Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:2.1.1.69 (BMT)
2,655 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 16-month-old Aboriginal boy was diagnosed with chronic granulomatous disease (CGD) when he presented with frequent significant infections from the age of 12 months. BMT was performed from a HLA-matched sibling after conditioning with busulphan, cyclophosphamide, etoposide and antithymocyte globulin. A small bowel obstruction developed in the first week and settled with conservative treatment. VOD occurred in the third week, resolving after treatment with tissue plasminogen activator. Sustained engraftment has occurred, as indicated by return of the nitroblue tetrazolium (NBT) test to normal when performed at 11 weeks and 7 months post-BMT. No further infections have occurred. BMT can be successfully performed for CGD. Complications occurring post-BMT may be related to the underlying disease (CGD). BMT remains an attractive option for CGD in children who have a matched sibling donor.
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PMID:Successful bone marrow transplantation in a child with X-linked chronic granulomatous disease. 883 19

We investigated the nature of hemostatic alterations occurring after bone marrow transplantation. In 45 patients, we evaluated the coagulation parameters, naturally occurring anticoagulants and thrombomodulin at days +15 and +22 after conditioning therapy. It was observed that endothelial cell damage is a central pathogenetic mechanism in some BMT complications. The increased plasma level of thrombomodulin after conditioning therapy is therefore discussed as a marker of endothelial cell injury. At day +15 a significant increase of fibrinogen from 276.1 mg/dI to 389.1 mg/dI was observed, while the natural anticoagulants all decreased significantly. Eleven patients with clinical complications related to endothelial damage had a significant thrombomodulin increase which, in uncomplicated patients, remained unchanged or resulted in lower than baseline values. Analysis of the data shows a strong correlation between clinical findings, reflecting endothelial cell injury and thrombomodulin increase when the increment is > or = 30%. We found a significant elevation in thrombomodulin in 70% of clinical complications related to endothelial cell damage namely: septicemia, GVHD, VOD. There were four cases (or 9%) of false positive data, and only two (or 4.5%) of false negative results. We therefore propose thrombomodulin assessment as a valid parameter to monitor chemotherapy toxicity-related complications.
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PMID:Increased plasma level of vascular endothelial glycoprotein thrombomodulin as an early indicator of endothelial damage in bone marrow transplantation. 886 50

An 18-year-old white male developed severe hepatic veno-occlusive disease (VOD) during an autologous bone marrow transplant for primary refractory nodular sclerosing Hodgkin's disease. As a result of VOD-induced hepatic dysfunction, coagulation studies revealed depression of vitamin K dependent procoagulant factor VII. Intravenous recombinant tissue plasminogen activator 20 mg over h on 4 consecutive days and continuous heparin infusion (1000 unit bolus followed by 150 units/kg/day) resulted in rapid reversal of the VOD syndrome. During treatment, procoagulant factors II, VII, IX and X levels increased indicating the return of hepatic synthesizing capacity. Factor V levels, which were elevated pre-therapy, also rose dramatically. Plasma antigen levels of protein C, a natural anticoagulant, remained severely depressed. No clinical evidence of bleeding and only minimal systemic fibrinolysis was noted. Despite concerns regarding the use of lytic therapy in a thrombocytopenic post-BMT patient, serial measurements of coagulation parameters during severe VOD suggested that low dose rt-PA improved portions of the systemic hemostatic profile.
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PMID:Treatment of hepatic veno-occlusive disease with low-dose tissue plasminogen activator: impact on coagulation profile. 887 29

Cilastatin, an inhibitor of the tubular brush border enzyme dehydropeptidase-I, is added in a fixed combination to imipenem. Cilastatin has been demonstrated in different animal models and in one clinical trial, to reduce the nephrotoxicity associated with cyclosporin A. To evaluate a possible nephroprotective effect of cilastatin following allogeneic BMT we conducted a retrospective analysis of 104 patients transplanted in our BMT Unit from January 1991 to January 1995. Imipenem/cilastatin (I/C) was used in a non-randomized manner in 64 patients during this period. Acute renal failure (ARF) was diagnosed in 32 patients (30%). ARF was not associated with gender, sepsis, conditioning regimen, underlying disease, bilirubin, or age. VOD occurred in 12/32 (37.5%) of patients with ARF whereas it occurred in only 7/72 (9.7%) of patients without ARF (P < 0.0007). ARF was not correlated with use of aminoglycosides, vancomycin, ciprofloxacine, ceftazidime or amphotericin-B. However, 13 patients of 64 exposed to I/C (20.3%) developed ARF vs 19 of 40 patients (47.5%) who were not exposed to I/C (P < 0.003; OR 0.28). Stratified analysis and multiple logistic regression confirmed the I/C nephroprotective action. The mean cyclosporin A levels in the I/C group were significantly decreased (208.6 +/- 64.9) vs the non-I/C group (265 +/- 118). We conclude that these results suggest I/C may counteract acute cyclosporin A nephrotoxicity following BMT and further prospective clinical trials are needed to confirm if routine administration of cilastatine confers benefit in the BMT setting.
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PMID:Nephroprotective effect of cilastatin in allogeneic bone marrow transplantation. Results from a retrospective analysis. 889 92

