Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In order to determine the incidence and causes of death during the first 100 days after BMT (early deaths) in a pediatric population we have examined data reported in the AIEOP BMT Registry. Up to July 1990, data on 486 children who underwent allogeneic (180) or autologous (306) BMT were evaluable. The children had acute lymphoblastic leukemia (148 cases), acute non-lymphoblastic leukemia (127 cases), neuroblastoma (82 cases), chronic myelogenous leukemia (15 cases), aplastic anemia (nine cases), solid tumors, lymphoma, immunodeficiency or storage diseases. The overall survival is 55% for allogeneic HLA matched and 38% for autologous transplants at 5 years, 24% for HLA mismatched graft at 2 years. Out of the 486 children, 70 (14%) died during the first 100 days after BMT: 33/306 (11%) after autologous BMT, 24/150 (16%) after allogeneic matched BMT and 13/30 (43%) after mismatched BMT. Causes of early death were as follows: disease progression: 12 children (10/306 after autologous and 2/180 after allogeneic BMT); infection: 12 children (five after autologous and seven after allogeneic BMT); interstitial pneumonitis: 21 children (seven after autologous and 14 after allogeneic BMT); cardiac failure: five children (four after autologous BMT); veno-occlusive disease: eight children (three after autologous, five after allogeneic BMT); acute renal failure: three children (one after autologous and two after allogeneic BMT); multiple organ failure: two cases (one after autologous BMT); cerebral hemorrhage: three children (one after autologous BMT); hypertension: one child; acute GVHD: three children (12% of early deaths after allogeneic BMT).
 ...
PMID:Early deaths in children after BMT. Bone Marrow Transplantation Group of the Italian Association for Pediatric Hematology and Oncology (AIEOP) and Gruppo Italiano Trapianto di Midollo Osseo (GITMO). 146 3

Leukaemia and its associated therapy result in pathophysiological peculiarities relevant to anaesthesia. Leukaemic patients suffer from anaemia, coagulation disorders, and the consequences of immunosuppression. In addition, some patients show infiltrations of the oropharynx, potentially resulting in difficult intubation and/or pharyngeal haemorrhage. Mediastinal masses can induce complete airway obstruction during general anaesthesia. Patients with a white blood cell count (WBC) greater than 100,000/mm3 (hyperleukocytosis) can suffer from the leukostasis syndrome with acute respiratory failure as well as cerebral vascular occlusions and bleeding due to increased blood viscosity and disturbed microvascular perfusion. Since this syndrome may be triggered by surgery, the WBC should be reduced prior to general anaesthesia in patients with hyperleukocytosis. To avoid development of the leukostasis syndrome, transfusion of packed red cells should be restricted in these patients. Hyperleukocytosis can simulate in-vitro hypoxaemia due to the excessive oxygen consumption of the mass of leukaemic blood cells during routine blood gas analysis. Therapy of leukaemia can lead to the tumor-lysis syndrome with hyperuricaemia, hyperphosphataemia, hyperkalaemia, hypocalcaemia, and hypoglycaemia, and may induce acute renal failure. Since drug interactions have only been evaluated for the combination of two or three drugs, interactions of cytotoxic agents with anaesthetics can hardly be predicted because of the large number of drugs simultaneously administered to leukaemic patients. The heart and lungs are target organs for the acute or chronic side effects of cytotoxic drugs, resulting in non-cardiogenic pulmonary oedema (e.g., cytosine-arabinoside), lung fibrosis (e.g., bleomycin), or arrhythmias and cardiac failure (e.g., adriamycin). The severity of these side effects depends on pre-existing organ disease and only in part on drug dosage. Only HLA- and CMV-compatible blood components should be administered to leukaemic patients. Hyperleukocytosis and the first days of cytotoxic treatment represent relative contraindications to general anaesthesia.
 ...
PMID:[Pathophysiologic and anesthesiologic characteristics of patients with leukemia]. 152 54

A 14-year-old girl with acute promyelocytic leukemia (APL) developed cardiomyopathy following chemotherapy for remission induction and subsequent consolidation consisting of cumulative doses of 644 mg/m2 of daunorubicin and 31 mg/m2 of mitoxantrone. Six months after the first complete remission, when relapse of APL was recognized an allogeneic bone marrow transplantation (BMT) from her HLA-identical brother was performed. A preconditioning regimen, consisting of cytarabine (Ara-C, 2 g/m2/day x 3 days and 4 g/m2/day x 3 days), total body irradiation (TBI, 1200 cGy) and etoposide (VP-16, 50 mg/kg) caused moderate gastrointestinal symptoms and transient hemorrhagic cystitis, but did not worsen her cardiac function. Both continuous intravenous administration of heparin to control DIC and continuous low dose dopamine infusion to prevent cardiac failure achieved their purpose. The patient is leukemia-free and has no symptoms related to cardiomyopathy at the eight month after BMT. A preconditioning regimen (Ara-C, TBI and VP-16) appeared to be suitable for BMT to a patient with anthracycline-induced cardiomyopathy.
 ...
PMID:[A successful allogeneic bone marrow transplantation for acute promyelocytic leukemia with anthracycline-induced cardiomyopathy at relapse]. 160 7

