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)

Cell lineage and cell function antigens were studied immunohistochemically in human immunodeficiency virus-associated oral Kaposi's sarcoma to provide insight into tumor pathogenesis. All tumors were composed predominantly of spindle cells that expressed endothelium-associated antigens, CD34 and CD36 (factor VIII-related antigen was expressed by considerably fewer numbers of tumor cells). Infrequently, spindle tumor cells also expressed actin. Factor XIIIa positive spindle and dendritic stromal cells comprised up to 9% of the tumor cell population. Other spindle and dendritic cells expressing macrophage-associated antigen, CD68, accounted for up to 15% of the tumor cells. Mast cells occurred frequently within and around tumors. Leukocyte function antigen (CD18) was expressed by approximately 13% of tumor cells, and its ligand, intercellular adhesion molecule (ICAM), was expressed by some tumor-associated capillaries (which also expressed endothelial leukocyte adhesion molecule, ELAM) and occasional stromal cells. Staining for proliferating cell nuclear antigen was noted in both interstitial and vascular lining cells. All tumors were non-reactive for human Papillomavirus antigen and HIV p24 antigen. Oral KS is a heterogeneous cellular proliferation composed predominantly of endothelial or endothelium-related spindle cells. Other spindle/dendritic (XIIIa-positive and CD68-positive) cells and mast cells are also present and may contribute to tumor development. ICAM and ELAM expression within tumors may assist infiltration of macrophages and other inflammatory cells into these lesions.
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PMID:Human immunodeficiency virus-associated oral Kaposi's sarcoma. A heterogeneous cell population dominated by spindle-shaped endothelial cells. 810 Apr

Infection with the human immunodeficiency virus (HIV) can result in several cardiac abnormalities including dilated cardiomyopathy. These phenomena have been described in people contracting the virus through sexual intercourse, injection drug use and by vertical transmission. We have identified recently two Scottish haemophiliacs who have developed dilated cardiomyopathy in the context of HIV infection acquired through treatment with contaminated factor VIII. The significance of this finding is discussed.
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PMID:Dilated cardiomyopathy in haemophiliacs infected with the human immunodeficiency virus. 823 60

This prospective study was carried out with the aim of evaluating the efficacy of solvent/detergent inactivation of the hepatitis C virus (HCV) as applied to a chromatographic factor VIII concentrate. In parallel, the markers for other viruses, either lipid-enveloped (human immunodeficiency virus types 1 and 2 [HIV-1 and -2] and hepatitis B virus [HBV]) or non-lipid-enveloped viruses (such as B19 parvovirus and hepatitis A virus [HAV]) were evaluated. The study included 14 hemophilia centers, which enrolled 36 previously untreated patients (median age, 3 years; range, 1-56). The length of follow-up was 12 months, during which HCV (first- and second-generation assays and recombinant immunoblot assay), HIV-1 and -2, HBV, HAV (IgG and IgM), and parvovirus (IgG and IgM) antibodies, as well as alanine aminotransferase values were evaluated. Thirty-one patients were analyzable; none seroconverted for HCV, HBV, or HIV after exposure to a total of 165,000 IU of factor VIII (41 different lots). In one patient, alanine aminotransferase values rose to 167 mU per mL, 6 weeks after the first concentrate infusion, and this patient seroconverted for HAV 1 week later. Furthermore, 10 patients seroconverted for parvovirus during follow-up. This study suggests that the solvent/detergent method of virus inactivation is efficient in relation to lipid-enveloped blood-borne viruses but not in relation to non-lipid-enveloped viruses.
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PMID:Prospective study of the evaluation of hepatitis C virus infectivity in a high-purity, solvent/detergent-treated factor VIII concentrate: parallel evaluation of other markers for lipid-enveloped and non-lipid-enveloped viruses. The Ad Hoc Study Group of the Fondazione dell'Emofilia. 823 21

