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Titolo:
The Malmo International Brother Study (MIBS): further support for genetic predisposition to inhibitor development
Autore:
Astermark, J; Berntorp, E; White, GC; Kroner, BL;
Indirizzi:
Univ Lund, Dept Coagulat Disorders, Malmo, Sweden Univ Lund Malmo Sweden niv Lund, Dept Coagulat Disorders, Malmo, Sweden Res Triangle Inst, Rockville, MD USA Res Triangle Inst Rockville MD USARes Triangle Inst, Rockville, MD USA
Titolo Testata:
HAEMOPHILIA
fascicolo: 3, volume: 7, anno: 2001,
pagine: 267 - 272
SICI:
1351-8216(200105)7:3<267:TMIBS(>2.0.ZU;2-E
Fonte:
ISI
Lingua:
ENG
Soggetto:
SEVERE HEMOPHILIA-A; FACTOR-VIII ANTIBODIES; MODEL;
Keywords:
genetics; haemophilia; inhibitors; siblings; twins;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
20
Recensione:
Indirizzi per estratti:
Indirizzo: Astermark, J Univ Hosp, Dept Coagulat Disorders, SE-20502 Malmo, Sweden Univ Hosp Malmo Sweden SE-20502 ers, SE-20502 Malmo, Sweden
Citazione:
J. Astermark et al., "The Malmo International Brother Study (MIBS): further support for genetic predisposition to inhibitor development", HAEMOPHILIA, 7(3), 2001, pp. 267-272

Abstract

The issue of factors predisposing for inhibitor development in haemophiliapatients is still largely unresolved. In an attempt to address this problem, we initiated a registry in 1996 of siblings with haemophilia and with orwithout a history of inhibitors. Four hundred and sixty families have accrued, of whom 388 suffer from haemophilia A and 72 haemophilia B. Twenty-five of the brother pairs are twins. The inhibitor incidence in all families with severe haemophilia A was 31.7%. The corresponding figure in the caucasian patients was 27.4%, whereas a higher incidence of inhibitors was reported in the black subjects (55.6%). Twins were reported in six of the 100 inhibitor families, for whom monozygocity was confirmed in three cases. In 32 families (32%), at least two brothers had a history of inhibitors. In 22 (69%) of these families, the inhibitor was also of the same type, i.e. either high- or low-responding. The overall concordance within the severe haemophilia A families was found to be 78.3% (195/249) compared to an expected figure of 68.0% and 58.0% using an inhibitor incidence of 20 and 30%, respectively (P < 0.0001). The corresponding figure for the twins was 88.2% (15/17). Moreover, the risk for inhibitor development in families with a previous inhibitor history was found to be 48% (95% confidence interval [CI] 35-62%),whereas the risk in families with no previous known inhibitor was only 15%(95% CI 11-21%) corresponding to a relative risk of 3.2 (95% CI 2.1-4.9). Immune-tolerance induction was reported in 24 families, of whom 13 siblingswere successfully treated. Our data clearly support the concept that a genetic predisposition for inhibitor development exists. However, the markers of this predisposition remain to be elucidated and we believe that the MIBSregistry will be useful for this purpose.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 06/04/20 alle ore 08:48:26