Hepatic veno-occlusive disease (VOD) is a common cause of morbidity and mortality after BMT. Although treatment of VOD is primarily supportive, some success has been obtained recently with fibrinolytic therapy. However, for critically ill patients liver transplantation may be the only therapeutic option. Nevertheless, this procedure is associated with high mortality and can only be performed in a minority of cases. The transjugular intrahepatic portosystemic stent-shunt (TIPS) is a non-surgical, side-to-side shunt consisting of an intraparenchymal duct between a main branch of the portal vein and a hepatic vein. In this report we describe a patient who underwent TIPS placement for severe VOD following autologous PBPC transplant. No complications developed and gradual improvement in clinical status and liver function was observed early after this therapy. Nine months after TIPS, the patient is asymptomatic with normal liver function. TIPS provides an interesting alternative to invasive therapies for patients with severe VOD after bone marrow or PBPC transplants.
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PMID:Successful treatment of hepatic veno-occlusive disease in a peripheral blood progenitor cell transplant patient with a transjugular intrahepatic portosystemic stent-shunt (TIPS). 900 42

Hepatitis C virus (HCV) genotypes were investigated in 57 HCV-infected patients undergoing allogeneic BMT at four European BMT units where death resulting from liver failure (LF) in HCV-infected patients varied from < 1% to > 80%. The aim of the study was to determine whether differing HCV genotypes could account for the different severity of post-transplant liver disease (LD). Sera from patients with pre (n = 22) or post-BMT (n = 35) HCV infection were collected from Italy (Genova, Monza), Sweden (Huddinge) and Germany (Ulm). Patients were grouped as follows: LF: 19/57; acute hepatitis (AH): 10/57 or chronic hepatitis (CH): 22/57; no liver disease (LD): 6/57. HCV genotypes were identified by hybridisation of the 5'UTR amplified products with type-specific oligonucleotides probes according to Simmonds (Hepatology 1994; 19: 1321-1324). Genotype HCV 1 was identified in 34 patients (60%), HCV 2 in 15 (26%), HCV 3 in three (5%), mixed infection in three (5%) and undefined in two (3.5%). In the LF group HCV 1 was identified in 10/19 and other genotypes in 9/19. Median timing of LF was earlier in patients infected with HCV 1 compared to other genotypes (45 and 68 days, respectively), largely due to the cause of LF; death from veno-occlusive disease (VOD) and hepatitis occurred at 30 and 68 days post-BMT, respectively. Genotype 1 was also identified in cases with no LD. These data indicate that there was no evident correlation between HCV genotype and type or severity of post-transplant liver disease.
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PMID:Hepatitis C virus genotypes and liver disease in patients undergoing allogeneic bone marrow transplantation. 902 52

We have previously demonstrated that syngeneic marrow mixed with H-2 haploidentical marrow transplantation could provide not only protection against graft-versus-host disease (GVHD) but also anti-leukemic (GVL) effects in mice. In the present studies, we report clinical observations using autologous marrow mixed with HLA-haploidentical allogeneic marrow transplantation for treatment of patients with malignant blood diseases. Sixteen cases, including 12 with acute leukemia and four with advanced malignant lymphoma, were treated by autologous marrow, which was purged in vitro by hyperthemia (42.5 degrees C for 70 min) following incubation for 5 days with interleukin 2 (IL-2) in liquid culture and mixed with HLA haploidentical marrow cells from their sibling or parent. Acute GVHD was not observed in any patient after transplantation. Hematological rescue in the clinical setting was demonstrated in all cases but one who died early from hepatic veno-occlusive disease (VOD). Five cases who were transplanted at the time of CR2 or CR3 and in advanced phase of lymphoma, relapsed 4 to 7 months after transplantation. The relapse rate was 31.3%. None of eight patients who received allogeneic BMT within 2 h after ABMT relapsed with median follow-up of 12 months and two of them died from procedure-related complications. Seven cases are still alive and disease-free with a median follow-up of 12 months. Mixed chimerism was found in 3/6 cases, who had different sex donors, by analysis of sex chromosomes. These results show that mixed transplantation is a safe, effective and new approach to treating patients with malignant tumors. In order to detect the effects of GVL, studies are now in progress in our clinic.
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PMID:Autologous bone marrow mixed with HLA-haploidentical allogeneic marrow transplantation for treatment of patients with malignant blood diseases. 911 6

