Clinical aspects of patients with sarcoglycanopathies under steroids therapy

Imagem de Miniatura
Arquivos
art_ALBUQUERQUE_Clinical_aspects_of_patients_with_sarcoglycanopathies_under_steroids_2014.PDF (81.67 KB)
Citações na Scopus
8
Tipo de produção
article
Data de publicação
2014
DOI
SciELO
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
ASSOC ARQUIVOS NEURO- PSIQUIATRIA
Autores
Citação
ARQUIVOS DE NEURO-PSIQUIATRIA, v.72, n.10, p.768-772, 2014
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
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.
Palavras-chave
steroids, limb-girdle muscular dystrophy, sarcoglycan proteins, myopathy
URI
https://observatorio.fm.usp.br/handle/OPI/8997
Referências
  1. Angelini C, 2007, MUSCLE NERVE, V36, P424, DOI 10.1002/mus.20812
  2. Angelini C, 1998, MUSCLE NERVE, V21, P769, DOI 10.1002/(SICI)1097-4598(199806)21:6<769::AID-MUS9>3.0.CO;2-5
  3. Bauer R, 2008, CARDIOVASC RES, V79, P652, DOI 10.1093/cvr/cvn131
  4. Bushby Kate, 2009, Pract Neurol, V9, P314, DOI 10.1136/jnnp.2009.193938
  5. Connolly AM, 1998, MUSCLE NERVE, V21, P1549
  6. Crosbie RH, 2000, HUM MOL GENET, V9, P2019, DOI 10.1093/hmg/9.13.2019
  7. Darin N, 2007, EUR J PAEDIATR NEURO, V11, P353, DOI 10.1016/j.ejpn.2007.02.018
  8. da Silva EC, 2012, ARQ NEURO-PSIQUIAT, V70, P191, DOI 10.1590/S0004-282X2012000300007
  9. Fisher I, 2005, FASEB J, V19, P834, DOI 10.1096/fj.04-2511fje
  10. Godfrey C, 2006, ANN NEUROL, V60, P603, DOI 10.1002/ana.21006
  11. Henricson EK, 2013, MUSCLE NERVE, V48, P55, DOI 10.1002/mus.23808
  12. Holt KH, 1998, J BIOL CHEM, V273, P34667, DOI 10.1074/jbc.273.52.34667
  13. Kirschner Janbernd, 2011, Handb Clin Neurol, V101, P41, DOI 10.1016/B978-0-08-045031-5.00003-7
  14. Lebel DE, 2013, J BONE JOINT SURG AM, V95A, P1057, DOI 10.2106/JBJS.L.01577
  15. Machado Darlene L, 2012, BMC Res Notes, V5, P435, DOI 10.1186/1756-0500-5-435
  16. Manzur AY, 2008, COCHRANE DB SYST REV, V1, DOI 10.1002/14651858.CD14003725.PUB14651853
  17. Melacini P, 1999, MUSCLE NERVE, V22, P473, DOI 10.1002/(SICI)1097-4598(199904)22:4<473::AID-MUS8>3.0.CO;2-5
  18. Nigro V, 2011, CURR OPIN NEUROL, V24, P429, DOI 10.1097/WCO.0b013e32834aa38d
  19. Parreira SLS, 2010, ARQ NEURO-PSIQUIAT, V68, P683, DOI 10.1590/S0004-282X2010000500002
  20. Schram G, 2013, J AM COLL CARDIOL, V61, P948, DOI 10.1016/j.jacc.2012.12.008
  21. Tamma R, 2013, LAB INVEST, V93, P592, DOI 10.1038/labinvest.2013.46
  22. Vainzof M, 1999, J NEUROL SCI, V164, P44, DOI 10.1016/S0022-510X(99)00040-4
  23. Walter MC, 2013, ORPHANET J RARE DIS, V8, DOI 10.1186/1750-1172-8-26
  24. Wong-Kisiel LC, 2010, NEUROMUSCULAR DISORD, V20, P122, DOI 10.1016/j.nmd.2009.11.005

Coleções
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