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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Donor scarcity and the limitations of organ preservation dictate that a wide size range be used to maximize effective donor utilization in pulmonary transplantation. Problems of size disparity are magnified in the pediatric population, where relevant dimensions vary considerably by age. There have been 10 pediatric lung recipients (7 bilateral, 2 single, 1 heart-lung) at our institution since 1991. The age range was 3 to 26 years (mean, 13.7), and the weight range was 15 to 57 kg (mean, 33.6). Diagnoses included cystic fibrosis (5), pulmonary fibrosis (2), pulmonary hypertension (2), and arteriovenous malformation (1). The donor-to-recipient weight ratio ranged from 0.45 to 1.9, and the donor-to-recipient thoracic height ratio ranged from 0.6 to 1.6. Lobar resection and delayed chest closure was required in one case and was successful. Cardiopulmonary bypass was used electively in seven cases. No reexplorations for bleeding were necessary. Two of the three hospital deaths resulted from right ventricular failure, judged to be secondary to excessive pulmonary artery pressures intraoperatively associated with small donor allografts. Elective bypass had not been used. Inhaled nitric oxide (6 patients) and/or extracorporeal membrane oxygenation (2 patients) were used for temporary postoperative support. The hospital survival rate was 70% (7/10). One late death occurred 14 months postoperatively and was caused by obliterative bronchiolitis. The authors conclude that size disparity is a significant problem in pediatric lung transplantation. However, with elective use of cardiopulmonary bypass and aggressive postoperative support, a broad size range can be used.
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PMID:Size disparity in pediatric lung transplantation. 796 25

Lung transplantation nowadays has become a therapeutic modality in the treatment of patients with a variety of end-stage lung diseases. Between July 1991 and December 1992, twelve patients received an isolated lung transplant (eight single lungs and four double lungs) at the University Hospitals of Leuven. The indication for transplantation was emphysema in five patients, pulmonary fibrosis in three, cystic fibrosis in three and primary pulmonary hypertension in one. There were four early, in-hospital deaths (30%): two from sepsis and multi-organ failure, one from anoxia following a bronchial dehiscence and another patient exsanguinated following stent insertion for a partial bronchial dehiscence. Three more patients have died during follow-up: two from chronic respiratory failure secondary to the development of obliterative bronchiolitis (one at 8 months and one at 17 months), and one from a late bronchovascular fistula 4 months following transplantation. The overall actuarial one and two year-survival was 50.0% and 41.6% respectively. All patients discharged from hospital were oxygen free with an improved lung function and exercise capacity. We conclude that lung transplantation is a viable therapeutic option for selected patients with end-stage, irreversible lung disease. In our experience, the bronchial anastomosis remains an important keystone in the early success. Lung transplantation provides a good quality of life in patients free from infection and rejection. Nevertheless, chronic rejection resulting in obliterative bronchiolitis is a major problem in long-term survivors.
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PMID:Isolated lung transplantation; initial experience at the University Hospitals Leuven. Leuven Lung Transplant Group. 797 65

Computed tomography (CT) is useful in evaluating bronchial and bronchiolar abnormalities. Common bronchial and bronchiolar abnormalities include bronchiectasis, asthma, bronchial obstruction, and various forms of bronchiolitis. Causes of bronchiectasis include cystic fibrosis, childhood viral infection, allergic bronchopulmonary aspergillosis, and pulmonary fibrosis. On CT scans, cystic fibrosis may manifest with bronchial wall thickening, bronchiectasis (usually cylindrical, with varicose and cystic forms seen in advanced cases), and mucus plugs in the bronchi. Allergic bronchopulmonary aspergillosis is characterized by central, varicose or cystic bronchiectasis, predominantly in the upper lobes, with mucoid impaction in the bronchi. Traction bronchiectasis may be seen on CT scans of pulmonary fibrosis. Asthma is characterized by bronchial wall thickening due to inflammation. Bronchial dilatation and air trapping may also be seen. Bronchiolitis obliterans is manifested by direct and indirect signs on CT scans; the former consist of centrilobular branching structures and nodules, and the latter consist of bronchiectasis and bronchiolectasis, mosaic perfusion, and air trapping. CT findings of bronchiolitis obliterans organizing pneumonia (also known as cryptogenic organizing pneumonia) include air-space consolidation and nodules, with occasional bronchial dilatation and pleural effusions.
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PMID:CT of bronchial and bronchiolar diseases. 799 28

