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.

Long-Term Survival Following Primary Graft Dysfunction Development in Lung Transplantation.

INTRODUCTION: Primary graft dysfunction (PGD) is a known risk factor for early mortality following lung transplant (LT). However, the outcomes of patients who achieve long-term survival following index hospitalization are unknown. We aimed to determine the long-term association of PGD grade 3 (PGD3) in patients without in-hospital mortality. METHODS: LT recipients were identified from the United Network for Organ Sharing Database. Patients were stratified based on the grade of PGD at 72 h (No PGD, Grade 1/2 or Grade 3). Groups were assessed with comparative statistics. Long-term survival was evaluated using Kaplan-Meier methods and a multivariable shared frailty model including recipient, donor, and transplant characteristics. RESULTS: The PGD3 group had significantly increased length of stay, dialysis, and treated rejection post-transplant (P < 0.001). Unadjusted survival analysis revealed a significant difference in long-term survival (P < 0.001) between groups; however, following adjustment, PGD3 was not independently associated with long-term survival (hazard ratio: 0.972; 95% confidence interval: 0.862-1.096). Increased mortality was significantly associated with increased recipient age and treated rejection. Decreased mortality was significantly associated with no donor diabetes, bilateral LT as compared to single LT, transplant in 2015-2016 and 2017-2018, and no post-transplant dialysis. CONCLUSIONS: While PGD3 remains a challenge post LT, PGD3 at 72 h is not independently associated with decreased long-term survival, while complications such as dialysis and rejection are, in patients who survive index hospitalization. Transplant providers should be aggressive in preventing further complications in recipients with severe PGD to minimize the negative association on long-term survival.

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.

Novel and Engaging Teaching Method for Pulmonary Physiology.

BACKGROUND: Pulmonary physiology is a challenging, necessary component of pediatric pulmonary fellowship education. Our pediatric pulmonology fellowship program provided this education utilizing a flipped classroom approach but satisfaction with the method was declining. Evidence suggests that adult learners benefit from an engaging lecture method, but no evidence exists to compare the flipped classroom approach to the engaging lecture approach for pulmonary physiology education. OBJECTIVE: To develop the most effective physiology curriculum for pediatric pulmonary fellows by comparing the flipped classroom approach to an engaging lecture method. METHODS: Five physiology teaching sessions were assigned to the flipped classroom method and 5 to the engaging lecture style. Anonymous surveys assessing satisfaction, utilizing a five-point Likert scale, were completed by fellows at the end of each session. An unpaired t-test was used to compare responses. RESULTS: Seven pediatric pulmonary fellows enrolled in the study. The average attendance per session was 5 fellows. There was no significant difference in fellows' perceived understanding of the assigned physiology topic prior to the flipped classroom versus engaging lecture sessions. Fellows' perceived understanding of the topics improved after both session types, with significantly more improvement after the engaging lecture sessions. Fellows also reported that engaging lecture sessions were more useful and improved their test-taking confidence. They were more likely to recommend engaging lecture sessions to future pulmonary fellows. CONCLUSIONS: Pediatric pulmonary fellows benefited from the use of the engaging lecture style for physiology education.

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.

ATS Core Curriculum 2021. Pediatric Pulmonary Medicine: Pulmonary Infections.

The following is a concise review of the Pediatric Pulmonary Medicine Core reviewing pediatric pulmonary infections, diagnostic assays, and imaging techniques presented at the 2021 American Thoracic Society Core Curriculum. Molecular methods have revolutionized microbiology. We highlight the need to collect appropriate samples for detection of specific pathogens or for panels and understand the limitations of the assays. Considerable progress has been made in imaging modalities for detecting pediatric pulmonary infections. Specifically, lung ultrasound and lung magnetic resonance imaging are promising radiation-free diagnostic tools, with results comparable with their radiation-exposing counterparts, for the evaluation and management of pulmonary infections. Clinicians caring for children with pulmonary disease should ensure that patients at risk for nontuberculous mycobacteria disease are identified and receive appropriate nontuberculous mycobacteria screening, monitoring, and treatment. Children with coronavirus disease (COVID-19) typically present with mild symptoms, but some may develop severe disease. Treatment is mainly supportive care, and most patients make a full recovery. Anticipatory guidance and appropriate counseling from pediatricians on social distancing and diagnostic testing remain vital to curbing the pandemic. The pediatric immunocompromised patient is at risk for invasive and opportunistic pulmonary infections. Prompt recognition of predisposing risk factors, combined with knowledge of clinical characteristics of microbial pathogens, can assist in the diagnosis and treatment of specific bacterial, viral, or fungal diseases.

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.

