Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Bronchiolitis obliterans is a nonspecific pathologic lesion seen after fume inhalation and infections, which is associated with connective tissue disorders and is a complication of organ transplantation. Bronchiolitis obliterans with organizing pneumonia is also associated with the connective tissue disorders but is usually idiopathic and has better prognosis with corticosteroid therapy. Bone marrow-related obliterative bronchiolitis is limited to patients who develop chronic graft-versus-host disease. Symptoms begin with cough in 3 to 6 months and progress to dyspnea and severe airflow obstruction. The roentgenogram is normal or shows hyperinflation. Prognosis is poor and most patients develop disabling irreversible airflow obstruction. Bronchiolitis obliterans is the most important clinical complication in heart-lung transplant recipients. It is not preceded by typical features of chronic graft-versus-host disease, but has the same clinical course of dyspnea, airflow obstruction, and poor response to therapy. Bronchiolitis obliterans in transplant recipients may represent a form of allograft rejection.
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PMID:Bronchiolitis obliterans and airways obstruction associated with graft-versus-host disease. 306 87

Bronchiolitis obliterans occurred in the setting of chronic graft-versus-host disease 1 year after allogeneic bone marrow transplantation for chronic myelogenous leukemia. The severe obstructive pulmonary disease followed an episode of interstitial pneumonitis. The etiology and possible relationship to graft-versus-host disease of this rare pulmonary lesion following bone marrow transplantation are discussed.
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PMID:Bronchiolitis obliterans after bone marrow transplantation. 388 55

The application of lung transplantation to the pediatric population was a natural extension of the success realized in our adult transplantation program, which began in 1982. Twenty pediatric patients (age range 3 to 18 years) have had heart-lung (n = 11), double lung (n = 8), and single lung (n = 1) transplantation procedures. The causes of end-stage lung disease were primary pulmonary hypertension (n = 7), congenital heart disease (n = 5), cystic fibrosis (n = 4), pulmonary arteriovenous malformation (n = 2), graft-versus-host disease (n = 1), and desquamative interstitial pneumonitis (n = 1). Four (20%) patients had thoracic surgical procedures before the transplantation operation. The survival was 80% at a mean follow-up of 2 years. Immunosuppressive drugs included cyclosporine (n = 9) or FK 506 (n = 11) based therapy with azathioprine and steroids. Children were followed up by means of spirometry, transbronchial biopsy, and primed lymphocyte testing of bronchoalveolar lavage fluid. The mean number of treated episodes of rejection was 1.4 at 30 days, 0.5 at 30 to 90 days, and 1.4 at more than 90 days, and the first treated rejection episode occurred on average 28 days after the operation. Obliterative bronchiolitis developed in four (25%) of 16 patients surviving more than 100 days. Results of pulmonary function tests have remained good in almost all recipients. The greatest infectious risk was that of cytomegalovirus: one death and one case of pneumonia. Posttransplantation lymphoproliferative disease was diagnosed in two (12.5%) patients; both recovered. The most common complications were hypertension (25%) and postoperative bleeding (15%). Early results indicate that lung transplantation is a most promising therapy for children with severe vascular and parenchymal lung disease.
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PMID:Pediatric lung transplantation. The years 1985 to 1992 and the clinical trial of FK 506. 767 72

