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Titolo:
Bone marrow transplantation for T-B- severe combined immunodeficiency disease in Athabascan-speaking native Americans
Autore:
OMarcaigh, AS; DeSantes, K; Hu, D; Pabst, H; Horn, B; Li, L; Cowan, MJ;
Indirizzi:
Univ Calif San Francisco, Pediat Bone Marrow Transplantat Div, Dept Pediat, San Francisco, CA 94143 USA Univ Calif San Francisco San Francisco CA USA 94143 ancisco, CA 94143 USA Tuba City Indian Hlth Serv, Dept Pediat, Tuba City, AZ USA Tuba City Indian Hlth Serv Tuba City AZ USA pt Pediat, Tuba City, AZ USA Univ Alberta, Dept Pediat, Edmonton, AB, Canada Univ Alberta Edmonton AB Canada berta, Dept Pediat, Edmonton, AB, Canada
Titolo Testata:
BONE MARROW TRANSPLANTATION
fascicolo: 7, volume: 27, anno: 2001,
pagine: 703 - 709
SICI:
0268-3369(200104)27:7<703:BMTFTS>2.0.ZU;2-C
Fonte:
ISI
Lingua:
ENG
Soggetto:
STEM-CELL TRANSPLANTATION; IMMUNE RECONSTITUTION; CHROMOSOME 10P; GENE; POPULATION; CHILDREN;
Keywords:
bone marrow transplantation; severe combined immunodeficiency; SCID; Athabascan;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
24
Recensione:
Indirizzi per estratti:
Indirizzo: Cowan, MJ Univ Calif San Francisco, Pediat Bone Marrow Transplantat Div, Dept Pediat, 505 Parnassus Ave,Box 1278, San Francisco, CA 94143 USA Univ Calif San Francisco 505 Parnassus Ave,Box 1278 San Francisco CA USA 94143
Citazione:
A.S. O'Marcaigh et al., "Bone marrow transplantation for T-B- severe combined immunodeficiency disease in Athabascan-speaking native Americans", BONE MAR TR, 27(7), 2001, pp. 703-709

Abstract

A distinct form of autosomal recessive T-B- severe combined immunodeficiency disease occurs with a high frequency among Athabascan-speaking Native Americans (SCIDA), including Navajo and Apache Indians from the southwestern US and Dene Indians from the Canadian Northwest Territories. The SCIDA genehas been linked to markers on chromosome 10p although its identity and role in the pathogenesis of this disease are unknown, We report our experiencein treating 18 Navajo and Dene children with SCIDA between 1984 and 1999; 16 underwent bone marrow transplants (BMT). All children were symptomatic within 2 months of birth, had the T-B-NK+ SCID phenotype and 67% presented with oral and/or genital ulcers, Three children had evidence of maternal engraftment prior to transplant. Two children died shortly after diagnosis. Three children required more than one BMT and 12 are alive with T cell reconstitution at a median follow up of 7 years, Three children developed normal B cell immunity, two of whom received ablative conditioning therapy with either radiation or busulfan, Three of the foils children who died received therapy with either radiation or busulfan and two of eight long-term survivors who were also recipients of cytotoxic chemotherapy have failed to develop secondary teeth. These results demonstrate the efficacy of BMT in treating infants with this distinct form of SCID, although B cell reconstitution remains a problem even with HLA-matched donors. Without conditioning, T cellengraftment is likely when closely HLA-matched donors are used. With T cell depletion of haplocompatible marrow, conditioning with immunosuppressive therapy may be necessary; however, children with SCIDA who were treated with intensive immunosuppressive and myeloablative therapy had a poor outcome.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 10/07/20 alle ore 15:55:50