From July 1990 to April 1993, 36 lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight children had been continuously supported with a ventilator for 3 days to 4.5 years before transplantation and three were supported by extracorporeal membrane oxygenation. Indications for lung transplantation in this pediatric population included the following: cystic fibrosis (n = 13), pulmonary hypertension, and associated congenital heart disease (n = 10), pulmonary atresia, ventricular septal defect and nonconfluent pulmonary arteries (n = 3), pulmonary fibrosis (n = 6), and acute respiratory distress syndrome (n = 1). Three children underwent retransplantation for acute graft failure (n = 2) or chronic rejection (n = 1). Pulmonary fibrosis was related to complications of treatment of acute of myelogenous leukemia with bone marrow transplantation in two children and to bronchiolitis obliterans, bronchopulmonary dysplasia, interstitial pneumonitis, and Langerhans cell histiocytosis in four others. Thirteen children underwent lung transplantation and concomitant cardiac repair. Bilateral lung transplantation, ventricular septal defect closure and pulmonary homograft reconstruction of the right ventricular outflow tract to the transplanted lungs was performed in three children by means of a new technique that avoids the need for combined heart-lung transplantation. Two patients had ventricular septal defect closure and single lung transplant for Eisenmenger's syndrome, two had ligation of a patent ductus arteriosus and transplantation, three additional children underwent atrial septal defect closure and lung transplantation, and two underwent lung transplantation for congenital pulmonary vein stenosis. Eight early deaths and three late deaths occurred (actuarial 1-year survival 62%). Lung transplantation in children has been associated with acceptable early results, although modification of the adult implantation technique has been necessary. Lung transplantation and repair of complex congenital heart defects is possible; heart-lung transplantation may only be required for patients with severe left heart dysfunction and associated pulmonary vascular disease. Bronchiolitis obliterans remains a major concern for long-term graft function in pediatric lung transplant recipients.
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PMID:Pediatric lung transplantation. Indications, techniques, and early results. 815 51

To study the role of transforming growth factor-beta 1 (TGF-beta 1) in the pathogenesis of pulmonary fibrosis we have examined lung biopsies from nine patients with systemic sclerosis and interstitial lung disease, eight with 'lone' cryptogenic fibrosing alveolitis, two with cystic fibrosis, two with extrinsic allergic alveolitis, two with Langerhans' cell histiocytosis, one with lymphangioleiomyomatosis, one with giant cell interstitial pneumonia, and one adenocarcinoma of the lung. In cryptogenic fibrosing alveolitis, both 'lone' and associated with systemic sclerosis alveolar macrophages, bronchial epithelium and hyperplastic type II pneumonocytes expressed intracellular TGF-beta 1. Extracellular TGF-beta 1 was found in the fibrous tissue immediately beneath the bronchial and hyperplastic alveolar epithelium. In normal lung, however, the alveolar epithelium and alveolar interstitium were negative for both forms of TGF-beta 1. There was strong expression of TGF-beta 1 in hyperplastic mesothelium and its underlying connective tissue and in Langerhans' cells in the two cases of histiocytosis. In the organizing pneumonia in cystic fibrosis, the intraalveolar buds of granulation tissue reacted strongly for the extracellular form of TGF-beta 1 and the overlying hyperplastic epithelium expressed the intracellular form. In lymphangioleiomyomatosis, the aberrant smooth muscle cells strongly expressed intracellular TGF-beta 1 and the extracellular form was expressed in the adjacent connective tissue. In giant cell interstitial pneumonia, the numerous alveolar macrophage including the multinucleate forms, expressed intracellular TGF-beta 1, as did the hyperplastic alveolar epithelium.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Immunohistochemical localization of transforming growth factor-beta 1 in the lungs of patients with systemic sclerosis, cryptogenic fibrosing alveolitis and other lung disorders. 818 7

Between February 1988 and January 1992, 61 patients have undergone bilateral lung transplantations (42 heart-lung and 19 double-lung) in Bordeaux. The underlying diseases were primary or secondary hypertension (20), emphysema (22), or other diseases including cystic fibrosis, pulmonary fibrosis, silicosis, and sarcoidosis (19). Actuarial survival for double-lung and heart-lung transplant recipients was 66% and 72% at 1 year and 57% and 53% at 3 years, respectively. Forty-two patients were still alive 6 months after operation, and we studied their pulmonary function at the short and long term. All parameters except arterial carbon dioxide tension had improved dramatically at 6 months (p < 0.0001). Vital capacity, forced expiratory volume in 1 second, and forced expiratory flow rate between 25% and 75% of vital capacity were at 79% +/- 3%, 92% +/- 5%, and 105% +/- 8% of the predicted values, respectively. Arterial oxygen tension was 88 +/- 3 mm Hg. Nine months after operation, a slight decrease in forced expiratory volume in 1 second and forced expiratory flow rate between 25% and 75% of vital capacity appeared but values remained more than 75% predicted. This was related to the occurrence of obliterative bronchiolitis in 6 patients (14%). At 9 months, flow rates and oxygen tension of these 6 patients were highly different from those of patients free of obliterative bronchiolitis (p < 0.0002 for flow rates and p < 0.01 for oxygen tension). Only 1 patient required retransplantation. The others are living an almost normal life. Our results are discussed in view of the published reports on single-lung transplantation. Short-term results of bilateral lung transplantation are thus excellent and maintained on a long-term basis. Therefore, in our opinion, bilateral lung transplantation is the therapy of choice for pulmonary hypertension and emphysema.
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PMID:Long-term functional results after bilateral lung transplantation. Bordeaux Lung and Heart-Lung Transplant Group. 832 78

