Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0001175 (AIDS)
 120,706 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The eradication of a high-response Factor VIII inhibitor in patients with severe hemophilia A is extremely rare even with prolonged immunosuppressive therapy. This report presents a patient with severe hemophilia A, in whom the disappearance of such an inhibitor coincided with the development of the acquired immunodeficiency syndrome (AIDS). Laboratory studies demonstrated a marked decrease in helper T-cells and marked depression of cell-mediated immunity by in vivo and in vitro testing. In addition, humoral immune responses were abnormal. Thus, anamnestic antibody formation to different antigens was absent and in vitro pokeweed mitogen-induced immunoglobulin synthesis by the patient's B-cells was markedly impaired even in the presence of normal T-cells. These findings indicate that the disappearance of the Factor VIII inhibitor and the lack of an anamnestic antibody response to infused Factor VIII observed in this patient may be secondary to a humoral immunodeficiency associated with AIDS.
 ...
PMID:Disappearance of a high response factor VIII inhibitor in a hemophiliac with AIDS. 312 44

Between January 1, 1981 and September 4, 1987, 407 cases of hemophilia-associated acquired immunodeficiency syndrome (AIDS) had been reported to the Centers for Disease Control. The number of cases diagnosed each year nearly doubled, except in 1986, when cases increased only 50 per cent. Demographic characteristics of the patients did not change over time. The majority (74 per cent) had severe hemophilia and 97 per cent received commercially produced concentrated clotting factors. The cumulative incidence of AIDS between 1981 and September 1987 for persons with severe hemophilia A was substantially greater than that for persons with severe hemophilia B (4.2 cases vs 1.9 cases per 100 persons).
 ...
PMID:Hemophilia-associated AIDS in the United States, 1981 to September 1987. 312 75

A patient with hemophilia A and transfusion-associated end-stage chronic liver disease underwent orthotopic liver transplantation. He had no requirement for exogenous factor VIII replacement during the 27 mo he survived. Although his hemophilia was cured, he had antibodies to the human immunodeficiency virus; ultimately he died of complications arising from acquired immunodeficiency syndrome. Liver transplantation for cirrhotic hemophiliacs can free them of the need for antihemophilic-factor therapy; however, application of this approach may be limited by the high prevalence of human immunodeficiency virus infection in multitransfused hemophiliacs.
 ...
PMID:Cure of hemophilia A by orthotopic liver transplantation. 313 Nov 78

Since 1983, the Medical-Scientific Committee of the Fondazione dell'Emofilia has carried out annual national surveys of patients with hemophilia and other congenital bleeding disorders to evaluate the prevalence and characteristics of human immunodeficiency virus (HIV) infection and related diseases. Clinical syndromes related to HIV infection were already present in a number of Italian hemophiliacs in 1983, but the first cases of acquired immunodeficiency syndrome (AIDS) only occurred in 1984. Subsequently, AIDS cases roughly doubled each year to reach the number of 57 in 1987. In this year, 637 of 2,792 patients (23%) were found to be anti-HIV-positive. The highest prevalence of seropositivity was found in hemophilia B patients (138 of 313, 44%), followed by hemophilia A (476 of 1,658, 29%), von Willebrand's disease (23 of 650, 4%) and other congenital bleeding disorders (2 of 171, 1%). Only 3 cases of seroconversions were observed since 1986, following the nationwide adoption of heat-treated concentrates. The differences in the prevalence of HIV infection between hemophiliacs from Italy and other European countries are discussed.
 ...
PMID:National survey of human immunodeficiency virus infection in Italian hemophiliacs: 1983-1987. The Medical-Scientific Committee of the Fondazione dell'Emofilia. 315 Jan 22

Pneumocystis carinii antigen and IgG antibody profiles were prepared on 17 pediatric patients with acquired immunodeficiency syndrome (AIDS) with pneumonia who were examined by a variety of invasive methods for P carinii organisms. Overall, the accuracy of the antigen assay in invasively examined pediatric patients with AIDS with pneumonia was 94% (sensitivity, 100%; specificity, 90%), as antigen and invasive test results agreed in 16 of 17 patients. No statistically significant differences in IgG titer were observed between controls and patients invasively examined for P carinii, whether the organism was observed in the specimen or not. Since 38% of all serum samples referred were derived from "blood-borne" cases of AIDS, including patients who contracted AIDS as a result of both transfusion and hemophilia A, this suggests that P carinii pneumonia or P carinii pneumonia- like pneumonias may be more common in these individuals.
 ...
PMID:Pneumocystis carinii serologic study in pediatric acquired immunodeficiency syndrome. 325 37

We tested serum samples from 11 HIV-seropositive and 32 HIV-seronegative Italian patients with hemophilia A or B for the presence of lymphocytotoxic antibodies (LCTAs). No patients had the acquired immune deficiency syndrome and all had received over many years heat-untreated commercial clotting factors. LCTAs directed mainly to B cells, were significantly present in 5/6 HIV-seropositive patients with hemophilia A and in 5/5 HIV-seropositive patients with hemophilia B. The presence of LCTAs in HIV-seronegative hemophiliacs was significantly correlated with both a decrease of T cells and an increase in serum levels of IgG and IgM.
 ...
PMID:Non-HLA lymphocytotoxic antibodies in HIV-seropositive and HIV-seronegative hemophiliacs. 325 80

