The Role of Cystic Fibrosis Transmembrane Conductance Regulator Modulators After Liver Transplantation in Persons With Cystic Fibrosis.

Despite advances in treatment for cystic fibrosis (CF), liver disease remains a major contributor to morbidity and mortality for persons with CF. Therefore, liver transplantation may be considered in end-stage CF-related liver disease. We present a young patient with CF who underwent solo liver transplantation and has successfully restarted on elexacaftor/tezacaftor/ivacaftor without significant pulmonary or hepatic complications after transplant.

Preventive service utilization among adults with cystic fibrosis covered by private insurance is comparable to the general population.

BACKGROUND: As the life expectancy of the cystic fibrosis (CF) population is lengthening with modulator therapies, diligent age-appropriate screening and preventive care are increasingly vital for long-term health and wellbeing. METHODS: We performed a retrospective analysis comparing rates of receiving age- and sex-appropriate preventive services by commercially insured adult people with CF (PwCF) and adults without CF from the general population (GP) via the Truven Health MarketScan database (2012-2018). RESULTS: We captured 25,369 adults with CF and 488,534 adults from the GP in the United States. Comparing these groups, we found that 43% versus 39% received an annual preventive visit, 28% versus 28% were screened for chlamydia, 38% versus 37% received pap smears every 3 years (21-29-year-old females), 33% versus 31% received pap smears every 5 years (30-64-year-old females), 55% versus 44% received mammograms, 23% versus 21% received colonoscopies, and 21% versus 20% received dyslipidemia screening (all screening rates expressed per 100 person-years). In age-stratified analysis, 18-27-year-old PwCF had a lower rate of annual preventive visits compared to adults in the same age group of the GP (27% versus 42%). CONCLUSIONS: We discovered a comparable-to-superior rate of preventive service utilization in adults with CF relative to the GP, except in young adulthood from 18-27 years. Our findings establish the importance of meeting the primary care needs of adults with CF and call for development of strategies to improve preventive service delivery to young adults.

Cystic fibrosis transmembrane conductance regulator modulators and the exocrine pancreas: A scoping review.

BACKGROUND: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators improve pulmonary outcomes in subjects with cystic fibrosis (CF); however, the effects on pancreatic manifestations are not well characterized. We hypothesized that CFTR modulators would improve measures of exocrine pancreatic function and outcomes. METHODS: We performed a systematic search to identify studies reporting measures of the exocrine pancreas in humans treated with CFTR modulators. Only studies reporting baseline and on-treatment assessments were included. RESULTS: Of 630 identified studies, 41 met inclusion criteria. CFTR modulators reduced acute pancreatitis events by 85% overall (rate ratio 0.15, 95% confidence interval (CI) 0.04, 0.52), with a greater effect seen in the subgroup with pancreas sufficient CF (PS-CF) (rate ratio 0.13 (95% CI 0.03, 0.53). Among 293 subjects with baseline and on-treatment evaluation of pancreas sufficiency, 253 were pancreas insufficient at baseline and 54 (21.3%) converted to pancreas sufficiency. Of 32 subjects with baseline FE-1 values <200 mcg/g, 16 (50%) increased to ≥200 mcg/g. Serum trypsin decreased by a mean of 565.9 ng/mL (standard deviation (SD) 311.8), amylase decreased by 38.2 U/L (SD 57.6), and lipase decreased by 232.3 U/L (SD 247.7). CONCLUSIONS: CFTR modulator use reduces acute pancreatitis frequency and improves indirect measures of exocrine pancreas function. Future interventional studies that evaluate the mechanism and impact of CFTR modulators on acute pancreatitis and pancreas sufficiency in patients with CFTR dysfunction are warranted.

Cystic fibrosis patients on cystic fibrosis transmembrane conductance regulator modulators have a reduced incidence of cirrhosis.

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.

Lung transplantation in the septuagenarian can be successfully performed though long-term results impacted by diseases of aging.

