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)

Parvovirus B19 infection is associated with a wide variety of symptoms and signs, and given that some clinical features, such as anemia, arthropathy and rash may be attributable to other causes, laboratory diagnosis of B19 markers is necessary. The principal aims were to study the behavior of B19 infection-associated diseases in the Chilean population and to compare B19 markers for recent or active infection and for immunity status in patients with clinical symptoms suspicious of B19 infection and control individuals. Sera from a total of 267 patients with diverse clinical manifestations associated with B19 and from 69 healthy controls were tested for B19 DNA using PCR and for specific IgM and immunoglobulin G (IgG) by enzyme-linked immunosorbent assay (ELISA). Out of 267 patients examined, 89 had B19-associated disease markers: 43 had B19 DNA without IgM, 25 had IgM without B19 DNA, and 21 had both B19 DNA and IgM. Also 49 patients were positive only for IgG without B19 DNA or IgM. Out of the 69 healthy controls, only 2 had B19 DNA without IgM and 30 had IgG without B19 DNA and/or IgM. The distribution of the clinical diagnoses associated with recent B19 infection, tested by B19 DNA and/or IgM, included 38.5% with hematological illnesses, 23.4% with rheumatic diseases, 45.7% with infectious diseases, 33.3% with indications of prenatal infection, 32.3% with conditions that induce immunodeficiency, and 15.8% with other miscellaneous conditions. The use of both markers, DNA and IgM, allows a more adequate diagnosis of infection by this virus.
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PMID:Parvovirus B19 infection in Chile: markers of infection and immunity in patients with clinical symptoms. 1793 73

Unselected intramuscular (IM) and intravenous (IV) immunoglobulins, as well as virus-specific hyperimmune globulins, occupy important roles as immunotherapy for viral infections. Standard IM immunoglobulins may be utilised in selected, susceptible patients for the prevention of hepatitis A and measles. Hyperimmune globulins to varicella zoster virus (VZV), hepatitis B virus and rabies have established indications for use as post-exposure prophylaxis. Cytomegalovirus (CMV) hyperimmune globulin has an indication for the prevention of primary CMV-associated disease in kidney transplantation and has been shown to decrease severe CMV-associated disease in liver transplantation. More recently, respiratory syncytial virus (RSV) hyperimmune globulin has been developed and is being utilised to prevent RSV disease in high risk infants and children during months of maximum risk for RSV infection. Unselected IV immunoglobulins (IVIg) have proven beneficial in preventing CMV-associated disease and graft-versus-host-disease in allogeneic bone marrow transplant recipients. In addition, IVIg plus ganciclovir is effective therapy for established CMV disease in both bone marrow and solid organ transplantation. IVIg for chronic anaemia associated with parvovirus B19 infection is gaining acceptance, as is the use of IVIg and intraventricular immunoglobulin for chronic meningoencephalitis associated with agammaglobulinaemia. Immunotherapy for the prevention or treatment of several other viral infections has been explored, but without clear conclusions. The use of human immunodeficiency virus (HIV) hyperimmune globulins in HIV-infected patients has yielded inconsistent results and the role of such therapy in the era of highly active antiretroviral therapy is uncertain. Oral immunoglobulins appear successful for rotaviral infections, but their exact use requires further clarification. Other immunotherapeutic modalities, such as monoclonal antibodies against CMV, RSV and HIV, have been developed but these agents have not undergone extensive clinical evaluation.
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PMID:Antiviral immunotherapy: a review of current status. 1802 May 81

