Influence of lung transplantation on the essential fatty acid profile in cystic fibrosis
This study sought to evaluate the EFA profile in both the plasma and erythrocyte membrane according to lung status by comparing CF patients with or without a lung transplant.A total of 50 homozygous F508del patients (33 CF patients [CF group] and 17 CF patients with a lung transplant [TX CF group]) were included. In comparison with the CF group, in the plasma, the levels of total n-3, α-linolenic, eicosapentaenoic, and docosahexaenoic acids were higher and the n-6/n-3 ratio was lower in the TX CF group. Yet, these differences were not observed in the erythrocyte membrane. This study supports that lung transplantation improves the EFA profile in the plasma but not in the erythrocyte membrane by means of the different mechanisms suggested in this article.
This article reviews the specific imaging features of CF using conventional imaging modalities (chest radiographs and high-resolution computed tomography [HRCT]) as well as emerging imaging technologies (digital chest tomosynthesis and MR imaging). In addition, the authors review the CF-specific HRCT imaging findings that are essential in the evaluation of these patients in the pre –lung transplant and post–lung transplant settings.
We describe the variability of these infiltrates on CD3/CD20 duplex staining in renal biopsies of long-term functioning allografts, in breast cancer cases, and in lung tissue of cystic fibrosis patients. The assessment in cludes automated cell detection, identification of regions of interest, and classification of lymphocytic clusters according to their degree of organization. We propose a neighborhood feature which considers the occurrence of edges with a certain type in the graph to distinguish between phenotypical ly different immune infiltrates. Our work addresses a medical need and provides a scalable framework that c...
This study reports on the largest single-center experience of lung transplantation for adult and pediatric CF patients. It also highlights the evolution of practice and outcomes over time.
Chronic lung infection with the opportunistic pathogen Pseudomonas aeruginosa (PA) is common in patients with cystic fibrosis (CF) due to reduced mucociliary clearance and is the major contributor to mortality in CF . Colonisation occurs in the paranasal sinuses where the inflammation is predominantly mediated by non-phlogistic immunoglobulin A (IgA) and from where PA is aspirated to the lungs. PA in CF patients is located in the airways and not systemically due to the aerobic conditions in combination with the reduced clearance of mucus in CF making the lungs and sinuses the ideal growth places for PA .
UNDERWEIGHT PATIENTS WITH CYSTIC FIBROSIS HAVE ACCEPTABLE SURVIVAL AFTER LUNG TRANSPLANTATION: A UNOS REGISTRY STUDY. Chest. 2020 Jan 17;: Authors: Ramos KJ, Kapnadak SG, Bradford MC, Somayaji R, Morrell ED, Pilewski JM, Lease ED, Mulligan MS, Aitken ML, Gries CJ, Goss CH Abstract BACKGROUND: Reduced body mass index (BMI) is an absolute contraindication for lung transplantation (LTx) at most centers in the United States (US). Our objective was to quantify post-LTx survival of moderate-to-severely underweight cystic fibrosis (CF) patients (BMI
Conclusions: ULT program allows transplantation in a significant percentage of patients with acceptable results. Pre-operative recipient selection is mandatory to improve clinical outcomes. PMID: 31903264 [PubMed]
Semin Respir Crit Care Med 2019; 40: 842-856 DOI: 10.1055/s-0039-3399554Cystic fibrosis (CF) remains the most common indication for lung transplantation in children and the third most common in adults and has the highest median survival posttransplant for all pretransplant diagnoses. Criteria for transplant in patients with CF vary widely among transplant centers and early referral to multiple centers may be needed to maximize opportunities for lung transplantation. Comorbidities unique to CF such as resistant and atypical pathogens like Burkholderia and Mycobacterium abscessus, and cirrhosis require special consideration ...
Conclusions: Therapeutic drug monitoring-guided meropenem may be a sound new therapeutic option in eradicating multidrug resistant Acinetobacter and offer a novel therapeutic option in the field of personalized medicine. PMID: 31871885 [PubMed]
Authors: Bouchara JP, Le Govic Y, Kabbara S, Cimon B, Zouhair R, Hamze M, Papon N, Nevez G Abstract Introduction: Considered for a long time to be exclusively responsible for chronic localized infections, fungi of the genus Scedosporium have recently received a renewed interest because of their recognition as common colonizing agents of the respiratory tract of patients with cystic fibrosis, and of the description of severe disseminated infections in patients undergoing lung transplantation. Recently, several studies have been carried out on these opportunistic pathogens, which led to some advances in the understan...
Cystic Fibrosis (CF) is a genetic disorder caused by mutations in the gene that encodes for the Cystic Fibrosis Transmembrane Regulator (CFTR) protein, an epithelial ion channel that is crucial in regulating the flow of negatively charged ions across membranes and ensuring adequate hydration of mucus . As a consequence, dysfunction results in a multisystem disorder that seriously impacts upon on the lungs, eventually leading to death or the need for lung transplantation. Lumacaftor/ivacaftor (LUM/IVA) is a combination agent that targets CFTR.