BACKGROUND: Advanced age is considered a risk factor for lung transplantation (LTX). We sought to evaluate the long-term outcomes of LTX in the septuagenarian. METHODS: LTX recipients in the UNOS transplant registry (May 1, 2005-June 12, 2020) were stratified into 18-59, 60-69, and > = 70 years of age. Recipient and transplant characteristics were evaluated for survival, cause of death (COD), length of stay (LOS), and complications. A Kaplan-Meier analysis examined long-term survival for all patients stratified by age, specifically looking at cause of death. RESULTS: A total of 27 632 recipients were identified. As recipients aged, we found a decrease in proportion of cystic fibrosis and an increase in restrictive disease while obstructive disease peaked in the 60-69yo cohort (P < .001). Septuagenarians had higher rates of single LTX, male gender, and white race (P < .001). Older recipients had significantly longer donor recovery distances traveled with paradoxical shorter ischemic times, shorter hospital LOS and were transplanted at higher volume centers. There was no difference with in-hospital mortality among groups (P = .5). Acute rejection during initial hospitalization, rejection within 1 year, and post-transplant dialysis incidence decreased with age. Graft failure was a common COD in younger patients while malignancy and cardio/cerebrovascular diseases were common COD in > = 70yo. CONCLUSION: Select septuagenarian LTX candidates may be safely transplanted with relatively few complications. Immunosenescence and conditions of the aged are likely contributing factors to the decreased rejection and graft failure observations. Septuagenarians should not be excluded from LTX consideration based solely on age. Transplantation in septuagenarians should only be done in very selected patients (screened for malignancies and atherosclerotic disease) and these recipients should be carefully followed after transplantation because of these risk factors.

Outcomes of inpatient cholecystectomy among adults with cystic fibrosis in the United States.

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.

Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use Is Associated With Reduced Pancreatitis Hospitalizations in Patients With Cystic Fibrosis.

INTRODUCTION: Acute pancreatitis (AP) occurs among patients with pancreas-sufficient cystic fibrosis (PS-CF) but is reportedly less common among patients with pancreas-insufficient cystic fibrosis (PI-CF). The incidence of AP may be influenced by cystic fibrosis transmembrane conductance regulator (CFTR) modulator use. We hypothesized that CFTR modulators would reduce AP hospitalizations, with the greatest benefit in PS-CF. METHODS: MarketScan (2012-2018) was queried for AP hospitalizations and CFTR modulator use among patients with CF. Multivariable Poisson models that enabled crossover between CFTR modulator treatment groups were used to analyze the rate of AP hospitalizations on and off therapy. Pancreas insufficiency was defined by the use of pancreas enzyme replacement therapy. RESULTS: A total of 10,417 patients with CF were identified, including 1,795 who received a CFTR modulator. AP was more common in PS-CF than PI-CF (2.9% vs 0.9%, P = 0.007). Overall, the observed rate ratio of AP during CFTR modulator use was 0.33 (95% confidence interval [CI] 0.10, 1.11, P = 0.07) for PS-CF and 0.38 (95% CI 0.16, 0.89, P = 0.03) for PI-CF, indicating a 67% and 62% relative reduction in AP hospitalizations, respectively. In a subset analysis of 1,795 patients who all had some CFTR modulator use, the rate ratio of AP during CFTR modulator use was 0.36 (95% CI 0.13, 1.01, P = 0.05) for PS-CF and 0.53 (95% CI 0.18, 1.58, P = 0.26) for PI-CF. DISCUSSION: CFTR modulator use is associated with a reduction in AP hospitalizations among patients with CF. These observational data support the prospective study of CFTR modulators to reduce AP hospitalizations among patients with CF.

National Trends of Hospitalizations in Cystic Fibrosis Highlight a Need for Pediatric to Adult Transition Clinics.

