Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Progressive multifocal leukencephalopathy (PML) due to Papovavirus is usually combined with a cellular immunodeficiency as a consequence either of a neoproliferative disease or of medical treatment. In the first description of a case of PML in combination with substituted hemophilia A with no known cellular immunodeficiency, possible pathogenetic relations to factor VIII substitution as well as to AIDS, caused by HTLV-III virus, are discussed.
 ...
PMID:[Progressive multifocal leukoencephalopathy in hemophilia A. Is there a relation to AIDS?]. 299 34

To examine the role of antihemophilic factor (factor VIII) preparations in the pathogenesis of subclinical immunodeficiency in hemophilia, we tested the in vitro effects of these products on immune function. Both lyophilized antihemophilic factor (LAHF) and cryoprecipitates inhibited lymphocyte proliferation in a dose-dependent fashion. Further studies indicated that LAHF interfered with an early event in proliferation and also that prolonged incubation of human lymphocytes with LAHF resulted in an irreversible inhibition of lymphocyte proliferation without detectable cytotoxic effects. LAHF also inhibited the production of interleukin-2 (IL-2) by human lymphocytes and by Jurkat tumor cells, suggesting that inhibition of IL-2 production was not mediated through effects on interleukin-1. Gel filtration of LAHF revealed two peaks of inhibitory activity; one with mol wt greater than 2 X 10(6) comigrated with factor VIII coagulant activity and antigen, whereas another with mol wt approximately 6 X 10(5) was devoid of factor VIII activity and antigen. Further study will ascertain whether administration of factor VIII-containing preparations contributes to the subclinical immunodeficiency seen in patients with hemophilia or serves as a cofactor in the development of clinical immunodeficiency after exposure to the retrovirus human T-lymphotropic virus type III.
 ...
PMID:Antihemophilic factor [factor VIII] preparations inhibit lymphocyte proliferation and production of interleukin-2. 308 91

Four patients with hemophilia A have undergone liver transplantation in our institution, three successfully. The first was a 21-year-old man with chronic active hepatitis (CAH) in whom the effects of previous abdominal operations prevented the satisfactory technical insertion of the new liver. He died intraoperatively. The second patient was a 15-year-old boy with CAH who began to synthesize factor VIII coagulant activity (F VIII:C) within 18 hours of successful liver transplantation and has continued to do so for almost 2 years (F VIII:C range 0.89 to 3.20 U/mL). The first 2 months of his postoperative course were complicated by infections, but since that time he has done well and has returned to school. The third patient was a 48-year-old man with portal fibrosis and severe ascites. He synthesized F VIII:C (range 0.96 to 1.50 U/mL) within six hours after reestablishment of circulation through the new liver. His postoperative course was complicated by numerous infections, and he died with sepsis and an acquired immunodeficiency-like syndrome 4 months after transplantation. The fourth patient was a 47-year-old mild hemophiliac with CAH who produced adequate factor VIII:C levels following transplantation (range 0.79 to 2.80 U/mL). These patients demonstrate that liver transplantation in hemophiliacs with end-stage liver disease may be lifesaving and results in correction of the F VIII:C deficiency and associated hemorrhagic tendency.
 ...
PMID:Liver transplantation in hemophilia A. 1947 Apr 40

Because there have been reports that factor IX concentrate is less immunosuppressive and therefore factor IX users have less immunologic aberrations, we have studied a group of 22 patients with hemophilia B and six patients with factor VIII deficiency and high titer inhibitors with respect to lymphocyte numbers and function, human immunodeficiency virus (HIV) serology, and factor usage. This group was compared to 111 patients with hemophilia A and a group of 28 healthy male volunteer controls. When the study began in 1983, the majority of patients with hemophilia B and with higher titer factor VIII inhibitors were seronegative, 77% and 83% respectively, as compared to only 30% of patients with hemophilia A. At that time the factor IX users also had milder immune aberrations than the hemophilia A group. However, with time and increasing clotting factor concentrate usage, seroconversion and more striking abnormalities in immune function have occurred in the hemophilia B group. In a subgroup of 16 patients with hemophilia B studied twice, the incidence of seropositivity increased from 31% in 1983 to 69% in 1985. We thus conclude that factor IX concentrate in itself is not less immunosuppressive than factor VIII concentrate. Seroconversion in factor IX concentrate users appears to be lagging behind seroconversion in factor VIII concentrate users, perhaps secondary to the lower cumulative dosage of concentrate that patients with hemophilia B utilize.
 ...
PMID:Immunologic aberrations, HIV seropositivity and seroconversion rates in patients with hemophilia B. 310 24

