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Titolo:
ANTIBODY DEFICIENCY IN WOLF-HIRSCHHORN-SYNDROME
Autore:
HANLEYLOPEZ J; ESTABROOKS LL; STIEHM ER;
Indirizzi:
UNIV CALIF LOS ANGELES,CHILDRENS HOSP,DIV IMMUNOL,22-387-MDCC,10833 LE CONTE AVE LOS ANGELES CA 90095 UNIV CALIF LOS ANGELES,CHILDRENS HOSP,DIV IMMUNOL LOS ANGELES CA 90095 GENZYME SANTA FE NM 00000
Titolo Testata:
The Journal of pediatrics
fascicolo: 1, volume: 133, anno: 1998,
pagine: 141 - 143
SICI:
0022-3476(1998)133:1<141:ADIW>2.0.ZU;2-3
Fonte:
ISI
Lingua:
ENG
Soggetto:
COMMON VARIABLE IMMUNODEFICIENCY; SELECTIVE IGA DEFICIENCY; GENES;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
12
Recensione:
Indirizzi per estratti:
Citazione:
J. Hanleylopez et al., "ANTIBODY DEFICIENCY IN WOLF-HIRSCHHORN-SYNDROME", The Journal of pediatrics, 133(1), 1998, pp. 141-143

Abstract

We identified antibody deficiencies in 9 of 13 infection-prone children with the Wolf-Hirschhorn syndrome (4p-monosomy). Eight of the immunodeficient children were identified by a questionnaire sent to 190 families with an affected child. Two of the children had common variable immunodeficiency, one had IgA and IgG2 subclass deficiency, three had IgA deficiency, and three had impaired polysaccharide responsiveness. T-cell immunity was normal. The association of antibody defects with Wolf-Hirschhorn syndrome suggests a regulatory gene within the deleted chromosome region that affects the B cell system.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 18/09/20 alle ore 15:40:55