OBJECTIVES: We hypothesized that hospitalizations in cystic fibrosis (CF) would reflect the development of age-related comorbidities. METHODS: A retrospective analysis was performed using the Nationwide Inpatient Sample (2002-2017). Hospitalizations for which the principal diagnosis was CF were analyzed regarding age at discharge and presence of comorbidities. Trends were assessed for significance using the Cochran-Armitage test. RESULTS: The mean age of patients hospitalized for CF increased from 19.7 years in 2002 to 23.0 years in 2017 (P = 0.017). Several comorbidities are more than 10 times more prevalent among adults as compared with children, including congestive heart failure, substance abuse, and chronic kidney disease (P < 0.001). In addition, diabetes with chronic complications was more prevalent in adults than children (10.0% vs 3.9%; P < 0.001), as was hypertension (7.2% vs 1.3%; P < 0.001) and osteoporosis (10.2% vs 1.9%; P < 0.001). More than 65% of CF hospitalizations in 2017 were in individuals older than 18 years. CONCLUSIONS: Hospitalizations for adults with CF are increasing, and individuals with CF are developing age-related comorbidities. Providers equipped to manage the health care needs of adults need to be ready and able to care for this unique and growing patient population.

Association of Preoperative Pneumonia With Postsurgical Morbidity and Mortality in Children.

BACKGROUND: Pneumonia is a common lower respiratory tract infection (LRI) and the leading cause of pediatric hospitalization in the United States. Given its frequency, children with pneumonia may require surgery during their hospital course. This poses serious anesthetic and surgical challenges because preoperative pulmonary status is among the most important risk factors for postoperative complications. Although recent adult data indicated that preoperative pneumonia was associated with poor surgical outcomes, comparable data in children are lacking. Therefore, our objective was to investigate the association of preoperative pneumonia with postoperative mortality and morbidity in children. METHODS: Using the National Surgical Quality Improvement Program database, we assembled a retrospective cohort of children (<18 years) who underwent inpatient surgery between 2012 and 2015. Our primary outcome was the time to all-cause 30-day postoperative mortality that we evaluated using Cox proportional hazards regression models. For the secondary outcomes, including 30-day postoperative morbidity events, we used Fine-Gray models to account for competing risk by mortality. We also evaluated the association of preoperative pneumonia with duration of postoperative mechanical ventilation and postoperative hospital length of stay. We used propensity score weighting methods to adjust for potential confounding factors, whose distributions differ across the pneumonia groups. RESULTS: Among 153,242 children who underwent inpatient surgery, 0.7% (n = 867) had preoperative pneumonia. Compared with those without preoperative pneumonia, children with preoperative pneumonia had a higher risk of mortality throughout the 30-day postoperative period (hazard ratio [HR], 4.10; 95% confidence intervals [CI], 2.42-6.97; P < .001). Although not statistically significant, children with preoperative pneumonia were twice as likely to develop cardiovascular complications compared to children without preoperative pneumonia (HR, 2.10; 95% CI, 1.17-3.75; P = .012). Furthermore, children with preoperative pneumonia had longer duration of postoperative ventilation (incidence rate ratio, 1.47; 95% CI, 1.26-1.71; P < .001). Finally, children with preoperative pneumonia were estimated to be 56% less likely to be discharged within the 30 days following surgery, compared to children without preoperative pneumonia (HR, 0.44; 95% CI, 0.40-0.47; P < .001). CONCLUSIONS: Preoperative pneumonia was strongly associated with increased incidence of postoperative mortality and complications in children. Clinicians should make concerted efforts to screen for preoperative pneumonia and consider whether proceeding with surgery is the most expedient course of action. Our findings may be helpful in preoperative discussions with parents of children with preoperative pneumonia for risk stratification and postoperative resource allocation purposes.

Lung Transplant Index: A Quality Improvement Initiative.

