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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)

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

Heart-lung transplantation and lung transplantation have become accepted techniques in adult patients with end-stage cardiopulmonary disease. We report here our experience between July 1985 and March 1993 with 34 children (< 20 years) who underwent heart-lung (n = 18) or lung transplantation (n = 17). Indications for transplantation included cystic fibrosis (n = 9), congenital heart disease with Eisenmenger complex (n = 9), primary pulmonary hypertension (n = 8), pulmonary arteriovenous malformations (n = 2), desquamative interstitial pneumonia (n = 2), Proteus syndrome with multicystic pulmonary disease (n = 1), graft-versus-host disease (n = 1), rheumatoid lung disease (n = 1), and bronchiolitis obliterans and emphysema (n = 1). Twenty-six patients (76%) have survived from 1 to 88 months after transplantation; most patients have returned to an active lifestyle. Of the eight deaths, four were due to infections, two to multiorgan failure, 1 to posttransplant lymphoproliferative disease, and one to donor organ failure. Four of the patients who died had cystic fibrosis. Despite considerable morbidity related to infection, rejection, and function of the heart-lung and lung allograft in some patients, our results with this potentially lifesaving procedure in the pediatric population have been encouraging.
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PMID:Experience with pediatric lung transplantation. 830 35

The application of lung transplantation to the pediatric population was a natural extension of the success realized in our adult transplant program, which began in 1982. Thirty-two pediatric patients (age range 1 to 18 years) have undergone heart-lung (n = 16), double-lung (n = 14), and single-lung (n = 2) transplantation procedures. The cause of end-stage lung disease was primary pulmonary hypertension (n = 7), congenital heart disease (n = 7), cystic fibrosis (n = 9), pulmonary arteriovenous malformation (n = 2), desquamative interstitial pneumonitis (n = 2), graft-versus-host disease (n = 1), emphysema (n = 1), rheumatoid lung (n = 1), cardiomyopathy (n = 1), and Proteus syndrome (n = 1). Six patients (19%) underwent pretransplantation thoracic surgical procedures. The survival rate was 78% at a mean follow-up of 1.8 years. The survival rate in the 23 recipients without cystic fibrosis was 87% (95% since 1985). The actuarial 1-year survival rate in the nine recipients with cystic fibrosis was 55%. Immunosuppression was cyclosporine (n = 9) or FK 506 (n = 23)-based therapy with azathioprine and steroids. Children were followed up by spirometry, transbronchial biopsy, and primed lymphocyte testing of bronchoalveolar lavage fluid. The mean number of treated episodes of rejection per patient in the groups treated with cyclosporine and FK 506, respectively, was 1.0 and 1.2 at 30 days, 0.67 and 0.38 at 30 to 90 days, and 2.33 and 0.46 at greater than 90 days (p < 0.001, Fisher exact test).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pediatric lung transplantation: expanding indications, 1985 to 1993. 831 44

We describe a woman post allogeneic bone marrow transplantation (BMT), who presented to the emergency room with subcutaneous emphysema and pneumomediastinum as the first manifestation of bronchiolitis obliterans complicating mild chronic graft versus host disease (GVHD). In contrast to other patients with pneumomediastinum described in the literature, this patient suffered from only mild GVHD. She did not receive methotrexate as GVHD prophylaxis, and the pneumomediastinum was a presenting manifestation rather than a terminal event. In addition, this is the first description of subcutaneous emphysema with this setting. Therefore, bronchiolitis obliterans should be highly suspected in post-BMT patients presenting with pneumomediastinum and subcutaneous emphysema, and prompt therapy should be initiated.
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PMID:Bronchiolitis obliterans presenting as subcutaneous emphysema and pneumomediastinum: a case report. 915 69

An adolescent female underwent bone marrow transplantation for relapsed leukemia and developed acute and chronic graft-versus-host disease and idiopathic pneumonia syndrome. Her lung disease responded to large doses of methylprednisolone but evolved to pulmonary fibrosis and pneumomediastinum and subcutaneous emphysema in the convalescent period. Pulmonary function tests revealed a restrictive pattern. Pneumomediastinum and subcutaneous emphysema are complications not only of obstructive but also of restrictive lung disease and vary with respect to time of onset.
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PMID:Pneumomediastinum, subcutaneous emphysema, and pulmonary fibrosis in a patient with idiopathic pneumonia syndrome after bone marrow transplantation. 1068 22