Two main types of safety procedures must be applied to biological products, including plasma derivatives: (i) preventive procedures and (ii) elimination procedures. Prevention includes epidemiological control of donor populations; checks on each donor's health condition; analysis of each donation for the main pathogens using serological methods; additional analysis of all plasma for human immunodeficiency virus (HIV), hepatitis B virus (HBV), hepatitis C virus (HCV), hepatitis A virus (HAV) and the B19 virus, using nucleic acid amplification techniques (NAT). A 60 days or longer inventory hold of all plasma donations is applied, to allow additional time to discard previous donations from potential seroconverting or otherwise rejectable donors. Elimination procedures minimize the low residual risk of transmitting pathogens, including unknown or previously undetected ones. Since the introduction 20 years ago of solvent-detergent treatment, very effective against enveloped viruses (HIV, HBV, HCV, West Nile virus, SARS, avian influenza virus etc), there have been no known cases of transmission of this type of pathogens by products manufactured according to this procedure. Other inactivation procedures such as pasteurization, dry-heat or nanofiltration may prove equally effective. In addition, dry-heat treatment and nanofiltration are capable of effectively eliminating non-enveloped viruses (HAV, B19 virus). Recent studies show that the B19 virus is much more sensitive to heat (in lyophilized state or by pasteurization) and acid pH than previously thought. Although there is no evidence for the transmission of classic transmissible spongiform encephalopathies (TSEs) through blood or blood-products transfusion, four possible cases have been reported in the United Kingdom involving transmission by non-leukoreduced blood components of the agent that causes variant Creutzfeldt-Jakob Disease (vCJD), a disease linked to the outbreak of bovine spongiform encephalopathy (BSE) which took place in that country. However, there are no cases of human TSE (classic or variant) transmission by plasma-derived products. Analytical methods capable of detecting the vCJD agent in patients' brains (where high titres are found) and other tissues (such as the spleen, appendix and lymph nodes, where much lower concentrations are found) are unable to detect the agent in blood or plasma from patients with vCJD, even in the clinical phase of the disease. Experiments by Grifols and other groups show that the capacity of the production processes to eliminate vCJD agent models is many orders of magnitude greater than the maximum expected load of the agent. In this regard, the efficacy of precipitation, affinity chromatography, depth filtration and nanofiltration are particularly notable.
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PMID:Safety procedures of coagulation factors. 1807 96

The viral safety of biologicals, either human blood derivatives or animal products or recombinant proteins issued from biotechnology, relies on the quality of the starting material, the manufacturing process and, if necessary, the control of the final product. The quality of the starting material is highly guaranteed for blood derivatives due to the individual screening for specific markers (antigens, genome, antibodies) for major blood borne viruses such as hepatitis B and C viruses (HBV, HCV) and human immunodeficiency virus (HIV). It can be reinforced by the detection through amplification procedures (polymerase chain reaction) in the plasma pool of genomes from viruses that have been implicated in contaminations of blood derivatives in the past (parvovirus B19, hepatitis A virus). The association in the manufacturing process of different steps dedicated to purification of plasma proteins (partitioning), virus inactivation (solvent/detergent treatment, heat inactivation) or specific procedures allowing virus removal (nanofiltration) allows to reduce the viral risk very efficiently. The validation studies using scaled down systems and model viruses allow to evaluate the virus safety of any product quantitatively. The aim of these procedures is to guarantee the lack of infectivity due to any virus, either known or unknown.
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PMID:[Viral safety of biologicals]. 1870 41

PARV4 is a recently discovered human parvovirus widely distributed in injecting drug users in the USA and Europe, particularly in those co-infected with human immunodeficiency virus (HIV). Like parvovirus B19, PARV4 persists in previously exposed individuals. In bone marrow and lymphoid tissue, PARV4 sequences were detected in two sub-Saharan African study subjects with AIDS but without a reported history of parenteral exposure and who were uninfected with hepatitis C virus. PARV4 variants infecting these subjects were phylogenetically distinct from genotypes 1 and 2 (formerly PARV5) that were reported previously. Analysis of near-complete genome sequences demonstrated that they should be classified as a third (equidistant) PARV4 genotype. The availability of a further near-complete genome sequence of this novel genotype facilitated identification of conserved novel open reading frames embedded in the ORF2 coding sequence; one encoded a putative protein with identifiable homology to SAT proteins of members of the genus Parvovirus.
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PMID:A third genotype of the human parvovirus PARV4 in sub-Saharan Africa. 1875 40

Plasma-derived factor VIII (FVIII) and von Willebrand Factor (VWF)/FVIII concentrates have been successfully used to treat haemophilia since the late 1960s. These products are derived from pools of plasma donations that may contain viral contaminants - including hepatitis B virus (HBV), hepatitis C virus (HCV) and human immunodeficiency virus (HIV) - and may therefore present a transmission risk to recipients. To ensure the safety of Haemate P/Humate-P, a plasma-derived VWF/FVIII concentrate, donors of plasma are carefully selected and all donations are screened for viral antigens (HBV), virus-specific antibodies (HIV-1/2, HCV) and genomic material [hepatitis A virus, HBV, HCV, HIV-1 and high titres of human parvovirus B19 (B19V)]. As a quality control measure, plasma pools for fractionation are only released for further processing when non-reactivity has been demonstrated in serological and genome amplification assays. The manufacturing process for plasma-derived products, especially the fundamental procedure of pasteurization, is effective in inactivating and/or removing a wide variety of viruses that may potentially be present despite the screening process. This has been demonstrated in virus validation studies using a range of different viruses. New emerging infectious agents, including prions, which potentially pose a threat to recipients of plasma derivatives, are also the subject of safety evaluations. The multiple precautionary measures that are inherent in the overall production process of Haemate P/Humate-P have resulted in an excellent safety record, documented during 25 years of clinical use, and will help to maintain the high safety margin in the future.
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PMID:Pathogen safety of plasma-derived products - Haemate P/Humate-P. 1878 11

