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Clinical aspects of patients with sarcoglycanopathies under steroids therapy.
Albuquerque, Marco A V; Abath-Neto, Osório; Maximino, Jéssica R; Chadi, Gerson; Zanoteli, Edmar; Reed, Umbertina C.
Afiliação
  • Albuquerque MA; Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
  • Abath-Neto O; Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
  • Maximino JR; Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
  • Chadi G; Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
  • Zanoteli E; Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
  • Reed UC; Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
Arq Neuropsiquiatr ; 72(10): 768-72, 2014 Oct.
Article em En | MEDLINE | ID: mdl-25337728
UNLABELLED: 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.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Pregnenodionas / Prednisolona / Sarcoglicanopatias / Glucocorticoides Tipo de estudo: Observational_studies Limite: Child / Female / Humans / Male Idioma: En Revista: Arq Neuropsiquiatr Ano de publicação: 2014 Tipo de documento: Article País de afiliação: Brasil País de publicação: Alemanha

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Pregnenodionas / Prednisolona / Sarcoglicanopatias / Glucocorticoides Tipo de estudo: Observational_studies Limite: Child / Female / Humans / Male Idioma: En Revista: Arq Neuropsiquiatr Ano de publicação: 2014 Tipo de documento: Article País de afiliação: Brasil País de publicação: Alemanha