Limited long-term survival is a recognized problem in adolescent/young adult lung transplant recipients. A quality improvement (QI) initiative included the development of a Lung Transplant Index (LTI) composed of key elements that we used as a comprehensive approach to screen and identify potential harms in this at-risk patient population. METHODS: A single-center, uncontrolled QI study was completed from January 2014 to February 2019. The elements of the LTI are events that should have occurred within the most recent 12 months. If an element did not occur, it was counted as a missed element of preventing harm and summated later serving as the LTI score. Implementation of the LTI occurred on January 1, 2015, with a retrospective chart review of patients seen in clinic the prior year serving as baseline measures for comparison. RESULTS: The year before implementing the LTI, numerous opportunities failed to identify preventable harm in our adolescent/young adult lung transplant population. The LTI resulted in a sustained reduction of these missed opportunities without negatively influencing patient/family satisfaction with lengthening of the clinic visit. CONCLUSIONS: A single-center QI initiative identified preventable harms in an adolescent/young adult lung transplant population and reduced the number of preventable harm elements not performed. Future work is needed to determine if this type of QI initiative is associated with less healthcare utilization.

Quality Improvement Initiative for Assessing Allografts after Lung Transplantation.

INTRODUCTION: The histologic evaluation of lung allografts after transbronchial biopsy (TBBx) is a key component of the clinical care of lung transplant recipients. With established guidelines on diagnosing allograft rejection, no specific recommendations exist on timeliness to reaching a diagnosis and initiating therapy. A quality improvement initiative focused on 3 key stages of achieving a prompt diagnosis of acute cellular rejection including tissue processing, interpretation, and notification to the treating transplant pulmonologist was initiated to minimize time to treatment onset. METHODS: We completed a single-center cohort study on all surveillance and clinically indicated TBBx from September 2006 to March 2018. The rapid tissue processing, interpretation, and notification system was instituted in March 2011 with data before this date serving as baseline. RESULTS: We enrolled 28 patients who underwent 210 TBBx (1 excluded due to unknown notification date). Thirty-eight TBBx were included at baseline before implementation of the rapid tissue processing and communication system; 171 were included after implementation. Median time to notification following the change was 0 days (interquartile range, 0-1) compared with 1 day (interquartile range, 1-1) before the change (P < 0.001). After the change, same-day notification increased, with 110 (64%) TBBx resulting in same-day notification compared with 0 before (P < 0.001). We initiated treatment of acute cellular rejection on the day of diagnosis for the entire cohort. CONCLUSIONS: This quality improvement initiative resulted in more efficient analysis of TBBx of allografts in lung transplant recipients and faster communication of results to the clinical team.

Risks associated with lung transplantation in cystic fibrosis patients.

INTRODUCTION: Survival after lung transplantation lags behind outcomes of other solid organ transplants, and complications from lung transplant are the second most common cause of death in cystic fibrosis. Evolving surgical techniques, therapeutics, and perioperative management have improved short-term survival after lung transplantation, yet have not translated into significant improvement in long-term mortality. Areas covered: We review risk factors for poor long-term outcomes among patients with cystic fibrosis undergoing lung transplantation to highlight areas for improvement. This includes reasons for organ dysfunction, complications of immunosuppression, further exacerbation of extrapulmonary complications of cystic fibrosis, and quality of life. A literature search was performed using PubMed-indexed journals. Expert commentary: There are multiple medical and socioeconomic barriers that threaten long-term survival following lung transplant for patients with cystic fibrosis. An understanding of the causes of each could elucidate treatment options. There is a lack of prospective, multicenter, randomized control trials due to cost, complexity, and feasibility. Ongoing prospective studies should be reserved for the most promising interventions identified in retrospective studies in order to improve long-term outcomes.

The expression of Mirc1/Mir17-92 cluster in sputum samples correlates with pulmonary exacerbations in cystic fibrosis patients.