Hemophilic patients may present immunological dysfunctions resulting from either human immunodeficiency virus (HIV) infection, or other factors like impure factor VIII concentrate and other viral infections. We evaluated prospectively the serologic response to polio vaccination of Israeli hemophilic patients who were vaccinated during an outbreak of poliomyelitis. Eighty-two hemophilic patients, 43 seronegative and 39 seropositive for human immunodeficiency virus (HIV), were vaccinated with enhanced inactivated poliovirus (eIPV). Titers of antibodies for poliovirus types 1-3 were determined before and 4 weeks after immunization. T helper and suppressor lymphocytes (T4 and T8), B and T lymphocyte mitogenic response, and natural killer cells were tested and correlated with the response to vaccination. Both groups responded to vaccination with increased titers of antibodies to the three viral types, 4 weeks after immunization. HIV-seronegative patients, however, exhibited higher titers than the HIV-seropositive group. The same pattern was found when 21 patients were tested 1 year after the exposure to eIPV. HIV seropositive patients were grouped according to their T4 count (between 16/microliter and 500/microliter). There was no statistically significant difference in the response of these different groups to vaccination. No correlation was found between the response to vaccination and other immune parameters. These results suggest that asymptomatic HIV-seropositive hemophilic patients respond well to eIPV, irrespective of their T4 count.
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PMID:Response of hemophilic patients to poliovirus vaccination: correlation with HIV serology and with immunological parameters. 836 Jun 37

To evaluate the effects of human immunodeficiency virus type 1 (HIV-1) infection on the loss of factor VIII alloantibodies, we identified 77 patients with a history of inhibitors from among a large cohort of HIV-1-infected participants enrolled in a natural history study of HIV-1 infection in hemophilia. Fifty-six patients were high responders with inhibitors titers greater than 5 Bethesda Units (BU) measured on at least one occasion. From May 1985 to December 1989, 13 of the high-responder patients were rechallenged with factor VIII concentrates after several years of treatment with other plasma products. All exhibited excellent hemostasis upon reinstitution of factor VIII. Seven of the 13 patients (11.3-46.3 years of age) were in the advanced stages of HIV-1 infection at the time of rechallenge. Inhibitor titers measured subsequent to the reinstitution of factor VIII were consistently less than 1 BU in five of these seven patients. The remaining six patients (6.1-57.5 years of age) had mild to moderate CD4+ lymphocyte depletion (absolute CD4+ cells: 262-935/mm3) at the time of factor VIII rechallenge. Follow-up inhibitor titers were negative 7-42 months after consistent factor VIII use in these six patients. The lack of anamnestic response to factor VIII in all 13 patients who were rechallenged indicates that HIV-1-infected patients who have a history of high-responder inhibitors frequently benefit from the reintroduction of factor VIII use for the control of bleeding, regardless of their stage of HIV-1 disease.
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PMID:Loss of high-responder inhibitors in patients with severe hemophilia A and human immunodeficiency virus type 1 infection: a report from the Multi-Center Hemophilia Cohort Study. 849 88

Viral RNA was extracted from plasma samples collected from five individuals during the period of viremia before seroconversion in primary infection with human immunodeficiency virus type 1 (HIV-1) and amplified by polymerase chain reaction. Nucleotide sequence analysis of amplified DNA from the V3 and V4 hypervariable regions indicated that the initial virus population of each acutely infected individual was completely homogeneous in sequence. No intrasample variability was found among the 44,090 nucleotides sequenced in this region of env, contrasting with the high degree of variability normally found in seropositive individuals. Paradoxically, substantial sequence variability was found in the normally high conserved gag gene (encoding p17) in most of the preseroconversion samples. The diversity of p17 sequences in samples that were homogeneous in V3 and V4 can most readily be explained by the existence of strong selection for specific env sequences either upon transmission or in the interval between exposure and seroconversion in the exposed individual. Evidence that localizes the selected region upon transmission to V3 is provided by the similarity or identity of V3 loop sequences in five individuals with epidemiologically unrelated HIV-1 infections, while regions flanking the V3 loop and the V4 hypervariable region were highly divergent. The actual V3 sequences were similar to those associated with macrophage tropism in primary isolates of HIV, irrespective of whether infection was acquired by sexual contact or parenterally through transfusion of contaminated factor VIII. Proviral DNA sequences in peripheral blood mononuclear cells remained homogeneous in the V3 and V4 regions (and variable in p17gag) for several months after seroconversion. The persistence of HIV sequences in peripheral blood mononuclear cells identical to those found at primary infection in the absence of continued virus expression provides an explanation for the previously observed differences in the composition of circulating DNA and RNA populations in sequential samples from seropositive individuals.
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PMID:Selection for specific sequences in the external envelope protein of human immunodeficiency virus type 1 upon primary infection. 849 55

