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)

This report underlines the occurrence of multiple early and late complications after allogeneic BMT. Most of these are caused by the conditioning regimen, and especially by the use of total body irradiation. This should discourage the use of radiation for patients with non-malignant disorders such as aplastic anemia. We have shown that interstitial pneumonia is greatly reduced after fractionated TBI, and this should also be considered when designing transplant protocols. Prolonged immunodeficiency post-BMT is responsible for a high rate of infections: this suggests that long-term prophylactic antibiotic therapy should be considered. Great attention should be given to the quality of life of long-term survivors: to this respect a specific program for monitoring and treating gonadal complication can be extremely useful.
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PMID:Late complications of allogeneic bone marrow transplantation. 182 Apr 92

Estimates show that 5 million people worldwide are infected with human immunodeficiency virus (HIV). Recent estimates are that 8-10 million new tuberculosis (TB) cases occur each year in the world. 2-3 million die. In developing countries, TB is one of the most common opportunistic infections in people who are seropositive for HIV-1. About 90% of the TB is pulmonary. Of those without pulmonary tuberculosis, 85% had lymphadenopathy, bone and joint disease, or pleural effusion. In adults and children over 15 who had pulmonary TB, 78% had positive sputum smears for acid-fast bacilli. 66% had cavitation on chest radiography. Many people with TB and HIV infection have typical clinical and radiologic features. However, African clinicians have seen a change. This makes TB harder to diagnose. In Bangui, Central African Republic, 30% of pulmonary TB patients were HIV seropositive. Studies from Zaire and Zambia also had patients with suspected TB and extrapulmonary TB with higher HIV seropositivity rates than those with sputum-positive TB. Haitians show a similar disease pattern to that of Africa. 70% of people with tuberculosis and the acquired immunodeficiency syndrome (AIDS) had extrapulmonary disease compared with 20% of the HIV-negative people with TB. Chemotherapy of TB in Africans who also have HIV infection is not certain. Clinical impressions suggest that the disease responds well to the usual therapy. However, a Central African study found that mortality 12 months after the start of the usual drug therapy was 32.5% in HIV-seropositives compared with 1.5% in HIV-seronegatives. Several countries in Africa use short-course drug therapy for smear-positive pulmonary TB. They use the usual regime for smear-negative and extrapulmonary TB. Since AIDS, there are more skin allergic reactions to the usual drug therapy. There are more severe reactions now. Thiacetazone is the drug which probably causes this reaction. BCG immunization is used to control TB in Africa. World Health Organization guidelines are to withhold BCG from HIV- seropositive people with symptoms. What about infants born to HIV- seropositive mothers?
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PMID:Tuberculosis and human immunodeficiency virus infection in developing countries. 197 Jan 1

Severe cutaneous hypersensitivity historically has been an extremely rare complication of antituberculous chemotherapy in African patients. However, the authors have observed 6 such cases in the past year alone in Malawi. In 5 of these cases, patients with sputum-negative pulmonary tuberculosis who were treated with streptomycin, isoniazid, and thiacetazone developed severe Stevens-Johnson syndrome in association with high fever in the third or fourth week of chemotherapy. The 6th patient with Stevens-Johnson syndrome in association with high fever in the third or fourth week of chemotherapy. The 6th patient with Stevens- Johnson syndrome was diagnosed with tuberculous pleural effusion. All 6 patients had concomitant human immunodeficiency virus (HIV) infection. It is unknown how HIV infection apparently promotes severe cutaneous side effects in antituberculous chemotherapy recipients. Thiacetazone is considered to be the agency responsible for this side effect, and use of the more expensive streptomycin, rifampicin, isoniazid, and pyrazinamide regimen is recommended. Given the association between HIV infection and a hypersensitivity reaction, all patients who developed Stevens-Johnson syndrome should be screened for HIV.
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PMID:Stevens-Johnson syndrome during anti-tuberculosis chemotherapy in HIV-seropositive patients: report on six cases. 206 Apr 84

Wiskott-Aldrich Syndrome (WAS) is a sex-linked disease characterized by immunodeficiency and thrombocytopenia. Supportive treatment of this disease is inadequate and bone marrow transplantation has been reported to result in excellent survival. The long-term follow-up of 8 male patients who received bone marrow transplantation for the WAS is reported here. All of these patients received ablative preparative treatment consisting of ATS (antithymocyte serum), cytoxan and either busulfan or TBI (total body irradiation). Bone marrow was transplanted from an HLA-matched donor. Seven of eight of these male patients have had excellent engraftment of their transplant and now have adequate lymphocyte and platelet function. In addition, they have had good growth and development. This suggests that ablative preparative treatment followed by early bone marrow transplantation from an HLA-matched donor is a highly successful therapy for this congenital disease.
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PMID:Bone marrow transplantation for the Wiskott-Aldrich syndrome. Long-term follow-up. 221 85

