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)

Recently it has become urgent to establish a risk control system against emerging and re-emerging infectious diseases. Many of the emerging and re-emerging infectious diseases such as AIDS and viral hepatitis, are induced by viral infection via blood. The main causative agents of blood-born viral infection are hepatitis B virus (HBV), hepatitis C virus (HCV), human immunodeficiency virus (HIV), human T cell leukemia virus type1 (HTLV1), cytomegalovirus (CMV), Epstein-Barr virus (EBV), and human parvovirus B19. They play the main role in viral hospital infections. The risk of them being transmitted by the transfusion of screened blood is very low, but it is always possible that infection may occur in a window period even after extensive blood screening tests. EBV has been recognized as a less serious infectious agent than CMV. Nowadays, biotechnology has revealed the broad spectrum of EBV related diseases as chronic active EBV infection, compromised lymphoma, gastric carcinoma and other lymphoproliferative disorders. There would be some immunocompromised cases needed monitoring after transfusion/transplantation as same as CMV infection. Double infection or co-infection of EBV and CMV are shown to be occasional. The most important tasks for risk control of hospital acquired infectious diseases are to prevent second drug related AIDS and prion infection due to the transplantation of dura mata derived from patients with Creutzfeldt-Jakob disease, and to prevent of needle stick infections. Therefore it is necessary to establish a network communication between clinical laboratories, institutes and public health organizations for more rapid and adequate care with rapid diagnosis by molecular analysis.
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PMID:[The blood-born viral infections]. 952 33

Haemophilia A is a bleeding disorder that affects approximately 1 in 10,000 males. It is caused by a deficiency of functional blood-clotting factor VIII. Protein-replacement therapy has been effective as treatment, resulting in a vast improvement in the quality of life and dramatically increasing the life expectancy of patients. However, therapy with plasma-derived factor VIII has allowed the transmission of several human viruses, such as hepatitis viruses, human immunodeficiency virus and parvovirus B19. To date, the safety of the therapeutic agent is one of the key issues in haemophilia A treatment. The use of recombinant factor VIII in haemophilia therapy can avoid the dependence on blood-derived products and prevent the occurrence of transfusion-associated infections with blood-borne pathogens. Gene therapy could go further, and offers the prospect of one-time treatment which may, optimally, achieve a total cure of the disease. Therefore, haemophilia is an appealing and challenging target for somatic-cell gene therapy. On the basis of the phenotypic correction that is achieved upon infusion of factor VIII protein, it is expected that an increase in the factor VIII plasma level to 10% of the level found in healthy individuals would suffice to prevent the manifestation of the bleeding tendency. In this paper, we review the progress and the problems of gene therapy for haemophilia, with special focus on the problems specifically associated with the transfer and expression of human factor VIII complementary DNA.
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PMID:Factors impeding efficient expression of factor VIII complementary DNA minigenes. 960 9

A 61-year-old heart transplant recipient with parvovirus B19 infection, presented as a severe pure red cell aplasia (PRCA) with hemoglobin level of 5 g/dl. Both blood and bone marrow cells were positive for parvovirus B19 DNA, whereas specific immunoglobulins IgG and IgM were not informative. Bone marrow smears revealed erythroid hypoplasia without giant pronormoblasts. Autologous and allogenic bone-marrow cultures revealed a high inhibition by patient's serum on BFU-E growth whereas the number of CFU-GM were normal. Spontaneous remission of the anemia was observed despite the persistence of severe immunodeficiency as demonstrated by development of a monoclonal EBV lymphoproliferative disorder two months later. The "recovery" serum reversed the initial serum BFU-E inhibiting property. This case pinpointed the usefulness of blood or marrow cultures in parvovirus B19 infection of immunocompromised patients without normal Ig responses, as in other PRCA. Further, it argues that the usual immunoglobulin therapy may not be necessary in order to obtain a viral clearance.
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PMID:Spontaneous recovery from severe parvovirus B19 pure red cell aplasia, in a heart transplant recipient, as demonstrated by marrow culture. 961 50

