Progressive Loss Of Pulmonary Function Research Articles

Association of body composition and lung function in children with cystic fibrosis

Survival in cystic fibrosis has improved significantly in the last 30 years, with major therapeutic goals of delaying the progressive loss of pulmonary function and maintaining normal growth. Dual-energy X-ray absorptiometry (DEXA) was performed in children with cystic fibrosis (CF) to assess both bone mineral density and body composition. We hypothesised that there would be an association between body composition and pulmonary function in children with CF. Fifty subjects with CF (28 males), mean age 12.7 years, participated in the study. Body composition was determined by DEXA. Body mass index (BMI) was calculated from the ratio of weight/height2 (kg/m2). Lung function was assessed by spirometry. Most patients (78%) had mild lung disease. The mean forced expired volume in 1 sec percent predicted (FEV1% predicted) for the 50 patients was 79.2% (range, 24-117%). There was a strong association between FEV1% predicted and BMI (R=0.59, P=0.0001). Fat-free mass had positive association with pulmonary function tests (R=0.30, P=0.03). Although fat mass showed a positive correlation with pulmonary function, this association did not reach statistical significance. In our group of children with CF and mild lung disease, pulmonary function was more strongly associated with BMI than with fat and fat-free mass.

Asbestos-related pleural disease due to tremolite associated with progressive loss of lung function: serial observations in 123 miners, family members, and residents of Libby, Montana.

The community of Libby, Montana has recently been the focus of national attention secondary to widespread amphibole contamination associated with vermiculite mining and processing. Patients who had occupational and non-occupational exposure to amphibole asbestos in Libby, Montana were evaluated for progressive loss of pulmonary function. Of the 123 patients evaluated, 94 demonstrated average age-corrected accelerated loss per year of vital capacity at 3.2%, total lung capacity (TLC) 2.3%, and DLCO 3.3%. All patients all had predominantly pleural changes with minimal to no interstitial disease. The study demonstrates a progressive loss of pulmonary function in patients exposed to tremolite asbestos.

489. Comparison of AAV2 and AAV2/5 CFTR Vectors in the Lungs of Cynomolgus Macaques

Cystic fibrosis is the most common autosomal recessive disorder in North America, leading to significant morbidity and early mortality. Cystic fibrosis is caused by a defect in the cystic fibrosis transmembrane regulator (CFTR) gene which encodes a chloride channel. The defect in chloride transport in epithelial cells affects a number of organs. The defect in airway epithelium in the lung leads to progressive loss of pulmonary function which is the primary cause of death. rAAV vectors have shown potential utility for treatment of cystic fibrosis. The primary objective of this study was to assess transduction efficiency of several full-length CFTR cDNA rAAV vectors. Two CFTR vectors, one with the AAV2 ITR as a promoter and the other with a small, synthetic 83 base promoter, were evaluated in either AAV type 2 or 5 capsids. Secondary objectives were to collect safety data and to assess the cell types transduced using GFP as a reporter gene. Safety parameters included clinical chemistry, hematology, gross necropsy, histopathology and AAV neutralizing antibody titers.

Case report: The treatment of a chronic parotid cutaneous fistula by the injection of a solution of lipiodol with cyanoacrylate

Cystic fibrosis is an autosomal recessive disorder, and the product of the gene results in an epithelial cell membrane chloride channel defect [2]. The abnormally low water content of mucus leads to blockage of bronchioles and bronchi resulting in infection and inflammation which progresses to bronchiectasis. The clinical course of bronchiectasis is interspersed with episodic acute exacerbations of pulmonary infection leading to peribronchial fibrosis and progressive loss of pulmonary function and respiratory failure. To treat this, double lung transplantation has been successful in cystic fibrosis patients with end stage lung disease [3]. On high resolution CT scans, cystic fibrosis is manifest as bronchiectasis and a mosaic pattern of lung attenuation which reflects small airways disease and air trapping. Expiratory high resolution CT scans accentuate the mosaic pattern of lung attenuation caused by air trapping [4,5], indeed, the expiratory high resolution CT scan is necessary to help to differentiate among different causes of a mosaic pattern of lung attenuation [4]. Air trapping is accentuated by expiration because the areas of decreased attenuation on the CT scan remain unchanged in volume and attenuation, whereas the uninvolved lung shows a homogenous increase in attenuation [5,6]. MinIP images accentuate the differences in lung attenuation due to the inherent high-contrast resolution. As expiration accentuates air trapping, expiratory MinlP images are potentially very useful to demonstrate the mosaic pattern of lung attenuation caused by small airways disease. Air trapping has been demonstrated following forced expiration of subjects with normal pulmonary function [7,8]. Up to 25% of the cross-sectional area of one section of lung has been shown to demonstrate air-trapping in normal subjects [7], in our patient approximately 70% and 60% of the cross-sectional area of the

Role of open lung biopsy for diagnosis in lung transplant recipients: Ten-year experience

Between November 1983 and August 1993, The Toronto Lung Transplant Program performed 153 transplantations in 144 recipients: 53 single-lung transplantations (SLT) and 100 double-lung transplantations (DLT). Thirty-eight open lung biopsies (OLBs) were done in 32 (22% of all recipients): 19 in SLT (36% of SLT) 12 in DLT (12% of DLT), and 1 in a patient who had a SLT and then a double retransplantation. Six recipients underwent OLB twice: 1 DLT, 3 SLT, and 2 who had OLB both before and after retransplantation. Indication for 11 early OLBs (< or = 45 days postoperative) was persistent parenchymal infiltrates. Indications for 27 late OLBs (> 45 days postoperative) included progressive radiologic disease with clinical findings or progressive loss of pulmonary function (18), persistent poor graft function (3), mass or nodules (3), persistent infiltrates without functional loss (2), and persistent lymphocytosis in bronchoalveolar lavage (1). Open lung biopsy confirmed a previous clinical or pathologic diagnosis in 11, suggested a diagnosis in 2, yielded nonspecific information in 16, and provided different diagnosis in 9. New diagnosis that changed therapy was made in 1 of 11 early OLBs and in 8 of 27 late OLBs. These 9 diagnoses included in SLTs: bronchiolitis obliterans (2), bronchiolitis obliterans organizing pneumonia (1), malignant lymphoma (1), and chronic vascular rejection (1) in SLT, and bronchiolitis obliterans organizing pneumonia (3) and Burkholderia cepacia infection (1) in DLT. We conclude that OLB is of little value in the perioperative period but yields useful information in approximately 30% of patients when performed late.