BACKGROUND Cystic fibrosis transmembrane conductance regulator(CFTR)modulators significantly improve pulmonary function in patients with cystic fibrosis(CF)but the effect on hepatobiliary outcomes remains unknown.We h...BACKGROUND Cystic fibrosis transmembrane conductance regulator(CFTR)modulators significantly improve pulmonary function in patients with cystic fibrosis(CF)but the effect on hepatobiliary outcomes remains unknown.We hypothesized that CF patients on CFTR modulators would have a decreased incidence of cirrhosis compared to patients not on CFTR modulators or on ursodiol.AIM To investigate the effect of CFTR modulators on the development of cirrhosis in patients with CF.METHODS A retrospective analysis was performed using Truven MarketScan from January 2012 through December 2017 including all patients with a diagnosis of CF.Patients were excluded if they underwent a liver transplantation or if they had other etiologies of liver disease including viral hepatitis or alcohol use.Subjects were grouped by use of CFTR modulators,ursodiol,dual therapy,or no therapy.The primary outcome was development of cirrhosis.Kaplan-Meier curves estimated the incidence of cirrhosis and log-rank tests compared incidence curves between treatment groups.RESULTS A total of 7201 patients were included,of which 955(12.6%)used a CFTR modulator,529(7.0%)used ursodiol,105(1.4%)used combination therapy,and 5612(74.3%)used neither therapy.The incidence of cirrhosis was 0.1%at 1 year and 0.7%at 4 years in untreated patients,5.9%and 10.1%in the Ursodiol group,and 1.0%and 1.0%in patients who received both therapies.No patient treated with CFTR modulators alone developed cirrhosis.Patients on CFTR modulators alone had lower cirrhosis incidence than untreated patients(P=0.05),patients on Ursodiol(P<0.001),and patients on dual therapy(P=0.003).The highest incidence of cirrhosis was found among patients treated with Ursodiol alone,compared to untreated patients(P<0.001)or patients on Ursodiol and CFTR modulators(P=0.01).CONCLUSION CFTR modulators are associated with a reduction in the incidence of cirrhosis compared to other therapies in patients with CF.展开更多
BACKGROUND Symptomatic biliary and gallbladder disorders are common in adults with cystic fibrosis(CF)and the prevalence may rise with increasing CF transmembrane conductance regulator modulator use.Cholecystectomy ma...BACKGROUND Symptomatic biliary and gallbladder disorders are common in adults with cystic fibrosis(CF)and the prevalence may rise with increasing CF transmembrane conductance regulator modulator use.Cholecystectomy may be considered,but the outcomes of cholecystectomy are not well described among modern patients with CF.AIM To determine the risk profile of inpatient cholecystectomy in patients with CF.METHODS The Nationwide Inpatient Sample was queried from 2002 until 2014 to investigate outcomes of cholecystectomy among hospitalized adults with CF compared to controls without CF.A propensity weighted sample was selected that closely matched patient demographics,patient’s individual comorbidities,and hospital characteristics.The propensity weighted sample was used to compare outcomes among patients who underwent laparoscopic cholecystectomy.Hospital outcomes of open and laparoscopic cholecystectomy were compared among adults with CF.RESULTS A total of 1239 inpatient cholecystectomies were performed in patients with CF,of which 78.6%were performed laparoscopically.Mortality was<0.81%,similar to those without CF(P=0.719).In the propensity weighted analysis of laparoscopic cholecystectomy,there was no difference in mortality,or pulmonary or surgical complications between patients with CF and controls.After adjusting for significant covariates among patients with CF,open cholecystectomy was independently associated with a 4.8 d longer length of stay(P=0.018)and an$18449 increase in hospital costs(P=0.005)compared to laparoscopic cholecystectomy.CONCLUSION Patients with CF have a very low mortality after cholecystectomy that is similar to the general population.Among patients with CF,laparoscopic approach reduces resource utilization and minimizes post-operative complications.展开更多
文摘BACKGROUND Cystic fibrosis transmembrane conductance regulator(CFTR)modulators significantly improve pulmonary function in patients with cystic fibrosis(CF)but the effect on hepatobiliary outcomes remains unknown.We hypothesized that CF patients on CFTR modulators would have a decreased incidence of cirrhosis compared to patients not on CFTR modulators or on ursodiol.AIM To investigate the effect of CFTR modulators on the development of cirrhosis in patients with CF.METHODS A retrospective analysis was performed using Truven MarketScan from January 2012 through December 2017 including all patients with a diagnosis of CF.Patients were excluded if they underwent a liver transplantation or if they had other etiologies of liver disease including viral hepatitis or alcohol use.Subjects were grouped by use of CFTR modulators,ursodiol,dual therapy,or no therapy.The primary outcome was development of cirrhosis.Kaplan-Meier curves estimated the incidence of cirrhosis and log-rank tests compared incidence curves between treatment groups.RESULTS A total of 7201 patients were included,of which 955(12.6%)used a CFTR modulator,529(7.0%)used ursodiol,105(1.4%)used combination therapy,and 5612(74.3%)used neither therapy.The incidence of cirrhosis was 0.1%at 1 year and 0.7%at 4 years in untreated patients,5.9%and 10.1%in the Ursodiol group,and 1.0%and 1.0%in patients who received both therapies.No patient treated with CFTR modulators alone developed cirrhosis.Patients on CFTR modulators alone had lower cirrhosis incidence than untreated patients(P=0.05),patients on Ursodiol(P<0.001),and patients on dual therapy(P=0.003).The highest incidence of cirrhosis was found among patients treated with Ursodiol alone,compared to untreated patients(P<0.001)or patients on Ursodiol and CFTR modulators(P=0.01).CONCLUSION CFTR modulators are associated with a reduction in the incidence of cirrhosis compared to other therapies in patients with CF.
文摘BACKGROUND Symptomatic biliary and gallbladder disorders are common in adults with cystic fibrosis(CF)and the prevalence may rise with increasing CF transmembrane conductance regulator modulator use.Cholecystectomy may be considered,but the outcomes of cholecystectomy are not well described among modern patients with CF.AIM To determine the risk profile of inpatient cholecystectomy in patients with CF.METHODS The Nationwide Inpatient Sample was queried from 2002 until 2014 to investigate outcomes of cholecystectomy among hospitalized adults with CF compared to controls without CF.A propensity weighted sample was selected that closely matched patient demographics,patient’s individual comorbidities,and hospital characteristics.The propensity weighted sample was used to compare outcomes among patients who underwent laparoscopic cholecystectomy.Hospital outcomes of open and laparoscopic cholecystectomy were compared among adults with CF.RESULTS A total of 1239 inpatient cholecystectomies were performed in patients with CF,of which 78.6%were performed laparoscopically.Mortality was<0.81%,similar to those without CF(P=0.719).In the propensity weighted analysis of laparoscopic cholecystectomy,there was no difference in mortality,or pulmonary or surgical complications between patients with CF and controls.After adjusting for significant covariates among patients with CF,open cholecystectomy was independently associated with a 4.8 d longer length of stay(P=0.018)and an$18449 increase in hospital costs(P=0.005)compared to laparoscopic cholecystectomy.CONCLUSION Patients with CF have a very low mortality after cholecystectomy that is similar to the general population.Among patients with CF,laparoscopic approach reduces resource utilization and minimizes post-operative complications.