A 45-year-old man was diagnosed as having Ph1+ acute lymphocytic leukemia (ALL) in February 1997. Complete remission was achieved by chemotherapy. Allogeneic BMT from his HLA-identical sister was performed on June 11, 1997. Engraftment was relatively quick, but acute GVHD (grade I) developed. The patient was discharged on day 113. Seven months after BMT, in January 1998, exertional dyspnea developed gradually. Chest X-ray examination showed diffuse interstitial pneumonia, for which corticosteroid was started immediately. The symptoms and signs gradually improved. However, on the 20th hospital day (February 23), bilateral subcutaneous emphysema developed in the neck and supraclavicular region. Chest X-ray and CT examinations showed pneumomediastinum without pneumothorax. The pneumomediastinum and subcutaneous emphysema gradually subsided after 3 weeks of bed rest. Subcutaneous emphysema and pneumomediastinum are relatively rare complications of allogeneic BMT.
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PMID:[Idiopathic mediastinal and subcutaneous emphysema in a patient with acute lymphocytic leukemia after allogeneic bone marrow transplantation]. 1119 33

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

It is difficult to treat lung complications caused by chronic graft-versus-host disease (GVHD) after allogeneic stem cell transplantation (allo-SCT). We retrospectively analyzed the characteristics of five patients with mediastinal emphysema (ME) and bilateral pneumothoraces (BP) caused by chronic lung GVHD after allo-SCT. Four of these patients had undergone unrelated SCT, and three had had HLA-identical unrelated donors. All patients received total body irradiation (TBI) during conditioning. Immunosuppressive agents were administered as GHVD prophylaxis, but two patients developed acute GVHD and all the five developed chronic GVHD. The onset of lung complications was 99-1915 days (median, 202 days) after SCT. The onset of ME and BP was 6-48 days (median, 23 days) after the onset of lung complications. Immunosuppressive agents were initially beneficial on the lung complications, but the patients later showed no response to therapy, and all died from respiratory failure 7-195 days (median, 28 days) after the development of ME and BP. The results suggest that these complications progress rapidly, are resistant to treatment, and have a poor prognosis. It is therefore important to start prophylaxis and treatment as early as possible.
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PMID:Mediastinal emphysema and bilateral pneumothoraces with chronic GVHD in patients after allogeneic stem cell transplantation. 1506 95

We reported 5 patients who developed air-leak syndrome (ALS) including pneumothorax, pneumomediastinum and subcutaneous emphysema after allogeneic stem cell transplantation (SCT). The underlying diseases were AML (n=2), ALL (n=1), MDS (n=1), and CML (n=1). All patients received allogeneic SCT from related donors including 2 donors with HLA mismatch. Total body irradiation was performed as a conditioning regimen in all patients. Late-onset noninfectious pulmonary complications (LONIPC) were detected in all patients before the development of ALS. The interval from diagnosis of LONIPC to onset of ALS was 10-360 days (median, 20 days). Four of 5 patients were treated with corticosteroid for chronic graft-versus-host disease and/or LONIPC. To date, three patients have died of respiratory failure. The others are currently alive and one of these surviving patients is receiving home oxygen treatment. Physicians should be aware of this rare complication following LONIPC, because treatment of ALS is difficult in some patients.
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PMID:[Air-leak syndrome in patients with non-infectious pulmonary complications after allogeneic hematopoietic stem cell transplantation]. 1922 28

Using animal models for autoimmune diseases, we have previously shown that allogeneic bone marrow transplantation (BMT) can be used to treat autoimmune diseases and that autoimmune diseases are hemopoietic stem cell (HSC) disorders. We have recently developed a new BMT method. The method consists of a perfusion method (PM) plus intrabone marrow (IBM)-BMT. The PM, in comparison with conventional aspiration, can minimize the contamination of BMCs with T cells from peripheral blood, and consequently no graft-versus-host disease develops. Because bone marrow cells collected by the PM contain not only HSCs but also mesenchymal stem cells (MSCs), the injection of both cells directly into the bone marrow cavity facilitates the engraftment of donor hemopoietic cells. Using this new method, we show that most age-associated diseases, such as osteoporosis and emphysema, are MSC disorders and, on the basis of this evidence, propose a new concept of stem cell disorders, including HSC and MSC disorders. We believe that the advent of IBM-BMT and our proposed concept herald a revolution in the field of transplantation (BMT and organ transplantation) and regeneration medicine.
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PMID:A new bone marrow transplantation method for stem cell disorders. 1975 28


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