
Appears in Collections: | Artigos e Materiais de Revistas Científicas - FM/MNE Artigos e Materiais de Revistas Científicas - HC/ICHC Artigos e Materiais de Revistas Científicas - LIM/15 Artigos e Materiais de Revistas Científicas - LIM/45 |
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art_ALBUQUERQUE_Clinical_aspects_of_patients_with_sarcoglycanopathies_under_steroids_2014.PDF | publishedVersion (English) | 81.67 kB | Adobe PDF | View/Open |
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