Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
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Comparative Study
Comparing Mycobacterium massiliense and Mycobacterium abscessus lung infections in cystic fibrosis patients.
Mycobacterium massiliense is closely related to Mycobacterium abscessus and is also a frequent cause of mycobacterial lung disease in patients with cystic fibrosis (CF). There has been no previous investigation of possible differences between M. massiliense and M. abscessus infections in the setting of CF. ⋯ Our data show a particular link between M. massiliense and malnutrition specifically in CF patients. Unlike M. abscessus, the bacteriological response of M. massiliense to combination antibiotic therapies containing clarithromycin was excellent. Distinguishing between M. massiliense and M. abscessus has major clinical implications for CF patients.
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Multicenter Study Observational Study
Epidemiology of nontuberculous mycobacteria among patients with cystic fibrosis in Scandinavia.
Nontuberculous mycobacteria (NTM) are an emerging threat to cystic fibrosis (CF) patients but their epidemiology is not well described. ⋯ NTM are significant CF pathogens and are becoming more prevalent in Scandinavia. MABSC and MAC appear to target distinct patient groups. Having multiple positive cultures despite treatment conveys a poor outcome.
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Comparative Study
Survival benefit of induction immunosuppression in cystic fibrosis lung transplant recipients.
Despite resistant microbes, induction immunosuppression is used in patients with cystic fibrosis (CF) undergoing lung transplantation (LTx). ⋯ Antibody-based induction immunosuppression had a survival benefit in CF patients undergoing LTx.
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Ivacaftor corrects the cystic fibrosis transmembrane conductance regulator (CFTR) gating defect associated with G551D mutation and is quickly becoming an important treatment in patients with cystic fibrosis (CF) due to this genetic mutation. ⋯ Based on our findings, HRCT imaging is a useful tool in monitoring response to ivacaftor therapy that corrects the gating defect associated with the G551D-CFTR mutation.
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Comparative Study
Anti-ETAR and anti-AT1R autoantibodies are elevated in patients with endstage cystic fibrosis.
Autoantibodies against endothelin-1 type A receptor (ETAR) are present in systemic sclerosis complicated by lung fibrosis and pulmonary hypertension. As increased serum levels and local overproduction of endothelin-1 in the airways are reported in cystic fibrosis (CF) patients, we reasoned that anti-ETAR antibodies could be prevalent in endstage CF patients prior to lung transplantation (LTx). Also, ETAR autoantibodies are frequently associated with autoantibodies against the angiotensin II type 1 receptor (AT1R). ⋯ Both autoantibody titers were strongly correlated. We hypothesize that due to prolonged exposure to bacterial infection, increased levels of AT1R and ETAR result in a deregulated immune response causing autoantibody formation. Further research is expedient to elucidate the occurrence of autoantibodies against ETAR and AT1R and their role in disease progression.