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Titolo:
Advances in the understanding and treatment of human severe combined immunodeficiency
Autore:
Buckley, RH;
Indirizzi:
Duke Univ, Med Ctr, Dept Pediat, Durham, NC 27710 USA Duke Univ Durham NCUSA 27710 Med Ctr, Dept Pediat, Durham, NC 27710 USA Duke Univ, Med Ctr, Dept Immunol, Durham, NC 27710 USA Duke Univ Durham NC USA 27710 Med Ctr, Dept Immunol, Durham, NC 27710 USA
Titolo Testata:
IMMUNOLOGIC RESEARCH
fascicolo: 2-3, volume: 22, anno: 2000,
pagine: 237 - 251
SICI:
0257-277X(2000)22:2-3<237:AITUAT>2.0.ZU;2-Q
Fonte:
ISI
Lingua:
ENG
Soggetto:
BONE-MARROW TRANSPLANTATION; STEM-CELL TRANSPLANTATION; RECEPTOR-GAMMA-CHAIN; ADENOSINE-DEAMINASE DEFICIENCY; IN-UTERO TRANSPLANTATION; OMENNS-SYNDROME; IMMUNE RECONSTITUTION; THYMIC FUNCTION; SOYBEAN LECTIN; SINGLE-CENTER;
Keywords:
severe combined immunodeficiency (SCID); genetic defects causing SCID; marrow transplantation; recent thymic emigrants; gene therapy;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
68
Recensione:
Indirizzi per estratti:
Indirizzo: Buckley, RH Duke Univ, Med Ctr, Dept Pediat, Box 2898, Durham, NC 27710 USA Duke Univ Box 2898 Durham NC USA 27710 8, Durham, NC 27710 USA
Citazione:
R.H. Buckley, "Advances in the understanding and treatment of human severe combined immunodeficiency", IMMUNOL RES, 22(2-3), 2000, pp. 237-251

Abstract

Human severe combined immunodeficiency (SCID) can result from mutations inany one of at least seven different genes, including those for adenosine deaminase, the common cytokine receptor gamma chain, Janus kinase 3, IL-7 receptor alpha chain, recombinase activation genes 1 and 2, and CD45. Except for adenosine deaminase, knowledge concerning the latter causes of human SCID has accrued since 1993. Advances in the treatment of this syndrome have been no less significant. Since 1982 it has been possible, by rigorous depletion of T cells from the donor marrow, to use related marrow donors other than HLA-identical siblings for successful treatment of infants with this condition. The success rate with the latter type of transplant exceeds 95% if a transplant can be per formed within the first 3.5 mo of life, making early diagnosis crucial. Recently, gene therapy has also been successful in infants with X-linked SCID.

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Documento generato il 27/11/20 alle ore 22:14:37