INTRODUCTION: Cystic fibrosis (CF) is a multi-organ disorder characterized by chronic sino-pulmonary infections and inflammation. Many patients with CF suffer from repeated pulmonary exacerbations that are predictors of worsened long-term morbidity and mortality. There are no reliable markers that associate with the onset or progression of an exacerbation or pulmonary deterioration. Previously, we found that the Mirc1/Mir17-92a cluster which is comprised of 6 microRNAs (Mirs) is highly expressed in CF mice and negatively regulates autophagy which in turn improves CF transmembrane conductance regulator (CFTR) function. Therefore, here we sought to examine the expression of individual Mirs within the Mirc1/Mir17-92 cluster in human cells and biological fluids and determine their role as biomarkers of pulmonary exacerbations and response to treatment. METHODS: Mirc1/Mir17-92 cluster expression was measured in human CF and non-CF plasma, blood-derived neutrophils, and sputum samples. Values were correlated with pulmonary function, exacerbations and use of CFTR modulators. RESULTS: Mirc1/Mir17-92 cluster expression was not significantly elevated in CF neutrophils nor plasma when compared to the non-CF cohort. Cluster expression in CF sputum was significantly higher than its expression in plasma. Elevated CF sputum Mirc1/Mir17-92 cluster expression positively correlated with pulmonary exacerbations and negatively correlated with lung function. Patients with CF undergoing treatment with the CFTR modulator Ivacaftor/Lumacaftor did not demonstrate significant change in the expression Mirc1/Mir17-92 cluster after six months of treatment. CONCLUSIONS: Mirc1/Mir17-92 cluster expression is a promising biomarker of respiratory status in patients with CF including pulmonary exacerbation.

Modifications of Pseudomonas aeruginosa cell envelope in the cystic fibrosis airway alters interactions with immune cells.

Pseudomonas aeruginosa is a ubiquitous environmental organism and an opportunistic pathogen that causes chronic lung infections in the airways of cystic fibrosis (CF) patients as well as other immune-compromised individuals. During infection, P. aeruginosa enters the terminal bronchioles and alveoli and comes into contact with alveolar lining fluid (ALF), which contains homeostatic and antimicrobial hydrolytic activities, termed hydrolases. These hydrolases comprise an array of lipases, glycosidases, and proteases and thus, they have the potential to modify lipids, carbohydrates and proteins on the surface of invading microbes. Here we show that hydrolase levels between human ALF from healthy and CF patients differ. CF-ALF influences the P. aeruginosa cell wall by reducing the content of one of its major polysaccharides, Psl. This CF-ALF induced Psl reduction does not alter initial bacterial attachment to surfaces but reduces biofilm formation. Importantly, exposure of P. aeruginosa to CF-ALF drives the activation of neutrophils and triggers their oxidative response; thus, defining human CF-ALF as a new innate defense mechanism to control P. aeruginosa infection, but at the same time potentially adding to the chronic inflammatory state of the lung in CF patients.

Safety of endoscopic sinus surgery in children with cystic fibrosis.

INTRODUCTION: Data on the safety of endoscopic sinus surgery (ESS) are limited in children with cystic fibrosis (CF). We used a multi-institutional surgical registry to examine ESS outcomes in children with CF. METHODS: The 2014-2015 American College of Surgeons' National Surgical Quality Improvement Program-Pediatric database was queried for patients age <18 years undergoing elective ESS. Prolonged hospital stay (>1 day), 30-day readmission, and 30-day unplanned reoperation were compared according to presence of CF diagnosis. RESULTS: The data included 213 children with CF (age 10 ± 5 years, 105/108 male/female) and 821 children without CF (age 10 ± 5 years, 504/317 male/female). CF patients were more likely than non-CF patients to require prolonged hospital stay (30% vs. 9%, p < 0.001), yet had similar rates of readmission (6% vs. 4%; p = 0.189) and reoperation (0 vs. 1%; p = 0.133). All readmissions but one among CF patients were unrelated to ESS. In the non-CF cohort, reasons for ESS-related readmissions included recurrence of sinusitis, postoperative pain, and bleeding. CONCLUSIONS: We demonstrate the safety of ESS in the largest cohort of children with CF reviewed to date. Multi-institutional review of ESS safety may contribute to monitoring expansion of this intervention in children with CF.

