Inherited complete factor I deficiency associated with systemic lupus erythematosus, higher susceptibility to infection and low levels of factor H

Inherited complete factor I deficiency associated with systemic lupus erythematosus, higher susceptibility to infection and low levels of factor H

Autor Amadei, N. Google Scholar
Baracho, Gisele Vanessa Google Scholar
Nudelman, Victor Autor UNIFESP Google Scholar
Bastos, W. Google Scholar
Florido, Marlene Pereira Carvalho Google Scholar
Isaac, Lourdes Google Scholar
Instituição Universidade de São Paulo (USP)
Universidade Federal de São Paulo (UNIFESP)
Fac Ciencias Med Santa Casa Misericordia Sao Paul
Resumo Here we describe two new cases of complete deficiency of factor I (fI) in two sisters from a consanguineous Brazilian family. the eldest sibling (20-year-old) developed systemic lupus erythematosus (SLE) early during childhood while the youngest had been committed on several occasions owing to repeated infections although she was asymptomatic for auto-immune diseases. We also detected lower concentrations of C3 and factor B in both sisters. Biological functions dependent on complement activation such as the production of opsonins and killing of phagocytozed micro-organisms, chemotactic factors and haemolytic activity were all significantly reduced in both probands. Consistent with the absence of fI and low levels of fH, a deregulated production of C3b was observed by bidimensional electrophoresis in sera of both the probands.
Idioma Inglês
Data de publicação 2001-06-01
Publicado em Scandinavian Journal of Immunology. Oxford: Blackwell Science Ltd, v. 53, n. 6, p. 615-621, 2001.
ISSN 0300-9475 (Sherpa/Romeo, fator de impacto)
Publicador Blackwell Science Ltd
Extensão 615-621
Fonte http://dx.doi.org/10.1046/j.1365-3083.2001.00931.x
Direito de acesso Acesso aberto Open Access
Tipo Artigo
Web of Science WOS:000169483700012
Endereço permanente http://repositorio.unifesp.br/handle/11600/26558

Exibir registro completo




Arquivo

Arquivo Tamanho Formato Visualização

Não existem arquivos associados a este item.

Este item está nas seguintes coleções

Buscar


Navegar

Minha conta