Forty children (aged 1 to 18 years, 27 female and 13 male) have undergone heart-lung (21), double lung (17), and single lung (2) transplant procedures at our center from 1985 through April 1994. The indications for transplantation have been diverse, primary pulmonary hypertension (10), cystic fibrosis (11), congenital heart disease (10), arteriovenous malformation (3), emphysema (1), graft-versus-host disease (1), rheumatoid lung (1), cardiomyopathy (1), desquamative interstitial pneumonitis (1), and Proteus syndrome (1). The actuarial 1-year survival was 73% (mean follow-up 2 years). One-year actuarial survival for disease groups ranged from 60% for cystic fibrosis to 90% for congenital heart disease. We have identified six issues critical to the patient and programatic survival of pediatric lung transplantation. Our experience and management strategies in these areas are reviewed. Cytomegalovirus: Cytomegalovirus disease developed in six of eight patients with cytomegalovirus mismatching (donor +/recipient-) and in seven of 32 patients who survived more than 30 days (23%). All but cytomegalovirus donor -/recipient- patients were treated with ganciclovir for 4 weeks after transplantation. Obliterative bronchiolitis: Obliterative bronchiolitis developed in seven of 32 (25%) patients who survived more than 30 days. Obliterative bronchiolitis was manifest within the first posttransplantation year as a rapid decline in small airway function. Aggressive augmentation of immunosuppression has been used with little success. Posttransplantation lymphoproliferative disease: Posttransplantation lymphoproliferative disease developed in five of 32 (15%) patients who survived more than 30 days developed. One patient died (17% mortality) despite retransplantation. In four patients the disease resolved with reduction in immunosuppression alone, and one required the addition of interferon alfa. Cystic fibrosis: We have changed our management strategies to avoid triple drug immunosuppression, perioperative blood and bronchial cultures, aggressive antimicrobial therapy, and exclusion of patients with panresistant organisms; this has resulted in elimination of infectious mortalities thus far in the pediatric cystic fibrosis group. Airways: In 21 heart-lung recipients with tracheal anastomoses we have had no airway complications. The double and single lung transplant recipients accounted for 34 bronchial and one tracheal anastomoses. Three (9%) bronchial stenoses developed. Two were treated with silicone stents and one with balloon dilation.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Critical issues in pediatric lung transplantation. 781 8

Between June 1988 and February 1993, combined heart-lung transplantation was performed in 30 children and adolescents aged 3.6 to 18.6 years (mean, 12.2 years) at The Hospital for Sick Children in London. Original diagnoses included cystic fibrosis (n = 25), Eisenmenger's syndrome (n = 4), and chronic graft-versus-host disease of the lung (n = 1). Posttransplantation maintenance immunosuppression comprised a triple regimen, with methylprednisolone and antithymocyte globulin given perioperatively and for episodes of allograft rejection. Actuarial survival was 63% (95% confidence interval: 42%-78%) at 1 year and 48% (95% confidence interval: 27%-66%) at 3 years. Obliterative bronchiolitis has been diagnosed in 13 patients (43%). Actuarial freedom from obliterative bronchiolitis in survivors was 76%, 59%, and 37% at 12, 24, and 36 months after transplantation, respectively. Recipients in whom obliterative bronchiolitis developed within the first year (n = 6) had more episodes of pulmonary rejection during the first 6 months after transplantation (mean, 5.7 episodes per patient) than those in whom "premature" obliterative bronchiolitis did not develop (mean, 3.2 episodes per patient). Infection of the pulmonary allograft was implicated to a lesser extent in predisposing to obliterative bronchiolitis. At 2, 3, and 6 months, tracheal stenosis developed in three patients, all of whom died with obliterative bronchiolitis within 10 months of transplantation. Noncompliance with therapy was considered a contributory factor in producing obliterative bronchiolitis in four adolescent recipients. The high incidence of obliterative bronchiolitis observed in this pediatric cohort may have a multifactorial cause.
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PMID:Incidence of obliterative bronchiolitis after heart-lung transplantation in children. 831 13

Bone marrow transplantations (BMT) are limited by complications connected with infection and non-infection processes. One of them, after allogenic bmt is a graft versus host disease. Bronchiolitis obliterans is observed in patients with GVHD. It seems that bronchiolitis obliterans is not observed after autologic bmt. If this observation is proved, it will mean that GVHD is the main factor of the development bronchiolitis obliterans.
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PMID:[Bronchiolitis obliterans: a potential complication after bone marrow transplantation]. 1050 46