Age-Dependent Gender Disparities in Post Lung Transplant Survival Among Patients With Idiopathic Pulmonary Fibrosis.

BACKGROUND: The influence of age and gender on survival after lung transplant in patients with idiopathic pulmonary fibrosis (IPF) is not well defined. METHODS: The United Network for Organ Sharing database was queried to identify IPF patients receiving lung transplant between 2005 and 2015. RESULTS: There were 6,677 patients receiving lung transplant between May 2015 and June 2015 who met the inclusion criteria, predominantly males (n = 4,769, 71%). Within 1 year posttransplant, the survival curves of male and female recipients diverged, with male recipients having significantly worse survival (log-rank test p = 0.008). Univariate Cox proportional hazards regressions demonstrated no gender difference in survival below age 65 years (HR = 1.051; 95% CI = 0.945, 1.168; p = 0.362) but a significant increase in mortality hazard associated with male gender among patients age 65 years and older (HR = 1.161; 95% CI = 1.000, 1.347; p = 0.049). Multivariable Cox regression accounting for age modulation of the gender effect further demonstrated the emergence of a male disadvantage in post-transplant survival above age 65 years at transplantation. CONCLUSIONS: In patients with IPF receiving lung transplant at greater than 65 years of age, male gender is associated with significantly increased risk for death, so referral for lung transplant in IPF should be considered early in the disease course.

The effect of the affordable care act dependent coverage provision on patients with cystic fibrosis.

BACKGROUND: The Patient Protection and Affordable Care Act (ACA), enacted in 2010, expanded private insurance coverage of young adults through the dependent coverage provision. This policy's implications for patients with cystic fibrosis (CF) are unknown. METHODS: The CF Foundation Patient Registry was used to identify patients seen at CF centers, 3 years before and after ACA implementation. Patients were grouped according to eligibility for the dependent care provision (18-25 years old in 2010) or ineligibility (26-35 years old). Year-level difference-in-difference logistic regressions evaluated the association between ACA enactment and insurance status (private, public, or no insurance). Routine annual care consistent with CF Foundation guidelines (≥4 clinic visits, ≥4 respiratory cultures, and ≥2 pulmonary function tests/year) was a secondary outcome. RESULTS: The analysis included 4,024 and 3,132 patients in the eligible and ineligible groups, respectively (35,353 patient-years). In the eligible group, 62% had private insurance before and after ACA; 18% had public insurance before and after ACA; and 5% switched from public to private insurance. In the eligible group, lack of insurance coverage became more common in the post-ACA period (relative risk ratio vs. private insurance [RRR] = 1.95; 95%CI: 1.57, 2.43; P < 0.001). Public insurance coverage also became more common (RRR = 1.50; 95%CI: 1.39, 1.62; P < 0.001). Use of routine care increased post-ACA, but more strongly in the ineligible group than in the eligible group. CONCLUSIONS: The ACA dependent coverage provision did not increase private insurance coverage or use of routine care among CF patients who were potentially affected by this policy. Pediatr Pulmonol. 2017;52:458-466. © 2017 Wiley Periodicals, Inc.

Novel Computed Tomography Scoring System for Sinus Disease in Adults With Cystic Fibrosis.

