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Query: UMLS:C0042373 (vascular disease)
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Ultrastructural study of pulmonary biopsy specimens from patients with fibrotic lung disease disclosed the presence of nuclear inclusions in 1% or less of cuboidal alveolar epithelial cells in 9 of 19 patients, including 6 of 12 patients with idiopathic pulmonary fibrosis, 2 of 3 patients with collagen-vascular disease, and 1 of 3 patients with sarcoidosis. Nuclear inclusions were not observed by ultrastructural study in 5 control patients. The inclusions consisted of masses or aggregates of tubules which probably were derived from the inner nuclear membranes. These tubules were smooth-walled, showed branchings and bifurcations, were composed of single trilaminar membranes, usually had a clear content, and ranged from 500 to 1000 A in diameter. They resembled nuclear tubules which occur in other cell types under conditions of rapid growth or specific hormonal stimulation. Statistically significant differences between the groups of patients with and without nuclear inclusions in cuboidal alveolar epithelial cells were not found with respect to smoking history, degree of fibrosis in the lung biopsy specimen, or the degree of pulmonary physiologic impairment. However, the average age of the patients having nuclear inclusions was significantly greater than that of patients not having nuclear inclusions. In addition, the frequency of indentations in the nuclei of cuboidal alveolar epithelial cells was greater in patients with nuclear inclusions than in patients without nuclear inclusions. Highly significant correlations were observed between the presence of nuclear inclusions and the presence of a) anchoring fibrils and hemidesmosomes along the basal surfaces of alveolar epithelial cells and b) multilayering of the alveolar epithelium.
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PMID:Nuclear inclusions in alveolar epithelium of patients with fibrotic lung disorders. 42 30

Bronchopulmonary lavage (BAL) has provided a fresh dimension for the investigation of pulmonary and multisystem disorders. BAL fluid may be analysed for cells and chemical mediators in the diagnosis and also serially for the management of several granulomatous disorders including sarcoidosis, extrinsic allergic alveolitis, chronic beryllium disease, talc granulomatosis, tuberculosis, Langerhans' histiocytosis-x and Crohn's disease. It may also provide information in fibrosing alveolitis, collagen vascular disease, occupational and drug-induced lung disease, acquired immune deficiency syndrome, bronchial asthma, neoplasia, transplantation, pulmonary alveolar proteinosis and eosinophilic lung disease. This survey analyses the value of BAL and how it has provided a new window for the chest physician.
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PMID:Bronchopulmonary lavage (BAL). A window of the lungs. 134 40

Heart-lung transplantation remains the only therapeutic option for patients with combined end-stage cardiopulmonary disease. Because of the scarcity of heart-lung donors, we have been investigating other surgical alternatives for patients with end-stage vascular and parenchymal lung disease. From June 1989 through June 1991, 48 patients underwent pulmonary transplantation. Seventeen of the 48 patients underwent single lung transplantation. Of the 17 patients in the single lung group, eight patients had pulmonary hypertension and nine had parenchymal lung disease. Four of the 17 patients underwent repair of a cardiac defect with single lung transplantation. One-year actuarial survival was 68%. Pulmonary function has been excellent. The forced expiratory volume in 1 second was 79.6 +/- 13.6 (percent predicted), forced expiratory flow 25%-75% was 72.6 +/- 14.5 (percent predicted), and arterial oxygen tension was 82.8 +/- 10.06 mm Hg when measured at annual follow-up in a group of eight patients without obliterative bronchiolitis. Pulmonary artery pressures of systemic level or greater in the group with pulmonary vascular disease were normal at annual catheterization. Most patients had at least one episode of allograft rejection. Actuarial freedom from rejection at the end of 3 months was 30%. Three of the 17 single lung patients receiving lung lobes were children. Two children received living-related lobe transplants and one neonate received a lobe from a 2-year-old cadaver donor. Single lung transplantation is an effective therapeutic option for selected patients with vascular or parenchymal lung disease. Expanding indications will permit more individuals to receive transplants from the existing donor pool. Living-related and cadaver lobe transplantation will also increase the options available to children in need of lung transplantation.
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PMID:Current trends in lung transplantation. Lobar transplantation and expanded use of single lungs. 140 64

Significant progress has been made since the first successful human heart-lung transplantation (HLT) for pulmonary vascular disease performed in 1981. The refinement of surgical techniques, use of cyclosporin as the main immunosuppressant, technique of distant organ procurement to expand the donor organ pool, and improved diagnosis and management of pulmonary infection and rejection have all contributed to this accomplishment. This has inevitably coincided with the extension of this procedure to other groups of patients with end stage heart and lung disease. Initially, HLT was offered to patients with cardiac disease associated with pulmonary hypertension. Because of the success, consideration was given to transplantation for parenchymal pulmonary diseases, initially pulmonary fibrosis and emphysema, and then suppurative lung disease such as in cystic fibrosis (CF). However, the application of HLT to patients with CF lagged behind because of concern related to the risk of sepsis, the systemic nature of the disease, malnourishment, and fear of recurrence of the epithelial CF defect in the transplanted lungs.
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PMID:Lung transplantation for cystic fibrosis. 145 9

In spite of a shortage of available donors, an increasing number of heart-lung transplantations have been performed within the last decade. This procedure, first limited to patients with pulmonary vascular disease, has been successfully extended to patients with end stage lung disease, including cystic fibrosis. More recently, single lung, double-lung and bilateral single lung transplantation have become other therapeutic options. Better selection of patients and donors as well as improvements in surgical techniques and immunosuppression regimens have contributed to the reduction in the high perioperative mortality experienced in the early stages. Moreover, the introduction of daily spirometry and transbronchial lung biopsies have permitted early and reliable diagnosis of opportunistic infection and rejection. The most serious late complication of lung transplantation is obliterative bronchiolitis and further research is urgently required to improve diagnosis and management of this condition.
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PMID:Lung allograft transplantation: indications, preoperative assessment and postoperative management. 148 75

