RESUMO
In France, patients with haemophilia were infected by HIV up until October 1985, with a maximum of seroconversion between 1983 and 1985. There was a progressive development of AIDS in the 1158 infected patients as reported by Health Authorities. By the end of 1992, 32% of the haemophilia population had developed AIDS and 38 had developed clinical or biological symptoms of immunodeficiency. However, 27% had no clinical symptoms and no severe disorder of the immune system. The present study was established to determine factors common to patients with prolonged survival.
RESUMO
The prevalence of serum antibodies to hepatitis A virus (HAV) in 793 hemophilia A (HA) and 89 hemophilia B (HB) patients coming from 10 French Hemophilia Centers and treated since 1986-1987 with solvent/detergent (SD)-treated products is reported. The results indicated seropositivity to HAV of 29.9% in HA and 40.4% in HB patients. There was no difference among the patients according to severity of the disease, HIV serology or administration of factor VIII during the last 12 months. Seropositivity increased with age from 5.2% in HA children to 42.4% in adults (in HB the respective prevalences in the same groups were 7.7% and 56.1%). When compared to normal controls (n = 585), the prevalence of HAV seropositivity was not excessive in HA patients (n = 206). 19/20 children exclusively treated with a very-high-purity SD-factor VIII concentrate (Centre régional de transfusion sanguine, Lille) remained HAV seronegative. Six cases of HAV contamination were reported in patients with severe HA, probably reflecting the level of HAV endemy in the normal population in France. No special risk of HAV transmission linked to the SD products used in France since 1986 had thus been identified.
Assuntos
Transfusão de Componentes Sanguíneos/efeitos adversos , Hemofilia A/terapia , Hepatite A/epidemiologia , Adolescente , Adulto , Criança , Fator VIII/efeitos adversos , França/epidemiologia , Hepatite A/transmissão , Humanos , Masculino , PrevalênciaRESUMO
Evolution of HIV infection was studied in 480 hemophiliacs A and 78 hemophiliacs B treated in the "Centre-West" Region. 23.3% hemophiliacs A and 46.1% hemophiliacs B were contaminated by HIV. In this region, HIV seroprevalence in hemophiliacs A was lower than the prevalence noted at the national level (51.2%); this is certainly due to the use of frozen cryoprecipitates in the treatment of a high number of hemophiliacs A. A higher number of hemophiliacs B developed the disease: 12.5% hemophiliacs A versus 22% hemophiliacs B. Moreover hemophilic B patients had a more rapid evolution towards the disease since 6 out of 14 hemophiliacs A and 7 out of 8 hemophiliacs B with AIDS died. The fact that hemophiliacs B were significantly older than hemophiliacs A might be one of the reasons, but it must be noted that the contamination often occurred earlier in hemophiliacs B and was perhaps more important. The more severe evolution in the hemophiliac B group noted in our region is not found in American studies, which may be due to the different ways of preparing Factor IX concentrates in France and the United States.
Assuntos
Fator IX/uso terapêutico , Fator VIII/uso terapêutico , Infecções por HIV/epidemiologia , Soroprevalência de HIV , Hemofilia A/epidemiologia , Hemofilia B/epidemiologia , Reação Transfusional , Adolescente , Adulto , Idoso , Doadores de Sangue , Criança , Pré-Escolar , Comorbidade , Contaminação de Medicamentos , França/epidemiologia , Infecções por HIV/etiologia , Infecções por HIV/mortalidade , Infecções por HIV/transmissão , Hemofilia A/terapia , Hemofilia B/terapia , Humanos , Lactente , Tábuas de Vida , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Análise de SobrevidaRESUMO
In a study of the transmissibility of human immunodeficiency virus (HIV) in haemophilic and non-haemophilic children living together in a boarding school in France, half of the haemophilic children had seroconverted by the end of a 3-year study period. By contrast none of the non-haemophilic children seroconverted. All children had had close casual contact, some of them for several years. Hepatitis B virus (HBV) markers detected in all polytransfused haemophiliacs were found in 4 of 20 control children in the school, whereas all healthy youngsters living with their families were HBV negative. This study adds support to the theory that transmissibility of HIV among casual contacts is low and that there is no reason to exclude HIV-antibody carriers from communities.
Assuntos
Síndrome da Imunodeficiência Adquirida/transmissão , Hemofilia A/complicações , Síndrome da Imunodeficiência Adquirida/imunologia , Doença Aguda , Adolescente , Adulto , Anticorpos Antivirais/análise , Criança , Pré-Escolar , Contaminação de Medicamentos , Fator IX/uso terapêutico , Fator VIII/uso terapêutico , França , Anticorpos Anti-HIV , Hemofilia A/imunologia , Hemofilia A/terapia , Vírus da Hepatite B/imunologia , Humanos , Imunoglobulina G/análise , Lactente , Recém-Nascido , Linfócitos/classificação , Masculino , Características de Residência , Estudos RetrospectivosRESUMO
About one homozygote female for haemophilia, authors report circumstances where we can see an haemophilic female: lyonisation, chromosomal abnormalities of X chromosome, and homozygosis. They report the cases of literature.
Assuntos
Hemofilia A/genética , Adulto , Mecanismo Genético de Compensação de Dose , Feminino , Homozigoto , Humanos , Linhagem , Aberrações dos Cromossomos Sexuais/genética , Cromossomo XRESUMO
Two probably monozygotic twins with cerebral gigantism are reported. They showed three typical features: pneumo-encephalography demonstrated a fifth anterior ventricular dilatation; in both patients dermatoglyphic findings showed a thenar exit of the Aline and a vertical palmar alignement; Growth hormone and sulfatation factor blood concentration were low but within normal limits.
