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Query: UMLS:C0206061 (interstitial pneumonia)
 6,105 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Between November 1989 and April 1991, 14 bilateral single lung transplantations (BSLT) were performed at our institution using the technique we have described without omentoplasty and rarely cardiopulmonary bypass. The indications included emphysema (8), cystic fibrosis (3), infected fibrosis (1), alveolar microlithiasis (1) and lymphocytic interstitial pneumonitis (1). Maximum mean pulmonary artery pressure was 53 mmHg and minimal right ventricular ejection fraction was 15%. Two patients experienced bronchial complications: 1 complete left bronchial dehiscence, 1 late partial stenosis which required a temporary insertion of a stent. One patient had a posterior dehiscence which healed spontaneously. Five patients died postoperatively (3 of infection, 1 after a volume mismatch and 1 after a circulating anticoagulant). BSLT is the technique of choice for double lung transplantation in adults and heart lung transplantation has very few indications in infected end-stage pulmonary disease. We hope that modification of our immunosuppressive regimen will decrease postoperative mortality.
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PMID:Bilateral single lung transplantation. Complications and results in 14 patients. 851 59

Previous studies have suggested that immune mechanisms contribute to lung injury in cystic fibrosis (CF); however, there have been no comprehensive studies of immunofluorescent staining patterns in CF lung tissue. We performed immunofluorescence (IF) studies for immunoglobulins, C3, and fibrinogen on autopsy frozen lung tissue from 21 CF patients. Results were compared with lung findings in patients without CF. In CF-derived lung tissue fibrinogen was ubiquitous along the alveolar wall, alveolar space, and interstitium. Free immunoglobulin G (IgG) and IgA coated the alveolar surface segmentally in 14 and 6 cases, respectively. Unequivocal interstitial deposits were infrequent and IgM was present in blood vessels in one patient only. Intra-alveolar and interstitial inflammatory cells demonstrated cytoplasmic IgG, IgA, and IgM, respectively, in 18, 14, and 6 patients. C3 was seen only segmentally along the alveolar wall in two patients and in blood vessels in one. Antinuclear antibody (ANA) staining of interstitial cells for C3 and immunoglobulins was seen in five patients, four of whom had interstitial pneumonitis. Insignificant amounts of alveolar or interstitial fibrinogen and immunoglobulins in inflammatory cells were seen in controls in the absence of lung inflammation. The IF patterns were similar in the inflammatory lesions of CF and control specimens. The IF patterns observed in CF lung tissue are consistent with nonspecific vascular leakage and chronic inflammation with little evidence of immune complex deposition in the interstitium or blood vessels. This study confirms previous reports of ANA activity in CF patients, although the significance of this finding is unknown.
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PMID:Immunofluorescence studies of lung tissue in cystic fibrosis. 140 35

We recently described a technique for bilateral sequential lung transplantation that replaces the en bloc double-lung operation, a procedure that was accompanied by frequent problems with airway healing. Twenty-seven patients have undergone 28 bilateral sequential lung transplantations over the past 14 months. Eighteen patients had transplantation because of end-stage emphysema; 6, cystic fibrosis; and 1 each, obliterative bronchiolitis, usual interstitial pneumonitis with pulmonary fibrosis, and bronchiectasis. Cardiopulmonary bypass was used electively in the first 5 patients until it was recognized that the procedure could be done safely without it, and in only 3 additional recipients has it been employed. Mean ischemic time for the first lung was 276 +/- 43 minutes and for the second lung, 410 +/- 64 minutes. There have been five deaths, three in the postoperative period (11% operative mortality) and two late. The other patients are alive and well and do not require oxygen 2 to 15 months after transplantation. Mean forced expiratory volume in 1 second rose from 16% +/- 8% of predicted to 84% +/- 17% at 12 weeks. Six-minute walk values increased from a mean of 251 +/- 91 m to 666 +/- 42 m at 24 weeks. The excellent exposure afforded to both hemithoraces by the thoracosternotomy incision and the rare need of cardiopulmonary bypass have allowed us to offer the option of transplantation to patients who formerly would have been turned down because of previous pulmonary resection or pleurectomy. On four occasions, ventilator-dependent patients underwent successful transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Bilateral sequential lung transplantation: the procedure of choice for double-lung replacement. 189 30