Malnutrition is a documented problem in some types of endstage lung disease (ESLD). Recently, isolated lung transplants have successfully reversed the respiratory failure of patients suffering from ESLD. In this study, we compare the preoperative and postoperative nutritional states of lung transplant recipients using weight-to-height ratios, anthropometric measurements, subjective global assessment, and biochemical blood values. Patients with emphysema, cystic fibrosis, and other types of bronchiectasis, but not patients with pulmonary fibrosis or pulmonary hypertension, were malnourished preoperatively. All groups had normal biochemical profiles. Caloric intake of patients with cystic fibrosis and bronchiectasis was increased above predicted basal energy expenditure levels. By six months to one year postoperatively, all groups of malnourished patients had significantly improved their nutritional status. Emphysema patients improved nutrition by maintaining preoperative caloric intake levels--however, both cystic fibrosis and bronchiectasis patients were able to achieve the same goal with significantly decreased caloric intakes. We conclude that malnourished ESLD patients receiving isolated lung grafts are able to achieve normal nutrition within one year posttransplant. Since this occurs in all cases with a reduced, or at best maintained, caloric intake, more study is needed to elucidate the factors that contribute to ESLD malnutrition.
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PMID:A comparison of preoperative and postoperative nutritional states of lung transplant recipients. 835 88

The significance of cytomegalovirus (CMV) infection after lung transplantation was investigated in 20 patients (ten women and ten men; mean age 46 [21-67] years). Indications for transplantation were emphysema (n = 6), cystic fibrosis (n = 2), primary pulmonary hypertension (n = 2), pulmonary fibrosis (n = 5), obliterating bronchiolitis (n = 2), cystic lung (n = 2) and bronchiectasis (n = 1). Incidence, diagnostic parameters (serology, virus isolation and histology) and efficacy of prophylactic and therapeutic measures were recorded. 16 of the 20 patients developed a CMV infection, which in 12 was clinically significant. CMV pneumonia developed in two patients, proving fatal in one. The infection occurred a median of 47 (17-200) days after the transplantation. Administration of Ganciclovir (5 mg/kg twice daily intravenously) brought about remission of symptoms in all but one of the patients and improved the clinical parameters.--This experience demonstrates that regular monitoring of the patients for possible CMV infection and its early therapy can achieve a low death rate.
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PMID:[Cytomegalovirus infection following lung transplantation]. 838 98

Cystic fibrosis, an autosomal recessive disorder, is the most common genetic disease of Caucasians. One in 25 Caucasians are carriers of the gene. The gene is found far less commonly in other races. There are over 230 different alleles of the gene, located on the 7th chromosome. The gene encodes for a membrane protein that functions as an ion channel. The survival of cystic fibrosis patients has been gradually increasing, with a mean survival in 1990 of 28 years. If the current trend of improved survival continues, it is estimated that half of cystic fibrosis patients will be over 18 years old by 1996. Disease is found in many organs including the lungs, sinuses, pancreas, gastrointestinal tract, hepatobiliary system, sweat glands and reproductive tract. The majority of patients die of pulmonary disease. The airways become chronically colonized with bacteria that cannot be eradicated, leading to bronchitis, bronchiectasis, and finally, pulmonary fibrosis with respiratory failure. The pulmonary disease may be complicated by massive hemoptysis and pneumothorax. Patient survival rates have increased because of antibiotic therapy and improved nutrition with pancreatic enzyme replacements. New treatments for the pulmonary disease are under clinical trial and include antiproteases, amiloride, a sodium channel blocker, and DNase. The insertion of the normal cystic fibrosis allele into an animal model using a modified adenovirus with effective transcription suggests that gene therapy may be possible in the future, but safety and technical problems have to be addressed.
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PMID:Cystic fibrosis. 842 40

Lung transplantation is becoming an acceptable mode of therapy worldwide for the end-stage lung disease. We present our initial experience with the first 10 consecutive lung transplant patients at Hadassah University Hospital. There were 5 males and 5 females with an age range 27 to 59 years. Eight patients underwent single lung transplantation, one patient had double lung transplantation and one had heart-lung transplantation. Indications were: pulmonary fibrosis in 4, emphysema in 4, cystic fibrosis in 1, and cystic bronchiectasis in 1. Two patients had primary graft failure (1 death). Nine patients had a serious infection after transplantation (1 death). Four patients developed airway complications including dehiscence of bronchial anastomosis (1 death), bronchial stenosis requiring placement of a stent in 2 patients, and pneumothorax in 1 patient. One patient required tracheostomy. One patient died of massive brain infarction secondary to pulmonary venous thrombosis and embolization. Six patients are intermediate-term survivors, with a follow-up period of 4-24 months. Four of them had at least one episode of rejection each. In all survivors pulmonary functions and quality of life improved and they do not need supplemental oxygen. We conclude that lung transplantation is a viable option for end-stage lung disease. Better selection of patients and perhaps improved immunosuppression agents will further improve outcome in lung transplantation. Shortage of donor supply is currently the limiting factor in successful lung transplantation in Israel.
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PMID:Early experience in lung transplantation. 864 73


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