Diseases of the lung are among the work-related conditions most widely recognized among nonspecialists and the lay public. Five pulmonary conditions for which occupational or environmental exposures are not typically emphasized are reviewed here in their clinical-pathologic context. These are diffuse alveolar hemorrhage, lipoid pneumonitis, granulomatous lung disease, pulmonary alveolar proteinosis, and pulmonary vascular disease.
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PMID:Unusual occupationally related disorders of the lung: case reports and a literature review. 149 26

The pulmonary circulation of the rat is widely used as an animal model for studies of human pulmonary hypertension. It is not difficult to understand its appeal. The species is a small laboratory animal which is readily accommodated in decompression chambers for studies for simulated high altitude. It is also very susceptible to the action of the metabolites of pyrrolizidine alkaloids which rapidly lead to severe pulmonary vascular disease in the absence of intrinsic heart and lung disease, thus suggesting its value as an animal model of primary pulmonary hypertension. However, these obvious advantages of the rat pulmonary circulation are outweighed by the fact that its pathological reactions to hypoxia and noxious dietary agents differ significantly from those found in human disease. This can lead to erroneous conclusions as to the nature of the remodelling of the human pulmonary vasculature in pulmonary hypertension.
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PMID:The rat is a poor animal model for the study of human pulmonary hypertension. 155 66

We retrospectively prepared step sections of nondiagnostic TBLB materials obtained from patients with diffuse or multiple lung disease and evaluated the diagnostic significance of the step section method. Among 112 patients with nondiagnostic TBLB findings, the preparation of step sections resulted in specific findings in seven cases. Step sections were especially useful for the detection of epithelioid granulomas and tumor tissue in patients with sarcoidosis and lymphangiosis carcinomatosa, respectively, but their contribution to the diagnosis of hypersensitivity pneumonitis, collagen-vascular disease, Mycoplasma pneumonia and pneumoconiosis was relatively small. In addition, step sections were useful for the detection of bronchiolitis obliterans affecting respiratory bronchioles. Overall, the examination of step sections was considered to be clinically useful in 30 cases (26.8 percent). Accordingly, the examination of step sections can be recommended before a further diagnostic procedure is chosen, if a TBLB performed in patients with diffuse or multifocal lung disease is nondiagnostic.
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PMID:Step section preparation of transbronchial lung biopsy. Significance in the diagnosis of diffuse lung disease. 191 11

Between December 1983 and April 1989, 222 combined heart and lung transplant operations were performed for treatment of pulmonary vascular disease and parenchymal lung disease at Harefield Hospital. Seventeen of these patients had emphysema, and 11 of them were followed up for a minimum of 1 year. There were nine male and two female patients. Their mean age was 39 (range, 32 to 54) years. Seven had alpha 1-antitrypsin deficiency. Six patients were receiving continuous oxygen therapy, and two patients were dependent on a ventilator. Postoperatively, the patients' lungs were ventilated for a median of 3 days (range, 24 hours to 2 weeks). Two patients subsequently required further periods of ventilation. Immunosuppression consisted of azathioprine and cyclosporine. Three patients also received low-dose oral steroids. There was one early death, which occurred on the second postoperative day. The remaining patients were followed up for 12 to 53 (mean, 22) months. One patient had cytomegalovirus pneumonitis 6 weeks postoperatively that responded to treatment. There was one late death at 14 months after reoperation for treatment of obliterative bronchiolitis. The overall survival was 91% at 1 year. All survivors achieved an excellent level of rehabilitation. It is concluded that the medium-term results of heart and lung transplantation for treatment of emphysema are good and that patients with alpha 1-antitrypsin deficiency may undergo transplant procedures without substitution therapy.
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PMID:Medium-term results of combined heart and lung transplantation for emphysema. 200 66

Light microscopic morphometric techniques have been used to study the lungs in 17 infants and young children aged 2 weeks to 3.5 years who were born prematurely, developed hyaline membrane disease (HMD), and died with chronic lung disease. They were divided into three groups. In group 1 the babies died without recovering from their initial illness at age 2-13 weeks. In group 2 they died, aged 4-15 months, having recovered from the neonatal illness. Neither group showed clinical or pathological evidence of cor pulmonale (CP). In group 3 children died aged 6 months to 3.5 years with CP. None of the 17 patients had classical fibrotic bronchopulmonary dysplasia (BPD) but all had a reduced alveolar number and an increase in bronchial smooth muscle in small airways. All three groups showed an increase in pulmonary arterial medial thickness (P less than 0.001). In group 1 the appearance of the arteries suggested persistance of the fetal state. In group 2 and 3 the vessels had a more mature structure; in group 3 a marked secondary increase in muscularity was found, approaching the thickness seen in fetal life. In groups 2 and 3 muscle extended into smaller arteries than normal. Vein wall thickness was increased. The alveolar to arterial ratio was normal, the alveolar number was reduced, therefore the total number of arteries was reduced in all three groups. Thus, babies who recover from HMD may have significant vascular abnormalities in the absence of CP; conversely, CP if present, does not mean advanced obliterative pulmonary vascular disease. Thus, pulmonary vascular morphology does not correlate with the presence or absence of CP.
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PMID:Pulmonary vascular damage and the development of cor pulmonale following hyaline membrane disease. 214 77


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