A spectrum of blood-borne infectious agents is transmitted through transfusion of infected blood donated by apparently healthy and asymptomatic blood donors. The diversity of infectious agents includes hepatitis B virus (HBV), hepatitis C virus (HCV), human immunodeficiency viruses (HIV-1/2), human T-cell lymphotropic viruses (HTLV-I/II), Cytomegalovirus (CMV), Parvovirus B19, West Nile Virus (WNV), Dengue virus, trypanosomiasis, malaria, and variant CJD. Several strategies are implemented to reduce the risk of transmitting these infectious agents by donor exclusion for clinical history of risk factors, screening for the serological markers of infections, and nucleic acid testing (NAT) by viral gene amplification for direct and sensitive detection of the known infectious agents. Consequently, transfusions are safer now than ever before and we have learnt how to mitigate risks of emerging infectious diseases such as West Nile, Chikungunya, and Dengue viruses.
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PMID:Transfusion-transmitted infectious diseases. 1923 Dec 36

Persistent pure red cell aplasia can be a manifestation of parvovirus B19 infection in immunocompromised hosts. Failure of the humoral immune response to clear parvovirus B19 in such patients results in persistent pure red cell aplasia. The authors describe a child who had T-cell immunodeficiency and persistent pure red cell aplasia due to parvovirus B19 infection. Interestingly, they detected human parvovirus B19 genome by polymerase chain reaction (PCR) not in the peripheral blood, but in the bone marrow specimen of the patient. In their patient, T-cell immunodeficiency may have caused impaired B-cell activation and failure of effective humoral immune response to neutralize the virus. Additionally, before the diagnosis of pure red cell aplasia, IVIG treatment given at a dosage of 400 mg/kg/day with 3-week intervals may result in sufficient neutralization of peripheral blood parvovirus B19, whereas it may not be sufficient for the neutralization of parvovirus B19 genome in bone marrow. Thus, peripheral blood parvovirus B19 serology (IgM and IgG) and PCR were negative, whereas bone marrow aspiration sample was positive for parvovirus B19 PCR in this patient. Reticulocytopenia and severe anemia may warn the physicians of parvovirus B19 infection, especially in immunocompromised children. Diagnosis may require demonstration of absence of late erythroid precursors in the bone marrow as well as serologic testing and detection of parvovirus B19 genome by PCR in the serum and/or bone marrow samples of the patient.
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PMID:Parvovirus B19-induced persistent pure red cell aplasia in a child with T-cell immunodeficiency. 1932 36

While transfusion of blood components is usually safe, there are risks of adverse effects that can have immunologic, nonimmunologic, or infectious causes. In patients, the fear of infectious disease transmission predominates, although the risk has been extremely low since the introduction of reliable serologic and molecular biological testing methods. This article addresses the incidence, clinical picture, and etiology of adverse effects of transfusion. It also reports on current knowledge concerning transfusion-associated acute lung injury, which has gained much attention in the last few years. Besides hepatitis and human immunodeficiency viruses, cytomegalovirus, parvovirus B19, prion transmission, and the risk of variant Creutzfeld-Jakob disease are also discussed.
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PMID:[Risks and side effects of blood transfusion]. 1975 92

X-linked lymphoproliferative disease (XLP) is a primary immunodeficiency affecting approximately 1 to 3 per million live male births. Patients are generally healthy until facing a viral infection such as Epstein-Barr Virus and then may develop fulminant infectious mononucleosis and die. XLP patients are also at increased risk of hemophagocytic lymphohistiocytosis (HLH), which may be triggered by assorted viruses. Here we report a novel case of HLH in a patient with XLP. Significant to his presentation is a paradoxical increase in natural killer (NK) cell activity. We hypothesize that this indicates that Parvovirus B19 activates NK cells via a signaling lymphocytic activation molecule-associated protein (SAP)-independent mechanism. Our case demonstrates an important etiology to consider in the differential diagnosis of XLP patients with nonfocal findings and febrile illnesses.
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PMID:Hemophagocytic lymphohistiocytosis in a patient with x-linked lymphoproliferative disease. 1977 67


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