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

Concern for transmission of human T-cell lymphotropic virus, type 1 (HTLV-1) infection to recipients of infected cellular blood products has prompted development of tests to eliminate blood units with HTLV-I antibodies. Most hemophilic men from the United States became infected with human immunodeficiency virus (HIV) before HIV donor screening and before blood products were processed to inactivate the virus. To assess whether these men might also be infected with HTLV-I, we examined the HTLV-I antibody status of 127 factor VIII (hemophilia A) recipients and 71 factor IX (hemophilia B) recipients. One HIV-seronegative and four HIV-seropositive persons were HTLV-I reactive by enzyme-linked immunosorbent assay (ELISA). Four of five ELISA-reactive serum samples were negative by HTLV-I immunoblot assay (IB); 1 reactive and 1 borderline reactive serum were indeterminate on IB (p19 reactivity), but negative by radioimmunoprecipitation assay (RIPA). Peripheral blood mononuclear cells from one patient with indeterminate HTLV-I IB were negative for HTLV-I genomic sequences by polymerase chain reaction. The other indeterminate patient's serum antibody pattern was stable over a 2-year period, suggesting this was not an instance of early HTLV-I seroconversion. These results reaffirm the safety of factor components in the United States with regard to HTLV-I but emphasize the importance and need for further testing of reactive HTLV-I ELISA results with a second more specific technique.
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PMID:Absence of human T-cell lymphotropic virus type I coinfection in human immunodeficiency virus-infected hemophilic men. 250 96

Human immunodeficiency virus (HIV-is) now considered the causative agent of acquired immunodeficiency syndrome(AIDS). A high risk of AIDS has been reported among patients with hemophilia who received lyophilized commercial factor VIII and IX concentrates of American origin. In a prevalence survey conducted from September to December 1985, HIV antibodies were found in all the 4 patients with hemophilia treated with the batch number W87307, 955 I.U. of American commercial factor VIII concentrate supplied by Armour pharmaceutical Company U.S.A. One of the seropositive patients developed AIDS-related complex (ARC) and died of cerebral hemorrhage. The other three sero-positive patients had abnormalities in cell mediated immunity; among them two developed left lumbosacral radiculopathy and hemorrhagic herpes zoster and one remains well so far.
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PMID:[Clinical analysis of 4 Chinese hemophiliacs with human immunodeficiency virus infection]. 251 71

Human immunodeficiency virus (HIV) is now considered as the causative agent of acquired immunodeficiency syndrome (AIDS). A high risk of AIDS has been reported among patients with hemophilia who received lyophilized commercial factor VIII and IX concentrates of American origin. At a prevalent survey from September to December 1985, HIV antibodies were found in all four patients with hemophilia treated with the batch number W87307, 955 I.U. of American commercial factor VIII concentrate supplied by Armour Pharmaceutical Company, USA. One of the sero-positive patients developed AIDS-related complex (ARC) and died of cerebral hemorrhage. The other three sero-positive patients had abnormalities in cell-mediated immunity. Of them two developed left lumbosacral radiculopathy and hemorrhagic herpes zoster and one remained well so far.
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PMID:Clinical analysis of four Chinese hemophiliacs with human immunodeficiency virus infection. 251 21

A group of 64 multitransfused individuals with hemophilia or congenital hemolytic anemias were tested for antibodies against the human immunodeficiency virus. Thirty five of them were also evaluated clinically and their blood products supply was investigated. Only four hemophiliacs were found to be seropositive. The major risk factor that seemed associated with the acquisition of the virus was the transfusion of lyophilized factor VIII concentrate imported from the USA. A suggestion for control of transfusion associated infection and of contamination of hemophiliacs is presented.
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PMID:Clinical and serological study of the human immunodeficiency virus infection in a cohort of multitransfused persons. 251 39

In 1983, six patients who were exclusively treated with factor IX (FIX) concentrate (greater than 40,000 units/yr) were prospectively matched for age and dosage to six patients treated exclusively with factor VIII (FVIII) concentrate and to six normal male controls. At baseline evaluation between October 1983 and May 1984, both groups had significantly decreased absolute T helper cell counts (mean of 452/microliters and 505/microliters for FIX- and FVIII-treated groups, respectively) compared to normal (1,157/microliters). By August 1988, three of the six FIX-treated group have developed AIDS and two are seropositive for antibody to the human immunodeficiency virus (HIV). Four of the six FVIII-treated group have HIV seropositivity or disease. The other three patients (1/6 for FIX and 2/6 for FVIII) declined HIV antibody testing. Our results support other studies showing a dose-related risk of HIV exposure for FIX concentrate-treated patients and do not support the view that FIX concentrate was intrinsically safer than FVIII concentrate.
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PMID:Controlled prospective study of factor IX concentrate therapy and immunodeficiency. 252 89

Since 1982, when the World Federation of Hemophilia first published a document on the state of the art of hemophilia diagnosis and care, there have been lights and shadows in this field. Although the widespread infection of hemophiliacs with the human immunodeficiency virus (HIV) contaminating clotting factor concentrates is still a threatening and formidable shadow, the gloomy picture brought about by the AIDS epidemic is partially lightened by spectacular improvements in therapy and diagnosis. Carrier detection and first-trimester prenatal diagnosis can now be performed accurately in most kindreds by analysis of DNA of the factor VIII or IX genes. An important step forward towards the elimination of the risk of blood-borne infections transmitted by plasma products was recently made through the application of virucidal methods to clotting factor concentrates. Since HIV appears more vulnerable to such methods than the hepatitis viruses, currently available concentrates can be considered substantially free from the risk of transmitting HIV infection. Even though transmission of hepatitis is much reduced but not totally abolished, virucidal methods are continuously being improved, so that it can be foreseen that concentrates will become safer and safer. Finally, factor VIII produced by recombinant DNA technology is undergoing the first clinical trials in hemophiliacs. Hopefully, it will free from the risk of transmitting infections and will be available in sufficiently large amounts to meet the need of hemophiliacs worldwide. In 1982, the World Federation of Hemophilia published a message on the status of diagnosis and treatment of hemophilia. Since then, hemophilia care has been complicated by widespread infection of hemophiliacs with human immunodeficiency virus (HIV).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Hemophilia: state of the art of hematologic care 1988. 265 17

