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Titolo:
HEREDITARY COMPLEMENT FACTOR-I DEFICIENCY
Autore:
VYSE TJ; SPATH PJ; DAVIES KA; MORLEY BJ; PHILIPPE P; ATHANASSIOU P; GILES CM; WALPORT MJ;
Indirizzi:
HAMMERSMITH HOSP,ROYAL POSTGRAD MED SCH,RHEUMATOL UNIT,DU CANE RD LONDON W12 0NN ENGLAND HAMMERSMITH HOSP,ROYAL POSTGRAD MED SCH,RHEUMATOL UNIT LONDON W12 0NNENGLAND SWISS RED CROSS,BLOOD TRANSFUS SERV,CENT LAB,ZLB BERN SWITZERLAND REG HOSP DELEMONT DELEMONT SWITZERLAND
Titolo Testata:
Quarterly Journal of Medicine
fascicolo: 7, volume: 87, anno: 1994,
pagine: 385 - 401
SICI:
1460-2725(1994)87:7<385:HCFD>2.0.ZU;2-K
Fonte:
ISI
Lingua:
ENG
Soggetto:
C3B INACTIVATOR DEFICIENCY; SYSTEMIC LUPUS-ERYTHEMATOSUS; CIRCULATING IMMUNE-COMPLEXES; INHERITED C-3 DEFICIENCY; 3RD COMPONENT; FACTOR-H; MENINGOCOCCAL DISEASE; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS; INCREASED SUSCEPTIBILITY; CONGENITAL DEFICIENCY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
65
Recensione:
Indirizzi per estratti:
Citazione:
T.J. Vyse et al., "HEREDITARY COMPLEMENT FACTOR-I DEFICIENCY", Quarterly Journal of Medicine, 87(7), 1994, pp. 385-401

Abstract

We describe four cases (from three families) of hereditary factor I deficiency, bringing the total number of cases now reported to 23. In one family there are two affected siblings: one has suffered recurrent pyogenic infections; the other is asymptomatic. In the second family, the patient had recurrent pyogenic infections and a self-limiting vasculitic illness; in the third family, the patient suffered recurrent pyogenic and neisserial infections. All four patients had markedly reduced concentrations of C3 in the serum (family 1 propositus: 28%; family1 asymptomatic sibling: 15%; family 2: 31%; and family 3: 31% normal human serum) which was in the form of C3b. Low IgG, levels may occur in primary C3 deficiency, and a reduction in IgG, concentration to 1.14g/l (normal: 1.30-5.90 g/l) was found in the patient from family 2. Using radioligand binding assays, we demonstrated increased binding of C3b to erythrocytes in a patient with factor I deficiency. This C3b could not be cleaved by autologous serum but could be cleaved by normal serum or purified factor I. We review and compare the published cases of C3, factor H and factor I deficiency.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 10/07/20 alle ore 00:15:58