A generalized lymphadenopathy syndrome (GLS) occurs in persons at high risk for development of acquired immunodeficiency syndrome (AIDS). The natural history and immunologic status of patients with GLS are not fully known, although in some persons GLS may progress to full AIDS. We present the clinical and immunologic findings in two children with severe hemophilia A with nonprogressive GLS for 18-24 months. The functional activity in vitro of lymphocytes from both peripheral blood and biopsied lymph nodes were compared. The peripheral blood lymphocytes responded normally to both mitogens and antigens; lymph node lymphocytes failed to respond to antigens, but did respond to mitogens. The implications of these abnormalities for understanding the pathogenesis of GLS are discussed.
 ...
PMID:Immunologic studies of lymph node lymphocytes in the generalized lymphadenopathy syndrome. 349 16

Studies in hemophiliacs receiving factor concentrates demonstrated T-cell defects in vitro. Recently, B-cell dysfunctions were described in AIDS and pre-AIDS and in some hemophiliacs. To investigate the B-cell function in hemophiliacs in relation to factor substitution, we examined five patients with mild (substitution less than 20,000 U/year) and seven with severe (greater than 100,000 U/year) hemophilia A and compared the data with normal control individuals. The B-cell proliferative response (3H-thymidine uptake) to Staphylococcus aureus Cowan I and the differentiation response (Ig secretion into culture supernatants) to T-cell-dependent or -independent polyclonal B-cell activators (PBAs) were studied in vitro. In contrast to T-cell dysfunctions, which correlate with the amount of clotting factor concentrates, the B-cell proliferative response was not affected. Stimulation with PBAs however failed to increase elevated spontaneous IgG levels and showed a diminished increase in IgM levels in severe, but not in mild, hemophilia. Our data give evidence of a T-cell-independent B-cell dysfunction in asymptomatic hemophiliacs that correlates with factor substitution.
 ...
PMID:Influence of factor substitution on the B-cell response in hemophiliacs. 350 Jul 81

Twenty-eight patients from the Nebraska Regional Hemophilia Center were studied for the prevalence and titers of antibodies to lymphadenopathy-associated virus/human T cell lymphotropic virus type III (LAV/HTLV-III) and for clinical symptoms of possible progression to the acquired immune deficiency syndrome (AIDS). Ten of 18 (56 percent) patients with hemophilia A who were frequently treated with commercial factor VIII concentrate were seropositive for LAV/HTLV-III antibodies as determined by immunofluorescent study and Western blot testing. Of the four factor VIII-deficient patients who were seronegative, one had received only heat-treated factor VIII concentrates, two had received only cryoprecipitate, and one had received no transfusions since 1983. None of the patients treated only with factor IX concentrate, volunteer donor plasma, or cryoprecipitate had LAV/HTLV-III antibodies. In nine of 10 seropositive hemophiliacs, titers of serum antibodies to LAV/HTLV-III ranged from 1:1,280 to 1:10,240, indicating a strong immune response against LAV/HTLV-III antigens and/or persistent infection with the virus. Serum from seropositive hemophiliacs interacted on Western blot testing with all the major LAV/HTLV-III polypeptides, including envelope proteins gp 42 and gp 120. Despite the possible exposure to LAV/HTLV-III during the past four years, none of the patients in this group had symptoms suggestive of progression towards AIDS. Whether or not immunity to the AIDS retrovirus developed in this group of patients remains to be determined.
 ...
PMID:High prevalence and high titers of LAV/HTLV-III antibodies in healthy hemophiliacs in the midwestern United States. 353 87

Persons with hemophilia who have received therapy since 1978 are at risk for the acquired immunodeficiency syndrome. Plasma and serum specimens collected from 1980 to 1985 from 73 New Mexico residents with hemophilia were tested by enzyme immunoassay for antibody to human T-cell lymphotropic virus type III (HTLV-III), and positive results were confirmed by Western blot. Antibody to HTLV-III was first detected in New Mexico residents with hemophilia in 1981. Among 49 persons tested in 1984-1985, seropositivity was found in 35%. Of these, 17 of 32 (53%) commercial concentrate versus 0 of 17 cryoprecipitate users were seropositive (P = .002). Of the 7 with hemophilia B, 3 (43%) were seropositive versus 14 of 42 (33%) with hemophilia A. Of 17 Albuquerque residents with hemophilia, 2(12%) were seropositive as compared with 15 of 32 (47%) persons with hemophilia who resided outside the city (P = .02). Compared with patients with hemophilia outside of Albuquerque, those living in Albuquerque tended to have milder disease and to use cryoprecipitate rather than commercial concentrate. Less frequent treatment (mild disease) and use of cryoprecipitate were associated with a decreased risk of HTLV-III infection.
 ...
PMID:Prevalence of HTLV-III antibody among New Mexico residents with hemophilia. 364


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>