Data from the first 103 human heart transplantations in 100 recipients performed at a single centre from November 1983 to January 1990 were analysed to detect risk factors for overall and cause-specific mortality. Twenty-two patients died. Cumulative 1 year graft survival was 82% and 5 year, 68%. Acute and chronic rejection was the cause of death in 9 patients, disseminated infection in 8 and cancer in 3. One patient died from cerebral haemorrhage and 1 from acute cardiac failure. The mean observation time was 803 days (range: 1-2 308 days). Total follow-up was 226.6 graft years. Risk factors were analysed by univariate and multivariate methods. The type of immunosuppression regimen and recipient age above 50 years were independent risk factors for mortality. Histocompatibility mismatching (HLA-DR) and type of immunosuppression were independent risk factors for lethal rejection and a female recipient was an independent risk factor for lethal infection. Prolonged time on extracorporeal bypass was an independent risk factor for both lethal rejection and infection, and also for overall mortality. The impact of extracorporeal bypass time on rejection and infection is discussed, and the importance of prospective HLA matching in heart transplantation is stressed. The association between recipient female sex and infection remains uncertain.
 ...
PMID:Risk factors for total and cause-specific mortality in human cardiac transplantation. Prolonged extracorporeal bypass time: a high risk factor for rejection and infection. 177 80

The mechanisms responsible for the decline in the density of beta-adrenoceptors in the failing myocardium have not been adequately defined. It is a possibility that the nature of the process leading to heart failure may determine, in large part, the pathogenesis of this decline. Sera of some patients with dilated cardiomyopathy contain antibodies directed against the beta-adrenoceptor, as judged by ligand binding inhibition, immunoprecipitation and immunoblotting assays. Because deranged immune function is thought to play a role in dilated cardiomyopathy, immunogenetic markers of the propensity to develop anti-beta-receptor antibodies were sought. The prevalence of HLA-DR4 was significantly higher in dilated cardiomyopathy patients (40 vs 24% in 511 normal subjects, pc less than 0.001). In contrast, no association was found between HLA phenotypes and alcoholic cardiomyopathy. Furthermore, 72% (13 of 18) of the HLA-DR4 dilated cardiomyopathy patients had anti-beta-receptor antibodies compared to 22% (7 of 33) HLA-DR4-negative patients; in the latter, presence of antibody was linked to the HLA-DR1 phenotype. Conversely, 67% (15 of 23) of the antibody-positive patients were typed as HLA-DR4 compared to only 10% of the antibody-negative patients. Interestingly, none of the 23 antibody-positive patients were typed as HLA-DR3 while 37% of the antibody-negative did. Only 25% of alcoholic cardiomyopathy patients had anti-beta-receptor antibodies and no preponderant HLA association could be demonstrated. These results suggest that the presence of anti-beta-receptor antibodies in patients with idiopathic dilated cardiomyopathy may be under the control of the major histocompatibility locus.
 ...
PMID:Anti-beta-receptor antibodies in human dilated cardiomyopathy and correlation with HLA-DR antigens. 215 17

Ten children between the ages of five and fifteen years old with leukemia (two with acute nonlymphocytic leukemia in first remission, four with acute lymphocytic leukemia in first or second remission, one with acute lymphocytic leukemia in relapse, and one with chronic myelocytic leukemia in chronic phase), malignant lymphoma (one) or severe aplastic anemia (one) were given transplants from HLA-matched or mismatched family members between March, 1982 and April, 1984. Two patients died of leukemia relapses on days 107 and 257 following transplantation. One patient died of cardiac failure on day 157. One patient who received HLA-mismatched marrow from his father died of pulmonary edema and acute graft versus host disease on day 32. Six are alive 268-843 days post transplantation. None of the ten patients developed interstitial pneumonia due to cytomegalovirus which is one of the major causes of death reported in other published studies.
 ...
PMID:Allogeneic bone marrow transplantation in children: Tokai experience 1982 to 1984. 301 May 9