The clinico-pathological and epidemiological features of 119 cases of Kaposi's sarcoma diagnosed during the years 1979-1986 in the main pathology department of Rwanda are presented. Skin involvement (89%) was predominant with almost 70% of cutaneous lesions localised on the lower limbs; 11% of cases presented with extracutaneous localisations, 77% (10 cases out of 13) in the lymph nodes. Incidence rises progressively with age, and males are more affected than females with a sex ratio (m:f) of 6.4:1. The highest frequencies were observed in the western prefectures which border the province of Kivu in eastern Zaire. Histologically, three types were encountered: a mixed type (84%), a spindle cell-predominant type (12.6%) and an anaplastic type (3.4%). The factor VIII-related antigen was present in all 40 cases tested by the PAP method. Antihuman immunodeficiency virus antibodies were looked for in 18 cases: 10 cases with localised Kaposi's sarcoma, all of whom were seronegative, and 8 cases with aggressive generalised Kaposi's sarcoma, all of whom were seropositive. The results are compared with those of other authors, and the histogenesis and pathogenesis, particularly the relationship with the acquired immunodeficiency syndrome, are discussed.
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PMID:[Kaposi's sarcoma in Rwanda: clinico-pathological and epidemiological aspects]. 266 80

Cofactors for the clinical expression of infection due to the human immunodeficiency virus (HIV) are not well understood. We asked if there was a familial tendency to the development of complications of HIV infection. We examined 35 hemophilic sibships in which at least two brothers with classic hemophilia (factor VIII deficiency) were infected with HIV. Twenty-four (34%) of the 70 patients had serious sequelae of infection, and 46 (66%) were asymptomatic or had only lymph node enlargement. Using Fisher's exact test, we found the concordance among siblings for serious sequelae of HIV infection was greater than would be expected by chance. When analysis was restricted to include only siblings known to be infected for more than two years, this concordance was still present. In the study population, birth order and mean yearly usage of factor VIII concentrate were unrelated to the outcome of HIV infection. The data indicate a familial tendency to serious complications of HIV infection. The factor(s) responsible for this familial tendency are currently under investigation.
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PMID:Tendency to serious sequelae of infection with the human immunodeficiency virus in sibships with hemophilia. 270 37

Zidovudine was first prescribed for patients from the anti-human immunodeficiency virus (HIV) positive cohort of haemophiliacs in Newcastle in May 1987. Prior to this therapy, seven patients had died of acquired immune deficiency syndrome, and episodes of serious opportunistic infection were common. To date, 22 patients have received zidovudine, seven with or without acyclovir in a prospective Wellcome trial. Of the 22 patients, three were children and one was an adult female. All haemophilic patients were infected around 1982 as a result of factor VIII concentrate contamination with HIV. There have been five deaths, two occurring within 6 weeks of the start of zidovudine therapy. A third death was due to myocardial infarction in week 45. The other two deaths occurred at 41 weeks and 47 weeks in transfusion dependent patients. Only three serious opportunistic infections (pneumocystis pneumonia) have been observed in the remaining patients, one within a week of starting therapy and one in a non-compliant patient at week 24. The latter patients had a further episode of Pneumocystis carinii pneumonia in week 51. The transfusion dependent patients who died presented with anaemia at weeks 5 and 13, and required 48 and 28 units of packed cells respectively. A further patient required a single transfusion at week 7 and at week 43 continues to maintain an acceptable haemoglobin level.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Zidovudine: experience at the Newcastle Haemophilia Centre. 278 16

The phenotype and functions of monocytes in patients with haemophilia A and age-matched controls were studied. Fourteen male haemophiliacs were classified in three categories according to the mean number of units of factor VIII received during the last 5 years. Eleven patients were positive for antibodies to human immunodeficiency virus but none of our patients were homosexuals or drug abusers, nor do they fulfill the criteria of acquired immunodeficiency syndrome. Patients treated with high amounts of factor VIII concentrates (greater than 3 x 10(5) U/year) showed a significantly lower percentage of monocytes expressing HLA-DR, LFA-1 and CR3 antigens as compared with patients receiving lower amounts of factor VIII (less than 2 x 10(6) U/year) or controls. Kinetics of DR, LFA-1 and CR3 in cultured monocytes showed tht they were lost faster by monocytes from haemophiliacs treated with large amounts of factor VIII than by control monocytes. Adherence ability and chemotactic response of monocytes from patients treated with less than 3 x 10(5) U/year of factor VIII were also impaired. Although phagocytic indices were in normal ranges in haemophiliacs, a significant difference was observed between percentages of phagocytic monocytes from haemophiliacs treated with the largest doses of factor VIII and normal controls. Tests for respiratory burst activity, measured by chemiluminescence and superoxide anion generation, and Staphylococcus aureus killing were in normal ranges in haemophiliacs' monocytes.
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PMID:Phenotypic and functional abnormalities in monocytes from patients with haemophilia A treated with factor VIII concentrates. 282 91


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