The inactivation of both transfusion-relevant and model viruses by modified pasteurisation (10 hours at 63 degrees C in solution) has been evaluated following the established guidelines of the EU CPMP Ad Hoc Working Party on Biotechnology/Pharmacy. This heat treatment was introduced into the manufacturing process of OCTAVI, a very high purity factor VIII concentrate stabilized only by von Willebrand factor, in the presence of a proprietary mixture of low molecular weight stabilizers. Both enveloped (human immunodeficiency virus, Sindbis virus, herpes simplex virus, pseudorabies virus) and nonenveloped viruses (poliovirus, Coxsackievirus, hepatitis A virus) were inactivated by this heating step by more than 4.7 log10. The combination of the solvent/detergent step used in the manufacture of OCTAVI with this modified pasteurization leads to a double virus-inactivated factor VIII concentrate (OCTATE) with a viral safety distinctly superior to monoinactivated products.
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PMID:Viral safety of a new highly purified factor VIII (OCTATE). 869 69

In human immunodeficiency virus 1 (HIV-1)-infected patients, a hypoperfusion is seen by SPECT analyses in different brain regions but a specific pattern for the predominance of a specific brain region has not been found. The vessels of the cerebral cortex of the frontal, temporal, parietal, and occipital lobes of acquired immunodeficiency syndrome (AIDS) brains and control brains were analyzed by immunohistochemistry and lectin histochemistry. Immunohistochemistry was performed for collagen IV, laminin (basal lamina), and factor VIII (endothelial cell) and lectin histochemistry [Ricinus communis agglutinin (RCA-I), Ulex europaeus agglutinin (UEA-I), wheatgerm agglutinin (WGA) and soybean agglutinin (SBA)] was used to study changes of glycoproteins in the endothelial cell membrane. Vessels were counted in the gray and white matter, and their staining intensity for the different antibodies and lectins was rated using a three-point scale. Immunoreactivity for collagen IV was reduced in AIDS brains, which may be related to thinning of the basal lamina of cerebral vessels, as has previously been shown by electron microscopy. Lectin histochemistry with SBA, UEA-I and WGA indicated loss of glycoproteins in the membrane of endothelial cells. The data from the present study show morphological changes of the endothelial cells and of the basal lamina in the brain of individuals with AIDS, and might represent the morphological sequelae of a disturbed blood-brain barrier, or may account for the hypoperfusion seen in SPECT analyses.
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PMID:Vascular changes in the cerebral cortex in HIV-1 infection. II. An immunohistochemical and lectinhistochemical investigation. 881 Nov 23

Patients with primary pulmonary hypertension develop vascular lesions characterized by proliferated blood channels, the so-called plexiform lesions. These lesions are often associated with concentric intimal obliteration of pulmonary vessels. We report that the lungs of three patients with scleroderma-associated pulmonary hypertension showed a predominance of obliterative-concentric lesions, with relatively few plexiform or combined lesions. In contrast, plexiform lesions predominated in the lungs obtained from three patients with human immunodeficiency virus (HIV)-associated pulmonary hypertension; pure obliterative-concentric lesions were infrequent. Both plexiform and concentric obliterative lesions stained strongly positive for the endothelial cell marker factor VIII-related antigen. Muscle-specific actin immunostaining highlighted the smooth muscle cells of the tunica media of plexiform vessels, but not the luminal layers of the concentric-obliterative lesions. Proliferating cells, as determined by immunostaining with the MIB-1 antibody, were only detected in the plexiform vascular lesions. We postulate that concentric-obliterative lesions and plexiform lesions are temporally and etiologically related. A scaffolding of proliferating endothelial cells could be the common denominator of both lesions. Our hypothesis that there exists a chronological continuum, proceeding from early, proliferative plexiform lesions to late, nonproliferative concentric-obliterative lesions in primary and secondary pulmonary hypertension, may lead to better targeted treatment strategies and disease classification.
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PMID:Pathogenesis and evolution of plexiform lesions in pulmonary hypertension associated with scleroderma and human immunodeficiency virus infection. 910 43

Recent guidelines published by the United Kingdom Haemophilia Centre Directors' Organisation state that recombinant factor VIII constitutes the treatment of choice for all patients with haemophilia A. However, certain categories may have to be accorded priority as financial resources are limited. The general order of priority for the introduction of recombinant factor VIII is (1) previously untreated patients, (2) human immunodeficiency virus (HIV)- and hepatitis C-negative patients, (3) HIV-negative, hepatitis C-positive patients and (4) patients seropositive for anti-HIV.
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PMID:Who should receive recombinant factor VIII? 935 33


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