This study compares the efficacy of 2 posttransplant immunosuppressive regimens for prevention of graft-versus-host disease (GVHD). Forty-four patients, ages 8-15 years, with homozygous beta thalassemia received marrow allografts from HLA-identical siblings following an ablative regimen of busulfan and cyclophosphamide. Twenty-two patients received cyclosporine (CsA) alone and 22 received cyclosporine, cyclophosphamide, and methotrexate for prophylaxis against GVHD. Two who received CsA alone have died (1 of graft rejection and 1 of acute GVHD) as did 4 patients who received 3 drugs (1 of rejection, 1 of acute GVHD, 1 of infection and cardiac failure before engraftment, and 1 of acute respiratory failure before engraftment). One patient in each group rejected the transplant and survives with thalassemia. The probability of developing acute GVHD was 41% for the CsA group and 15% for the 3-drug group (P = less than 0.05). Patients receiving CsA alone had a probability of event-free survival of 86% compared to 77% in the group receiving 3 drugs (P = 0.40) with a followup of 209-706 days. Although the study showed a decrease in the incidence of GVHD in recipients of the more intensive prophylactic regimen, this study was terminated since it was apparent that even if larger numbers of patients were studied it would be difficult to demonstrate a significant survival advantage with the use of this drug regimen.
 ...
PMID:A comparative trial of posttransplant immunosuppression in patients transplanted for thalassemia. Cyclosporine alone versus cyclosporine, cyclophosphamide, and methotrexate. 327 81

In a study of the outcome of marrow transplantation in patients with advanced thalassemia, 40 patients with homozygous beta-thalassemia who were 8 to 15 years of age (median, 10) received HLA-identical allogeneic marrow after treatment with busulfan and cyclophosphamide. Twenty-eight of the 40 patients were alive and free of disease 260 to 939 days after transplantation, and 2 patients were alive with thalassemia 372 and 1133 days after transplantation. The actuarial probabilities of survival and of disease-free survival at two years were 75 percent and 69 percent, respectively. Ten patients (25 percent) died. Three died of cardiac failure, interstitial pneumonitis, or septicemia within 14 days of transplantation. Three died of infectious complications associated with acute graft-versus-host disease at 46 to 97 days, and two died of infectious complications of chronic graft-versus-host disease at 249 and 290 days. Two patients had transplant rejection and died with marrow aplasia 115 and 192 days after transplantation. One patient had rejection after four months and while the marrow was aplastic underwent a successful second transplantation; the patient was alive without thalassemia 624 days after the first transplantation. The actuarial probability of grade 2 or higher acute graft-versus-host disease in the 32 patients with initial sustained engraftment was 35 percent. Three patients had chronic graft-versus-host disease, which was fatal in two and still active on day 710 in the third. We conclude that bone marrow transplantation can potentially save patients with advanced thalassemia from an otherwise inexorable progression to death from the complications of blood transfusions. The ultimate outcome in this group of patients must await a longer follow-up.
 ...
PMID:Marrow transplantation in patients with advanced thalassemia. 355 Apr 63

Dilated (congestive) cardiomyopathy is a disease of uncertain aetiology in which viral and immune factors are postulated to be of importance. Natural killer cells mediate natural resistance to viral infections and certain tumours. To evaluate natural killer (NK) activity in patients with cardiomyopathy, circulating peripheral blood mononuclear cells were obtained from 16 patients, from 54 normal blood donors, and from 14 patients with heart failure due to other causes (primarily coronary disease). NK activity was assessed as a lymphocyte to target cell ratio causing 50% killing (L/T50). L/T50 in normals was 17.2 +/- 11.4. A normal L/T50 distribution was also noted in heart failure controls (L/T50 = 19.6 +/- 9.9) and 8/16 cardiomyopathy patients (L/T50 = 17.5 +/- 9.5). NK activity was deficient (L/T50 greater than or equal to 50) in 8/16 patients with cardiomyopathy but in only 2/54 normals (p less than 0.001) and 0/14 heart failure controls (p less than 0.01). Of the several clinical and laboratory variables examined, only the frequency of the HLA-A3 tissue type was associated with NK deficiency. NK deficiency is an independent disease marker in about 50% of patients with cardiomyopathy and should be evaluated for a possible role in its pathogenesis.
 ...
PMID:Deficient natural killer cell activity in patients with idiopathic dilated cardiomyopathy. 618 39

The high incidence of rheumatic heart disease (RHD) in black South African children has been attributed mainly to poor socio-economic status and over-crowding. In order to elucidate whether other factors, in particular genetic, were responsible, the HLA-status of 61 black children with rheumatic heart disease was compared with that of 1165 normal controls. Overall, no differences were found, except a higher incidence of HLA-B25 and BW51 in the group with rheumatic heart disease, when the difference was not of statistical significance. Moreover, when the patients were considered in two groups, viz. (a) a "surgical" group which required cardiac surgery and (b) a "non-surgical" group in which cardiac failure was absent or could be easily controlled by medical therapy, the difference between the two groups was also not of statistical significance, though there was a higher incidence of HLA-A10 (which includes HLA-A25 and A26) in the "non-surgical" group. These data appear to agree with the results of other studies which found no significant association between HLA-status and RHD.
 ...
PMID:HLA antigens in black South African children with rheumatic heart disease. 618 86


1 2 3 4 5 Next >>