Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two hundred and three multi-transfused children with thalassemia attending the Thalassemia Clinic of the Charak Palika Hospital, New Delhi were screened for human immunodeficiency virus (HIV) antibodies by ELISA and all positive cases were confirmed by Western Blot. Of the 203 children screened, 18 (8.9%) were HIV positive, and in these children a detailed immunological work up was done and compared to 18 age-matched HIV negative thalassemics as controls. The tests included absolute lymphocyte counts (ALC), absolute and percentages of CD4+ and CD8+ cells and their ratios (CD4/CD8), immunoglobulin levels (IgG, IgM and IgA) and delayed cutaneous hypersensitivity (DCH) test by Multitest CMI in all the cases and the controls. Of the 18 HIV positive children, 6 were diagnosed to have clinical AIDS as per the WHO criteria. After immunological testing, the children were further classified according to the CDC criteria. By these criteria, 11 children were classified as P1 A (asymptomatic infection, normal immune function), 1 child as P1 B (asymptomatic infection, abnormal immune function), 2 children as P2 A (symptomatic infection with non-specific findings), 1 child as P2 C (lymphocytic interstitial pneumonitis), 1 child as P2 D1 (Pneumocystis carinii pneumonia) and 2 children as P2 D2 (symptomatic infection with infections). In this paper, the clinical features of the children with AIDS is described, and the immunologic functions of these children are compared with the HIV positive asymptomatic children and with controls. These are the first cases of AIDS in the pediatric age group from India.
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PMID:Acquired immunodeficiency syndrome (AIDS) in multitransfused children with thalassemia. 828 25

A retrospective survey was conducted over a 10-year period (1990-99) among 52 haematology divisions in order to evaluate the clinical and laboratory characteristics and outcome of patients with proven Pneumocystis carinii pneumonia (PCP) complicating haematological diseases. The study included 55 patients (18 with non-Hodgkin's lymphoma, 10 with acute lymphoblastic leukaemia, eight with acute myeloid leukaemia, five with chronic myeloid leukaemia, four with chronic lymphocytic leukaemia, four with multiple myeloma, three with myelodysplastic syndrome, two with myelofibrosis and one with thalassemia) who developed PCP. Among these, 18 (33%) underwent stem cell transplantation; only two received an oral prophylaxis with trimethroprim/sulphamethoxazole. Twelve patients (22%) developed PCP despite protective isolation in a laminar airflow room. The most frequent symptoms were: fever (86%), dyspnoea (78%), non-productive cough (71%), thoracic pain (14%) and chills (5%); a severe hypoxaemia was present in 39 patients (71%). Chest radiography or computerized tomography showed interstitial infiltrates in 34 patients (62%), alveolar infiltrates in 12 patients (22%), and alveolar-interstitial infiltrates in nine patients (16%). Bronchoalveolar lavage was diagnostic in 47/48 patients, induced sputum in 9/18 patients and lung biopsy in 3/8 patients. The diagnosis was made in two patients at autopsy. All patients except one started a specific treatment (52 patients trimethroprim/sulphamethoxazole, one pentamidine and one dapsone). Sixteen patients (29%) died of PCP within 30 d of diagnosis. Multivariate analysis showed that prolonged steroid treatment (P < 0.006) and a radiological picture of diffuse lung involvement (P < 0.003) were negative diagnostic factors.
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PMID:Pneumocystis carinii pneumonia in patients with malignant haematological diseases: 10 years' experience of infection in GIMEMA centres. 1197 21