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Master's Dissertation
DOI
10.11606/D.42.2000.tde-30032001-124158
Document
Author
Full name
Axel Gustavo Ulbrich
E-mail
Institute/School/College
Knowledge Area
Date of Defense
Published
São Paulo, 1999
Supervisor
Committee
Isaac, Lourdes (President)
Condino Neto, Antonio
Ferriani, Virginia Paes Leme
Title in Portuguese
Estudo de um caso de deficiência do componente C3 do sistema complemento humano.
Keywords in Portuguese
C3
complemento
deficiência
deficiência de C3
imunodeficiência
Abstract in Portuguese
Uma criança brasileira (LAS) vítima de infecções recidivantes e vasculite, cujos pais são consangüíneos em segundo grau apresentou 0,15 µg/mL de C3 plasmático e atividades hemolíticas nulas pelas vias clássica e alternativa, já outras proteínas do complemento e Igs estavam normais (exceto IgG4, que foi indetectável). Diferentemente de sua mãe os fibroblastos da criança não foram capazes de sintetizar as cadeias a e b de C3, como observado por SDS-PAGE. O probando possui dois alelos C3S, assim como seu irmão mais novo e saudável, enquanto a mãe é FS. A migração de leucócitos, em resposta ao soro do probando ativado com LPS foi menor que a obtida com soro normal e estatisticamente semelhante àquela gerada por SHN inativado a 56oC (SHNi). A ingestão e a morte de C. albicans, opsonizadas por soro do probando, por fagócitos normais foram semelhantes às dos fungos opsonizados por SHNi. Nós concluimos que, em conseqüência da incapacidade de sintetizar C3, o probando não é capaz de exercer as funções imunológicas dependentes do complemento, resultando em uma maior susceptibilidade a infecções.
Keywords in English
complement
Abstract in English
A brasilian child (LAS) victim of recurrent infections whose parents have second degree consanguinity presented 0.15 µg/mL of serum C3 and no hemolytic activities either after activation of the classical or alternative pathways. His mother presented C3 alpha and beta chains of normal sizes, while LAS's fibroblasts did not secrete any C3 as observed by SDS-PAGE. The proband possesses two C3S alleles, like his younger and healthy brother whereas his mother is FS. Leukocyte migration across nitrocellulose membrane in response to the proband's LPS-activated serum was less intense than that obtained in response to normal serum. Phagocytosis and killing of C. albicans opsonized with the proband's serum was comparable to fungi opsonized with inactivated serum, incdicating that chemotactic and opsonic activities of the proband's serum are greatly diminished. We colclude that as a consequence of C3 deficiency the proband's complement system is uncapable of performing it's normal effector functions resulting in greater susceptibility to infections.
 
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2001-12.pdf (707.43 Kbytes)
Publishing Date
2001-03-30
 
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