Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Sickle-cell disease in Brazzaville, Congo: Genetical, hematological, biochemical and clinical aspects
Autore:
Mouele, R; Boukila, V; Fourcade, V; Feingold, J; Galacteros, F;
Indirizzi:
Hop Henri Mondor, INSERM, U91, Unite Rech Genet Mol & Hematol, F-94010 Creteil, France Hop Henri Mondor Creteil France F-94010 Hematol, F-94010 Creteil, France Ctr Natl Transfus Sanguine, Brazzaville, Congo Ctr Natl Transfus SanguineBrazzaville Congo nguine, Brazzaville, Congo Ctr Hosp Univ, Serv Pediat Grands Enfants, Brazzaville, Congo Ctr Hosp Univ Brazzaville Congo diat Grands Enfants, Brazzaville, Congo Univ Paris 07, Lab Anthropol Biol, INSERM, U155,Unite Rech Genet Epidemiol, Paris, France Univ Paris 07 Paris France 55,Unite Rech Genet Epidemiol, Paris, France
Titolo Testata:
ACTA HAEMATOLOGICA
fascicolo: 4, volume: 101, anno: 1999,
pagine: 178 - 184
SICI:
0001-5792(1999)101:4<178:SDIBCG>2.0.ZU;2-M
Fonte:
ISI
Lingua:
ENG
Soggetto:
ALPHA-THALASSEMIA; GLOBIN GENE; HEMOGLOBIN-F; ANEMIA; HAPLOTYPES; AMPLIFICATION; POLYMERASE; SEVERITY; AFRICA;
Keywords:
Congo-Brazzaville; fetal hemoglobin; liver function tests; sickle cell anemia; a plus -thalassemia;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
27
Recensione:
Indirizzi per estratti:
Indirizzo: Mouele, R Hop Henri Mondor, INSERM, U91, Unite Rech Genet Mol & Hematol, 54 Ave Marechal de Lattre de Tassigny, F-94010 Creteil, France Hop Henri Mondor 54 Ave Marechal de Lattre de Tassigny Creteil France F-94010
Citazione:
R. Mouele et al., "Sickle-cell disease in Brazzaville, Congo: Genetical, hematological, biochemical and clinical aspects", ACT HAEMAT, 101(4), 1999, pp. 178-184

Abstract

Clinical, hematological and molecular features of 116 unrelated sickle-cell anemia patients from Brazzaville were investigated. The mean age of the patients was 9.4 +/- 5.3 years. 232 beta(s)-chromosomes were haplotyped and almost all sickle cell anemia patients (91%) were homozygous for the Bantu haplotype. All hematological indices were similar for males and females. Mean hemoglobin (Hb) concentration was 6.6 +/- 1.4 g/dl. Fetal Hb (HbF) levels were from 1 to 28%, with a mean of 8.8 +/- 5.8%. There was a curvilinear relationship between %HbF and age suggesting that HbF level had a selectiveeffect on the survival of patients. This effect was sex dependent. The (-alpha(3.7)) gene frequency was 0.45 and was not affected by stratification for age. Hematological characteristics of patients with (-alpha/alpha alpha,-alpha/-alpha) and without (alpha alpha/ alpha alpha) the -alpha(3.7) alpha-thal-2 deletion showed trends similar to those reported in Jamaican and US sickle cell anemia patients. Hyperbilirubinemia (>38 mu mol/l) was commonand high lactic dehydrogenase (LDH) concentrations were recorded. Bilirubin concentrations for males and females were similar whereas those for LDH concentration were not. Hepatomegaly and splenomegaly were common. Splenomegaly was strongly associated with the -alpha(3.7) alpha-thal-2 deletion. These clinical and hematological observations indicate a more severe form of hemolytic disease in Congolese SS patients.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/05/20 alle ore 15:12:29