Hepatitis B reactivation following chemotherapy withdrawal may result in hepatitis, hepatic failure and death. We studied the clinical outcome and the causes of hepatic events of hepatitis B surface antigen positive recipients undergoing bone marrow transplantation. Twenty-four hepatitis B surface antigen patients were matched with 24 hepatitis B surface antigen negative patients for age, sex, CMV positive serology, underlying hematological disease and type of bone marrow transplantation. Post-BMT, there were 18 patients in the hepatitis B surface antigen positive group and four patients in the hepatitis B surface antigen negative group who suffered from hepatitis (P < 0.05). Thirteen of the 18 hepatitis were related to HBV reactivation in the hepatitis B surface antigen positive group and none of the four hepatitis in the hepatitis B surface antigen negative group (P = 0.01). The hepatitis B surface antigen positive group also had an increased incidence of acute graft-versus-host disease of liver (6 vs 1, P = 0.03). However, there was no significant increase in the incidence of veno-occlusive disease (10 vs 7, P = 0.40) and persistent hepatitis (3 vs 0, P = 0.07) in the hepatitis B surface antigen positive group. Using the log-rank test, there was no significant difference in survival between the hepatitis B surface antigen positive and negative recipients.
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PMID:Hepatic events after bone marrow transplantation in patients with hepatitis B infection: a case controlled study. 913 71

VOD is an important cause of morbidity and mortality in patients following allogeneic bone marrow transplantation. Although VOD may improve in some patients, severe cases are often fatal. There is no established therapy to prevent progression to severe VOD; nor are there any conclusive or universally accepted methods for prevention of mortality associated with severe VOD. A treatment that could minimize hepatic damage and cause VOD to manifest as reversible liver damage rather than a progressive, fatal disease would indeed have a place in posttransplant therapy. Nontoxic ursodiol may play such a role by replacing hepatotoxic bile acids. Based on the limited available literature (2 studies), it is difficult to draw firm conclusions regarding the use of ursodiol to prevent VOD, although the preliminary results are promising. The studies, although small and not without weakness, suggested that ursodiol effectively reduces the incidence of VOD in allogeneic BMT patients. They do not, however, suggest that ursodiol is effective as treatment for existing VOD, as this was not studied. Also, conclusions were based on patients given busulfan and cyclophosphamide as conditioning therapy and thus might not apply to patients conditioned by other means such as total body irradiation. In summary, the available data do not definitively support the use of ursodiol; however, patients conditioned with busulfan and cyclophosphamide who are at risk for VOD (e.g., pretransplant liver disease, liver metastases) may be candidates for ursodiol prophylactic therapy. Larger, randomized clinical trials are warranted to further define the potential role of ursodiol for the prevention of venoocclusive disease of the liver in BMT patients.
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PMID:Ursodiol to prevent hepatic venoocclusive disease. 933 52

One hundred and thirty-seven consecutive patients who received bone marrow or peripheral blood stem cell transplantation were studied retrospectively to identify the risk factors for hepatic veno-occlusive disease (VOD). Of the 137 recipients, twenty (14.6%) patients were diagnosed with VOD using the McDonald's criteria. In these 20 patients with VOD, we analyzed various clinical parameters, including age, sex, HLA status, conditioning regimen, irradiation, immunosuppressive agents, mode of transplantation, history of hepatic dysfunction, pre-transplant hepatic and renal function, infectious episodes, antibiotics use, and serum viral titers. A history of hepatic dysfunction and low levels of pseudocholinesterase before transplantation were found to be statistically significant (P = 0.04 and 0.04). Low levels of pseudocholinesterase were significant by multivariate analysis using the logistic regression model (P = 0.02). These results suggest that pseudocholinesterase levels before transplant are important markers of VOD in patients receiving BMT.
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PMID:Risk factors for hepatic veno-occlusive disease after bone marrow transplantation: retrospective analysis of 137 cases at a single institution. 933 56


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