Tuberculosis in patients infected with human immunodeficiency virus (HIV) is a growing threat to public health in Africa. Thiacetazone, one of the continent's most widely used antituberculous agents, may lead to severe cutaneous reactions in the HIV infected individual. We describe the impact of this reaction on the tuberculosis (TB) control programme of a district hospital in Zambia in 1990, and examine the cost implications of changing the standard treatment regime. We carried out a retrospective survey of records of all patients beginning TB treatment in 1990, together with HIV test results and the cost of all treatments given. From this we derived estimates of costs of different regimes which are and could be used in TB control in Zambia. Severe reactions occurred in 18.7% of all HIV seropositive patients receiving thiacetazone, fatally so in 1.2% (odds ratio 16.6). The greatest part of the cost of the current regime is that attributable to the inpatient stay; we estimated that 29.4% of patients would be unable to receive drugs as out-patients but, even allowing for this, rifampicin-based regimes given to outpatients where possible would not cost more than the current strategy. We conclude that ethical and economic considerations support a change to rifampicin-based regimes in areas of Africa where HIV seroprevalence is high.
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PMID:Cutaneous reactions to thiacetazone in Zambia--implications for tuberculosis treatment strategies. 753 55

To be efficient, a synthetic vaccine should contain different T and B cell epitopes of human immunodeficiency virus (HIV) antigens, and the B epitope regions in the vaccine and in the HIV should be conformationally similar. We have suggested previously the construction of vaccines in the form of a protein with a predetermined tertiary structure, namely a four-alpha-helix bundle. Antigenic determinants of cellular and humoral immunity are blocks for the vaccine design. From experimentally studied HIV-1 T and B cell epitopes, we constructed a sequence of a four-helix protein TBI (T and B cell epitopes containing immunogen). The gene of the protein was synthesized and the protein was produced in C600 Escherichia coli cells under recA promoter from Proteus mirabelis. CD spectroscopy of the protein demonstrated that 30% of amino acid residues adopt an alpha-helical conformation. Mice immunized with TBI have shown both humoral and cellular immune responses to HIV-1. The obtained data show that the design of TBI was successful. The synthesized gene structure makes possible further reconstruction and improvement of the protein vaccine structure.
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PMID:Design of four-helix bundle protein as a candidate for HIV vaccine. 754 4

Allogeneic haematopoietic stem cell transplantation (HSCT) can be a highly successful treatment option for individuals with congenital immunodeficiency states. The strategy for HSCT is varied but in cases where there is preservation of residual T cell function, conditioning regimes have been used and have been based around a combination of busulphan and cyclophosphamide with or without serotherapy. In patients with coexisting organ damage this has resulted in significant morbidity and mortality. We have therefore used a low-intensity conditioning regime for transplantation in this group of immunodeficiency patients. Twenty-one patients with a variety of different immunodeficiencies were treated using the following conditioning regimes: (1) fludarabine/melphalan/ATG or Campath 1H (n=16), (2) fludarabine/cyclophosphamide/Campath 1H (n=1), (3) TBI/CyA/MMF (n=1), (4) fludarabine/melphalan/busulphan/ATG (n=3). In 13 cases matched (n=9) and 1 Ag mismatched (n=4) unrelated donors were used and in eight cases transplants from matched siblings (n=4), 1 Ag mismatched sibling (n=1), matched parent (n=1) and haploidentical parents (n=3) were performed. At a median follow-up of 13 months, 19 of 21 (90%) patients were still alive following the transplant procedure. Despite a T cell replete graft and the use of unrelated donor grafts in the majority of patients studied there was no evidence of significant organ disease. Immune reconstitution in terms of CD3+ and CD4+ T cell recovery and function was equivalent in comparison with a historical cohort. We believe that this low-intensity approach has significant implications for transplantation of individuals with immunodeficiency states with established organ disease.
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PMID:Non-myeloablative stem cell transplantation for congenital immunodeficiencies. 1178 37