Chronic graft-versus-host disease (GVHD) is a major cause of morbidity and mortality in long-term survivors of allogeneic stem cell transplantation. The immunopathogenesis of chronic GVHD is, in part, TH-2 mediated, resulting in a syndrome of immunodeficiency and an autoimmune disorder. The most important risk factor for chronic GVHD is prior history of acute GVHD and strategies that prevent acute GVHD also decrease the risk of chronic GVHD. Other important risk factors are the use of a non-T cell-depleted graft, and older age of donor and recipient. Whether recipients of peripheral blood stem cells are at increased risk of chronic GVHD remains unsettled. There are no known pharmacologic agents which can specifically prevent development of chronic GVHD. Agents which have efficacy in the treatment of autoimmune disorders have been utilized as therapy for established chronic GVHD and are associated with response rates of 20% to 80%. Most responses are confined to skin, soft tissue, oral mucosa and occasionally liver. Bronchiolitis obliterans responds infrequently to therapy and is associated with a dismal prognosis. Newer, promising therapeutic strategies under investigation include thalidomide, photopheresis therapy, anti-tumor necrosis factor and B cell depletion with anti-CD20 monoclonal antibody.
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PMID:Chronic graft-versus-host disease: clinical manifestation and therapy. 1150 29

Chronic graft-versus-host disease occurring in the setting of allogeneic bone marrow transplantation (BMT) can affect many organ systems, is a cause of significant morbidity, and contributes to late deaths. Bronchiolitis obliterans is a form of obstructive airway disease; when seen in the post-BMT setting, it is considered a manifestation of chronic graft-versus-host disease. Air-leak syndromes including pneumothoraces, pneumomediastinum and subcutaneous emphysema are rare complications of bronchiolitis obliterans. Here we describe a patient who developed pneumomediastinum, pneumopericardium, subcutaneous emphysema and pneumothorax secondary to severe bronchiolitis obliterans complicating the post bone marrow transplantation course.
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PMID:Spontaneous pneumomediastinum and subcutaneous emphysema complicating bronchiolitis obliterans after allogeneic bone marrow transplantation--case report and review of literature. 1152 71

A 9-year-old boy with secondary chronic myelogenous leukemia after treatment of acute lymphoblastic leukemia underwent allogeneic bone marrow transplantation (BMT) from an HLA-identical sibling in December 1998. Grade II acute GVHD developed on day 24 and chronic GVHD developed 5 months after BMT. Cough and dyspnea appeared 9 months after BMT. Despite administration of tacrolimus and methylprednisolone (m-PSL) pulse therapy, the dyspnea gradually increased in severity. Bronchiolitis obliterans was diagnosed on the basis of lung biopsy in January 1999. Because renal dysfunction made it difficult to continue the use of tacrolimus, we attempted antithymocyte globulin (ATG) + m-PSL therapy. Major BCR/ABL mRNA was transiently positive on RT-PCR after the ATG + m-PSL therapy, but no severe complications were observed. A decreasing V50/V25 level and increasing peak flow value were observed in respiratory function tests after ATG + m-PSL therapy, and the patient is currently free of dyspnea. Our findings suggest that ATG + m-PSL therapy is beneficIal for patients with drug-resistant bronchiolitis obliterans after BMT.
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PMID:[Successful treatment with combined anti-thymocyte globulin and methylprednisolone for bronchiolitis obliterans after allogeneic bone marrow transplantation in a child with chronic myelogenous leukemia]. 1186 61

Bone marrow transplantation (BMT) is a successful and recognised treatment option for patients with a number of haematological and non-haematological malignant and non-malignant conditions. Pulmonary complications both infectious and non-infectious are common after BMT. Multiple factors are thought to contribute to pulmonary complications, including the type and duration of immunological defects produced by the underlying disease and treatment, the development of graft-versus-host disease (GVHD), and the conditioning regimens employed. These complications are classified as early or late, depending on whether they occur before or after 100 days from transplantation. Early non-infectious pulmonary complications typically include pulmonary oedema, upper airway complications, diffuse alveolar haemorrhage, cytolytic thrombi, and pleural effusion. Bronchiolitis obliterans, veno-occlusive disease, and secondary malignancies occur late after BMT. Idiopathic pneumonia syndrome, GVHD, and radiation induced lung injury can occur in early or late period after BMT.
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PMID:Non-infectious pulmonary complications after bone marrow transplantation. 1215 65


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