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Titolo:
Factor H deficiency and renal involvement.
Autore:
Niaudet, P; Dudley, J; Soto, B; May, A; Levy, M; Gubler, MC; Weiss, L;
Indirizzi:
Hop Necker Enfants Malad, INSERM U423, F-75015 Paris, France Hop Necker Enfants Malad Paris France F-75015 423, F-75015 Paris, France
Titolo Testata:
ANNALES DE PEDIATRIE
fascicolo: 2, volume: 46, anno: 1999,
pagine: 99 - 103
SICI:
0066-2097(199902)46:2<99:FHDARI>2.0.ZU;2-#
Fonte:
ISI
Lingua:
FRE
Soggetto:
HEMOLYTIC-UREMIC SYNDROME; HOMOZYGOUS FACTOR-H; 3RD COMPONENT; COMPLEMENT;
Keywords:
factor H; hemolytic uremic syndrome; membranoproliferative; glomerulonephritis; renal failure;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
16
Recensione:
Indirizzi per estratti:
Indirizzo: Niaudet, P HopeNecker Enfants Malad, INSERM U423, 149 Rue Sevres, F-75015 Paris, Franc Hop Necker Enfants Malad 149 Rue Sevres Paris France F-75015 c
Citazione:
P. Niaudet et al., "Factor H deficiency and renal involvement.", ANN PEDIAT, 46(2), 1999, pp. 99-103

Abstract

Factor H is a regulatory protein of the alternate complement activation pathway. Factor H deficiency manifesting as permanent alternate pathway activation has been reported in patients with hemolytic uremic syndrome (HUS), collagen III glomerulopathy, IgA nephropathy, systemic lupus erythematosus with C2 deficiency, and membranoproliferative glomerulonephritis. Six pediatric cases of factor H deficiency are reviewed. Three (cases 1-3), all boys,presented with atypical HUS characterized by hemolytic anemia, severe hypertension, and progression to end-stage renal failure. Recurrent infections occurred in cases 1 and 2, both of whom had repeatedly low factor H levels with low C3 levels. Case 3 had C3 levels in the low-to-normal range and normal factor H levels with reduced factor H activity. Cases 4 and 5 were brothers born to consanguineous parents and presented with recurrent macroscopic hematuria in the absence of significant proteinuria or renal function impairment. Renal biopsy demonstrated mesangial proliferation with dense C3 deposits in both cases. Case 6 was a 12-month-old girl presenting with macroscopic hematuria, nephrotic syndrome, anemia, and glomerular filtration rate(GFR) reduction. Renal biopsy demonstrated proliferative crescentic glomerulonephritis with C3 deposits. Following a course of steroid therapy, proteinuria decreased and both GFR and factor I-I returned to normal. These six cases illustrate the diversity of clinical and histological manifestations seen in factor H-deficient patients. The exact pathogenic role of factor I-I deficiency remains to be determined.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/04/20 alle ore 11:18:44