Interstitial pneumonia is a poorly understood variant of lung injury in patients with cystic fibrosis (CF). The authors identified prominent interstitial pneumonia (defined as an inflammatory reaction predominantly involving alveolar septa) in 9 of 43 autopsied patients with CF. Lungs from these nine were studied by light microscopy to determine the histopathologic features, clinicopathologic correlates, and potential causes of CF-associated interstitial pneumonia. Two histologic variants were identified, alveoloseptal (in which the inflammatory reaction was confined to the alveolar wall) and organizing (in which intraalveolar granulation tissue was present in addition to septal inflammation), which respectively affected four and five patients. Cholesterol deposits and capillary proliferation were prominent associated features in some patients. Interstitial pneumonia was not diagnosed antemortem and was identified on chest roentgenogram in only two patients. There were no distinctive clinical features associated with different histologic subtypes, nor did histologic or clinical data identify a specific cause(s) of interstitial pneumonia. The authors conclude that CF-associated interstitial pneumonia, although usually an incidental finding at autopsy, may potentially contribute to respiratory impairment and death. Although there are multiple possible causes of interstitial lung injury associated with CF, the authors were unable to identify, with certainty, the pathogenesis of interstitial pneumonia in any patient in this study.
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PMID:The pathologic characteristics of interstitial pneumonia cystic fibrosis. A retrospective autopsy study. 271 52

There are relatively few observations on the influence of pregnancy in chronic respiratory illness, excluding asthma and interstitial pneumonia. Chronic airflow obstruction only presents at a relatively advanced age, past the menopause. Chronic respiratory failure due to restrictive lung disorders appears well tolerated as long as the vital capacity is greater than one litre. Mucoviscidosis has been the object of more detailed studies since the management of this disorder now raises hopes of survival compatible with pregnancy.
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PMID:[Chronic respiratory diseases and their decompensation during pregnancy (asthma excluded)]. 304

We studied lungs at autopsy from 40 patients with cystic fibrosis (CF) to determine the structural and clinicopathologic features of pneumonia associated with Pseudomonas cepacia respiratory tract colonization. Three clinical groups were identified: group A included 11 patients exhibiting a fulminant course following P cepacia colonization; group B included 20 patients who declined slowly following colonization; and group C included nine patients without P cepacia colonization. Acute pneumonia occurred in all groups but was most extensive and necrotizing in group A. Chronic lobular pneumonia involved all groups equally, whereas interstitial pneumonia predominated in group B. Diffuse alveolar damage occurred infrequently in all groups. Combinations of structural patterns were frequently seen. We conclude that, although there is great overlap in the structural appearance of pneumonia among patients with CF with different bacterial colonization histories, the evidence suggests that P cepacia is a cause of necrotizing pneumonia in some patients. Factors that predispose to this fulminant lung infection are poorly understood.
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PMID:Pseudomonas cepacia-associated pneumonia in cystic fibrosis. Relation of clinical features to histopathologic patterns of pneumonia. 333 28

Animal models are of two types those that occur spontaneously and those that the scientist produces by artefact. One value of spontaneously occurring models is that if pathogenetic mechanisms are identified, they give new leads for the study of human disease. There is a need for spontaneously occurring examples of so-called primary or idiopathic pulmonary fibrosis, pulmonary hypertension (arterial or venous), and emphysema. Acquired or artefactual models of each of these conditions are available and have led to better understanding of the pathological changes, but they have not led to identification of the basic or primary abnormality. A naturally occurring model of cystic fibrosis could be a major event in our control of this disease. A spontaneously occurring form of asthma is needed as a bridge between experiment and patient. Artefactual models that are needed are of bronchopulmonary dysplasia and shock lung. There is probably enough agreement--but only just--on the nature of bronchopulmonary dysplasia for specific needs to be identified. Here the questions concern the choice of an appropriate species--or several--in which to study the premature lung and its adaptation to air breathing and supportive therapy. Knowledge of comparative anatomy and physiology must influence choice of species for certain models. For adult respiratory failure, or shock lung, a model is needed that progresses to pulmonary hypertension. Spontaneous models of interstitial pneumonia and of infection, both viral and bacterial, are needed. An animal model of a disease is only as useful as the questions we ask of it.
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PMID:Needs for animal models of human diseases of the respiratory system. 696 87

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

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


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