Induction of immune tolerance in patients with severe hemophilia A and inhibitors to factor VIII was attempted by daily infusion of 50 U of factor VIII per kilogram of body weight without adjunctive immunosuppressive drugs. Modest initial anamnestic elevation of inhibitor levels occurred in six patients within the first month of therapy; inhibitor levels then fell sharply. The other six patients had no increase in inhibitor levels while on the study protocol. Inhibitors became undetectable within one to ten months in nine of the patients; they now receive smaller and less frequent infusions of factor VIII to maintain suppression. Inhibitors were not eradicated in three patients, who had the highest baseline and historic inhibitor levels. Ten patients gave consent for human immunodeficiency virus (HIV) testing; three had no antibody to HIV at the outset. Two of these patients seroconverted while on the protocol, one of whom had received only donor-screened, heat-treated factor VIII. Thus, the benefits of inhibitor suppression must be weighed against the risks of HIV seroconversion or transient elevation of inhibitor levels, as well as against the cost of the factor concentrate used.
 ...
PMID:Induction of immune tolerance to factor VIII in hemophiliacs with inhibitors. 311 78

At the change from unheated to heat-treated Factor VIII concentrates for the treatment of hemophilia A, 17 severe adult hemophiliacs (mean monthly dose, 4927 IU) were evaluated prospectively for signs of infection with human immunodeficiency virus (HIV). Viral serology and lymphocyte subpopulations (OKT3, OKT4, and OKT8-positive cells) were examined monthly for 1 year. One patient seroconverted for HIV in the enzyme-linked immunoabsorbent assay but was positive on the Western blot analysis from the outset. There was a slight but significant increase in OKT4+ cells and OKT4/OKT8 ratio. These data suggest that heat-treated Factor VIII concentrates even when used in large amounts have a low risk of transmitting HIV.
 ...
PMID:Risk of transmission of human immunodeficiency virus (HIV) by heat-treated factor VIII concentrates in patients with severe hemophilia A. 312 Mar 76

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

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

Short presentation of the common procedures to avoid transmission of human-immunodeficiency-virus (HIV) by hemoderivates especially clotting-factor-preparations. The stepwise seroconversion (ELISA, IFT, Western-blot) of HIV is shown in a 7 5/12 ys old boy with hemophilia A after administration of a dry-heated factor VIII-preparation. Seven similar observations were reported in the literature. On the other hand HIV-seroconversion could not be observed during treatment with wet-heated factor VIII-preparations. In consequence only wet-heated factor VIII-preparations and factor IX-preparations respectively should be administered to hemophiliacs without HIV-antibodies. By this precaution transmission of non-A, non-B-hepatitis may be avoided simultaneously.
 ...
PMID:[Transmission of the human immunodeficiency virus by a dry heat-treated Factor VIII concentrate?]. 314 70

To determine when the hemophiliacs in Fukuoka prefecture, Japan, first became positive for antibodies, we tested human immunodeficiency virus (HIV) antibodies on serum samples obtained from 1976-1987 stored at -30 C. Fifteen out of 64 hemophilia A patients (23.4%), five out of 11 hemophilia B patients (45.5%), but none of 17 patients with von Willebrand's disease (0%) were positive for HIV antibodies. In this series, two with hemophilia A became positive for HIV antibodies for the first time in 1983, and in 1984 another four with hemophilia A and one with hemophilia B became positive.
 ...
PMID:Prevalence of human immunodeficiency virus (HIV) infection among hemophiliacs in Fukuoka, Japan. 314 98


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