Discoveries of new human viruses and new technologies for their detection have made, and will continue to make, major contributions to the safety of blood transfusion. This article discusses the practical issues involved in the implementation of additional serological screening tests for viruses such as human T-lymphotropic virus, and reviews current information on the prevalence and pathogenicity of more recently discovered viruses, such as hepatitis G virus (HGV) or GB virus-C (GBV-C) and human herpes virus 8, a potential aetiological agent of Kaposi's sarcoma. Progress in the technology behind nucleic acid amplification techniques, such as the polymerase chain reaction (PCR), makes direct detection of viruses such as human immunodeficiency virus and hepatitis C virus possible. The use of such methods for screening will allow the earlier detection of acutely-infected individuals and the elimination of transmission from 'window' period donations before seroconversion for antibody. Establishing a framework for PCR-based screening would also enable the testing for others such as hepatitis A virus, parvovirus B19 and GBV-C/HGV for which serological detection methods cannot be or have not been developed.
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PMID:Transfusion virology: progress and challenges. 974 87

Despite careful donor selection and virus inactivation procedures, transmission of viruses by transfusion of blood and blood derivatives is still a threat. Outbreaks of hepatitis A among hemophiliacs having received highly purified, immune globulin depleted coagulation factor concentrates, put the importance of immune neutralization of viruses in blood derivatives in focus. Neutralizing antibodies may block several steps in the virus infection of a cell, from binding of virus to the cellular receptor to the uncoating of virus after uptake in the cell. The efficacy of antibody neutralizing activity depends on the availability and stability of the neutralizing epitopes. Hepatitis A and B viruses are very efficiently neutralized by antibodies and immune escape mutants rarely emerge. Anti-parvovirus B19 antibodies do not fully inactivate the virus, at least in low concentrations, but may prevent development of disease. The neutralizing epitopes on hepatitis C virus and human immunodeficiency virus are located on hypervariable regions of virus membrane proteins. The effects of neutralizing antibodies are thus marginal as immune escape mutants emerge at a relatively high frequency for both viruses. The neutralizing activity of anti-cytomegalovirus antibodies is also questionable as persons may become reinfected with cytomegolvirus despite high levels of antibodies. Plasma and plasma derivatives produced from large donor pools have the potential of being very efficient transmitters of viruses. Neutralizing antibodies are Nature's own, and very important barriers against the spread of many known and unknown viruses contaminating the plasma pools.
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PMID:Viral safety of blood derivatives by immune neutralization. 978 31

Infectious agents, including viruses, bacteria, and parasites, can be transmitted by human blood products. Of major importance are viruses such as human immunodeficiency virus types 1 and 2 (HIV-1/2), hepatitis B virus (HBV), hepatitis C virus (HCV), and human T-cell lymphotropic virus types I and II (HTLV-I/II). Also, other viruses such as cytomegalovirus, Epstein-Barr virus, human parvovirus B19, and hepatitis A and G viruses can be transmitted by blood products. Various methods are used to prevent transmission of blood-borne agents to recipients, such as donor selection, testing donated blood for various infectious agents, and viral inactivation of plasma derivatives. With all these precautionary measures, the estimated risk for infection by screened blood components in Europe and the United States is approximately 1 in 50,000 to 1.6 million (for HBV, HCV, and HIV-1/2) transfused blood components. In the future, the safety of blood components may be increased by testing donated blood by nucleic acid amplification techniques and by photochemical decontamination of cellular blood components.
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PMID:Transfusion-transmissible infections. 981 46

Gianotti-Crosti syndrome (GCS), a self-limiting papulovesicular acrodermatitis often associated with underlying viral infection, is mainly described in children. Nine children with GCS were evaluated with dermatologic examination and serologic tests for viral infections. Therapy was modified according to the subjective symptoms of patients, which included characteristic acrolocated papulovesicles, generalized skin eruption, and mild to severe pruritus. Results of serologic investigations revealed Epstein-Barr virus, Coxsackie A virus, parvovirus B19, and parainfluenza virus 1/2. In three children no underlying viral infection was found. Therapeutic interventions included topical clioquinol lotion 1 percent, topical application of corticosteroids, systemic antihistaminic therapy, and systemic methylprednisolone. Skin lesions resolved after 2 to 4 weeks in treated as well as in nontreated children. Although GCS in children often lacks close association with a causative viral infection, such severe infections as hepatitis B and human immunodeficiency virus must be considered. Whole-body involvement seems to correlate with severe pruritus and additional general symptoms requiring more intensive therapy.
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PMID:Gianotti-Crosti syndrome: clinical, serologic, and therapeutic data from nine children. 987 81