OBJECTIVE: There is no easy to use scoring system for computed tomography (CT) scans of the sinuses that is specific to cystic fibrosis (CF). We propose a simple and easily implemented scoring system to quantify severity of sinus disease in adults with CF. STUDY DESIGN: Case series with chart review. SETTING: Academic tertiary-care referral center. SUBJECTS: Sixty-nine adult patients with CF and 50 age-matched controls. METHODS: We validated a scoring system for CF sinus disease. The CT scans were interpreted by 3 physicians on 2 separate sittings. Parameters include maxillary opacification, nasal obstruction, lateral nasal wall displacement, uncinate process absence/demineralization, and presence/absence of mucocele. RESULTS: Patients with CF aged 21 to 30 years (mean = 24.7 ± 2.49). In CF cohort (n = 69), intrarater reliability for the 10 CT categories ranged from .70 to 1.00. Twenty-six (87%) were in the excellent range, and the remaining 4 (13%) were evaluated as good. In the non-CF cohort (n = 50), reliabilities ranged from .44 to 1.00. Twenty-seven (90%) were in the excellent range. For interrater reliability, in the CF cohort, 10 CT categories across the 3 raters ranged from .55 to 1.00. Excellent reliability was achieved in 15 (50%) of the observations. In the non-CF cohort, reliabilities ranged from .44 to 1.00. CONCLUSION: A novel and easy to use CT scoring system for CF sinus disease in adults was validated with inter- and intrarater reliability. This new CF sinus disease-specific scoring system can be used by clinicians, surgeons, and radiologists.

Impact of Donor Arterial Partial Pressure of Oxygen on Outcomes After Lung Transplantation in Adult Cystic Fibrosis Recipients.

INTRODUCTION: Donor PaO2 levels are used for assessing organs for lung transplantation (LTx), but survival implications of PaO2 levels in adult cystic fibrosis (CF) patients receiving LTx are unclear. METHODS: UNOS registry data spanning 2005-2013 were used to test for associations of donor PaO2 with patient survival and bronchiolitis obliterans syndrome (BOS) in adult (age ≥ 18 years) first-time LTx recipients diagnosed with CF. RESULTS: The analysis included 1587 patients, of whom 1420 had complete data for multivariable Cox models. No statistically significant differences among donor PaO2 categories of ≤200, 201-300, 301-400, or >400 mmHg were found in univariate survival analysis (log-rank test p = 0.290). BOS onset did not significantly differ across donor PaO2 categories (Chi-square p = 0.480). Multivariable Cox models of patient survival supported the lack of difference across donor PaO2 categories. Interaction analysis found a modest difference in survival between the two top categories of donor PaO2 when examining patients with body mass index (BMI) in the lowest decile (≤16.5 kg/m(2)). CONCLUSIONS: Donor PaO2 was not associated with survival or BOS onset in adult CF patients undergoing LTx. Notwithstanding statistically significant interactions between donor PaO2 and BMI, there was no evidence of post-LTx survival risk associated with donor PaO2 below conventional thresholds in any subgroup of adults with CF.

Attained Functional Status Moderates Survival Outcomes of Return to Work After Lung Transplantation.

BACKGROUND: Returning to work is a desirable outcome of lung transplantation that is selective on attained functional status. Survival implications of post-transplant employment are unclear. METHODS: The United Network for Organ Sharing registry was queried for first-time lung transplants performed from May 2005 to March 2015 in patients ages 18-64. Attainment of normal functional status post-transplant, defined as a 100 % score on the Karnofsky Performance Scale (KPS), was examined as moderating 5-year survival outcomes of work resumption, using Cox proportional hazards models. Supplemental analysis examined attainment of forced expiratory volume in 1 s (FEV1) ≥80 % predicted as moderating survival implications of post-transplant employment. RESULTS: Of 10,066 patients, 1824 (18 %) returned to work, while 9078 contributed follow-up data on functional status. Multivariable analysis demonstrated a protective effect of work resumption among patients who did not attain normal functional status before returning to work (HR = 0.62; 95 % CI = 0.51, 0.76; p < 0.001). This association was attenuated among transplant recipients who reached 100 % KPS while still unemployed (p < 0.001). Similarly, post-transplant survival was favorably associated with 5-year survival among patients who did not attain at least 80 % predicted FEV1 before returning to work (HR = 0.71; 95 % CI = 0.59, 0.86; p < 0.001). CONCLUSIONS: Early return to work after lung transplantation may benefit patients experiencing mild functional limitations. Timing the resumption of employment to coincide with attainment of maximal functional status around 1 year post transplant should be considered.