Tuberculosis (TB) flourishes where there is poverty, malnutrition, overcrowding, and deficient health care. Worldwide, 1 billion 1,722 people are infected with dormant TB. 9-11 million people have active TB predominately in Asia, Africa, and Latin America, and almost 3 million people die of TB annually including 1/2 million of children. WHO estimates that 4.5 million people are coinfected with the human immunodeficiency virus (HIV) and TB in whom active TB can flare up because of weakened immunity. In Uganda, confirmed cases of active TB doubled between 1984 and 1987; and in Zambia TB cases increased from 7000 in 1986 to 17,000 in 1990. Under an assumption of low risk of TB infection and low HIV prevalence scientists have projected that active TB cases in the 15-49 age group will rise by 2/3 by the year 2000. Under a worst case scenario of higher TB infection and higher HIV seroprevalence rates, a 12-fold increase of active TB cases is projected by 2000. Thiacetazone is the main antituberculosis drug in standard chemotherapy. According to WHO data TB has been on the rise in 9 out of 14 European countries with TB infection rates of 10% among HIV-infected people in Spain and Italy. Since 1986 the TB caseload, also has been increasing in the US, and the recent appearance of multi-drug resistant TB (MDR-TB) has raised alarm. MOR-TB almost exclusively infants people with HIV and AIDS with a mortality rate of 80%. The Centers for Disease Control in Atlanta, Georgia, advocates the drastic measure of court-ordered, involuntary detention for treatment to halt its spread.
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PMID:A dangerous liaison: tuberculosis and HIV. 1228 82

We describe the treatment of a 10-year-old girl with autosomal recessive Dyskeratosis congenita (DC), neutropenia, thrombocytopenia and combined immunodeficiency by nonmyeloablative hematopoietic stem cell transplantation. The conditioning regimen consisted of fludarabine 30 mg/m(2)/day (days -5, -4, -3) and 2 Gy TBI (0.07 Gy/min; day 0). For graft-versus-host disease (GVHD) prophylaxis a course of intravenous MMF and CSA was administered. At 2 years after transplantation of granulocyte colony-stimulating factor (G-CSF) mobilized peripheral blood stem cells from a healthy 11-year-old HLA-identical brother, peripheral blood counts and T- and B-cell functions have completely normalized and donor chimerism was 100% in all cell lineages. No GVHD occurred. Neurological examination and lung function remained normal. The current transplantation regimen appears suitable, safe and efficacious in patients with DC.
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PMID:Nonmyeloablative allogeneic hematopoietic stem cell transplantation for treatment of Dyskeratosis congenita. 1263 34

We compared the results of Tc-99 evaluation of glomerular filtration rate (GFR) vs. the calculation of the creatinine clearance (CCrC) as a predictor for the development of renal insufficiency in pediatric patients following hematopoietic stem cell transplantation (HSCT). We reviewed 95 consecutive patients receiving autologous (n = 37) or allogeneic (n = 58) HSCT at Children's Memorial Hospital between January, 1995 and February, 1998. Diagnoses included leukemia (n = 43), solid tumor (n = 27), bone marrow failure syndrome (n = 12), non-malignant disease (n = 8), CNS tumors (n = 5) and immunodeficiency (n = 3). Tc-99 GFR was compared with a calculated creatinine clearance derived from the Schwartz formula (CCrC) prior to HSCT. These measures of renal function were compared with the patient's subsequent clinical course to determine if patients who developed renal insufficiency of sufficient magnitude as to require continuous veno-venous hemofiltration (CVVH) or dialysis, could have been identified. Overall comparison of the two methods of evaluation of renal function showed low correlation with values obtained by CCrC, which were consistently higher in most patients (r-value 0.01 in the regression analysis and a p = 0.08 95% CI -24.15 to 1.48). When stratified for age, correlation between the two methods was excellent only in children younger than 5 yr of age p = 0.02 95%, CI 0.032-0.49). Eleven patients required therapy with CVVH or dialysis but neither CCrC nor Tc-99 GFR prior to transplant predicted this event. Patients who received TBI were statistically more prone to develop renal insufficiency than those without TBI (p < 0.0001, 95% CI 0.25-0.008). Neither the Tc-99 GFR nor the CCrC was predictive of the development of renal insufficiency in HSCT patients as the majority of patients who required dialysis had normal Tc-99 GFR prior to transplant. The characteristics found in the patients who developed renal insufficiency and required dialysis include: the use of total body irradiation as part of the transplant-conditioning regimen (p < 0.0001) and the use of continuous infusion CSA (p = 0.04).
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PMID:Comparison of Tc-99 measurement of glomerular filtration rate vs. calculated creatinine clearance to assess renal function pretransplant in pediatric patients undergoing hematopoietic stem cell transplantation. 1617 14


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