During the past decades major improvements in blood safety have been achieved, both in developed and developing countries. The introduction of donor counseling and screening for different pathogens has made blood a very safe product, especially in developed countries. However, even in these countries, there is still a residual risk for the transmission of several pathogens. For viruses such as the human immunodeficiency virus (HIV), and the hepatitis viruses B and C, this is due mainly to window-period donations. Furthermore, the threat of newly emerging pathogens which can affect blood safety is always present. For example, the implications of the agent causing new variant Creutzfeld-Jakob disease for transfusion practice are not yet clear. Finally, there are several pathogens, e.g. CMV and parvo B19, which are common in the general donor population, and might pose a serious threat in selected groups of immunosuppressed patients. In the future, further improvements in blood safety are expected from the introduction of polymerase chain reaction for testing and from the implementation of photochemical decontamination for cellular blood products. The situation in transfusion medicine in the developing world is much less favorable, due mainly to a higher incidence and prevalence of infectious diseases.
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PMID:Transfusion-transmitted diseases: risks, prevention and perspectives. 991 6

A human immunodeficiency virus (HIV)-infected individual was first diagnosed with red blood cell aplasia due to B19 parvovirus infection in late 1989. Over the subsequent seven-year period, he received a total of 119 units of red blood cells (RBCs) and intravenous immunoglobulin every 2-3 weeks. In 1996 combination antiretroviral treatment with a protease inhibitor was initiated. He received four more units during the following two months and then required no more transfusions for the subsequent 24 months of follow-up. His CD4 count progressively increased and DNA polymerase chain reaction for parvovirus B19 became undetectable. Aggressive antiretroviral treatment may effectively diminish transfusion requirements among HIV-infected individuals with pure RBC aplasia resulting from parvovirus B19 infection.
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PMID:Persistent parvovirus B19 related anemia of seven years' duration in an HIV-infected patient: complete remission associated with highly active antiretroviral therapy. 992 13

Infections caused by human parvovirus B19 are known to be controlled mainly by neutralizing antibodies. To analyze the immune reaction against parvovirus B19 proteins, four cell lines secreting human immunoglobulin G monoclonal antibodies (MAbs) were generated from two healthy donors and one human immunodeficiency virus type 1-seropositive individual with high serum titers against parvovirus. One MAb is specific for nonstructural protein NS1 (MAb 1424), two MAbs are specific for the unique region of minor capsid protein VP1 (MAbs 1418-1 and 1418-16), and one MAb is directed to major capsid protein VP2 (MAb 860-55D). Two MAbs, 1418-1 and 1418-16, which were generated from the same individual have identity in the cDNA sequences encoding the variable domains, with the exception of four base pairs resulting in only one amino acid change in the light chain. The NS1- and VP1-specific MAbs interact with linear epitopes, whereas the recognized epitope in VP2 is conformational. The MAbs specific for the structural proteins display strong virus-neutralizing activity. The VP1- and VP2-specific MAbs have the capacity to neutralize 50% of infectious parvovirus B19 in vitro at 0.08 and 0.73 microgram/ml, respectively, demonstrating the importance of such antibodies in the clearance of B19 viremia. The NS1-specific MAb mediated weak neutralizing activity and required 47.7 micrograms/ml for 50% neutralization. The human MAbs with potent neutralizing activity could be used for immunotherapy of chronically B19 virus-infected individuals and acutely infected pregnant women. Furthermore, the knowledge gained regarding epitopes which induce strongly neutralizing antibodies may be important for vaccine development.
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PMID:Generation of neutralizing human monoclonal antibodies against parvovirus B19 proteins. 997 77


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