Clinical aspects of patients with sarcoglycanopathies under steroids therapy.
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.
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