Chronic opioid use and clinical outcomes in lung transplant recipients: A single-center cohort study.

INTRODUCTION: Chronic opioid use is common after organ transplantation, and has been associated with poor outcomes in transplantation of abdominal organs. However, little is known about possible influences of chronic opioid use on outcomes of lung transplantation (LTx). OBJECTIVES: We assessed whether long-term chronic opioid use influenced clinical outcomes among LTx recipients at our program. METHODS: We retrospectively evaluated chronic opioid use among bilateral LTx recipients ages 12 and older followed at our institution 1-5 years post-transplant. Chronic opioid use was defined as ≥3 months of consecutive prescribed use. Outcomes included survival, hospitalization, emergency department and urgent care visits, forced expiratory volume in one second (FEV1), and allograft rejection. RESULTS: Twenty-one patients ages 15-50 years met inclusion criteria. On multivariable analysis, initiation of chronic opioid use was followed by increased mortality hazard (hazard ratio=7.1; 95% confidence interval [CI]: 1.1, 45.0, P = 0.037) and decreased FEV1 (-16%; 95% CI: -24%, -7%; P < 0.001), although no differences were observed in risk of acute care visits, inpatient admission, or chronic rejection. CONCLUSION: This analysis presents the first evidence that late-onset chronic opioid use may be associated with decreased lung function and increased mortality after LTx. Therefore, evaluation of chronic opioid use should be included in the routine monitoring of transplant recipients, to better define the impact of this risk factor on LTx outcomes.

Benefits of pulmonary rehabilitation in pediatric asthma.

INTRODUCTION: There are limited studies evaluating the role of pulmonary rehabilitation (PR) in pediatric asthma. METHODS: A retrospective chart review was performed of all pediatric patients with a diagnosis of asthma enrolled in PR. Demographics, medications, and clinical records were reviewed. In addition, PFTs, 6-min walk test (6MWT), and patient/parent symptom and quality of life surveys before and after PR were evaluated. RESULTS: A total of 38 patients were enrolled in PR; 18 (47%) female and 20 (53%) male. Mean participant age was 11.33 ± 3.37 (range 4-19) years. Twenty-two (58%) were Caucasian and nine (24%) African American. Chart review was limited by incomplete data sets for many participants. Following PR, significant improvement was noted in mean 6MWT distance (1541 vs 1616 feet, P = 0.05) and FEV1 (89.9% of predicted versus 96.4%, P = 0.04). Survey instruments demonstrated improvement in several clinical factors, however, there was no significant change in weight following PR despite scheduled cardiovascular exercise and dietary counseling. CONCLUSIONS: Structured PR for pediatric patients with asthma can improve 6MWT distance and FEV1 as well as subjective measures of SOB and QOL, suggesting a role for PR in the chronic management of pediatric asthma. Further prospective investigation is needed to determine if PR has positive effects on other clinical parameters of asthma control and its overall impact on childhood obesity.

Improved Lung Function after Sinus Surgery in Cystic Fibrosis Patients with Moderate Obstruction.

Objectives Cystic fibrosis (CF) is characterized by infection and inflammation of the sinorespiratory tract. Functional endoscopic sinus surgery (FESS) is an option for patients with severe sinusitis. We sought to evaluate pulmonary function testing after FESS in pediatric and adult patients with CF. Study Design Retrospective chart review using data from all patients with CF who underwent FESS from January 2009 to July 2014. Setting Patients were from a single institution. Subjects and Methods Data were extracted for 181 patients and 320 surgeries. Lung function data, including the forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and FEV1/FVC, were retrieved within 1 year before and after surgery. Mixed-effects regression was used to compare FEV1 trajectories before and after surgery. The effect of surgery was stratified by presurgery FEV1 to compare patients with mild/no lung disease (FEV1 >80%) and moderate/severe lung disease (FEV1 <80%). Results Of the 181 patients reviewed, 131 with primary FESS had FEV1 data. Presurgery average age was 16 years (95% confidence interval [CI], 14.27-17.73), and FEV1 mean was 85% (95% CI, 81.02-88.98). There were 88 patients with FEV1 >80% and 43 patients with FEV1 <80%. For the entire cohort, lung function did not change related to FESS. Among patients with FEV1 <80%, FEV1 declined presurgery by 3.5% per year (95% CI, -6.1% to -0.8%; P = .010), which halted after surgery with these patients, then showing no subsequent change in FEV1 (95% CI, 0.9%-3.7%; P = .240). No benefit was identified for patients with FEV1 >80%. Conclusion Pulmonary function testing improved in patients with moderate/severe lung disease 1 year following FESS. This suggests FESS may benefit pulmonary outcomes.