Journal article
Clinical aspects of patients with sarcoglycanopathies under steroids therapy
Arquivos de neuro-psiquiatria, Vol.72(10), pp.768-772
10/2014
DOI: 10.1590/0004-282X20140126
PMID: 25337728
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.
To present clinical aspects and outcomes of six children with sarcoglycanopathies treated with steroids for at least one year.
Patient files were retrospectively analyzed for steroid use.
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.
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.
Details
- Title: Subtitle
- Clinical aspects of patients with sarcoglycanopathies under steroids therapy
- Creators
- Marco A V Albuquerque - Universidade de São PauloOsório Abath-Neto - Universidade de São PauloJéssica R Maximino - Universidade de São PauloGerson Chadi - Universidade de São PauloEdmar Zanoteli - Universidade de São PauloUmbertina C Reed - Universidade de São Paulo
- Resource Type
- Journal article
- Publication Details
- Arquivos de neuro-psiquiatria, Vol.72(10), pp.768-772
- DOI
- 10.1590/0004-282X20140126
- PMID
- 25337728
- NLM abbreviation
- Arq Neuropsiquiatr
- ISSN
- 0004-282X
- eISSN
- 1678-4227
- Language
- English
- Date published
- 10/2014
- Academic Unit
- Pathology
- Record Identifier
- 9984277264302771
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