Innate immune responses are increased in children with acute asthma exacerbation.

BACKGROUND: Little is known about the immune responses during acute asthma exacerbation. In this study, we examined immune responses in children following an acute asthma exacerbation. METHODS: We evaluated pro-inflammatory cytokine levels and gene expression profiles in blood samples from pediatric patients admitted for acute asthma exacerbation. Viral PCR was performed to differentiate between viral or non-viral-associated exacerbations. RESULTS: Following informed consent, clinical data were obtained from 20 children with asthma (median [interquartile range, IQR]: age 11.5 [8.0, 14.2]) years and 14 healthy age-matched controls (10.5 [7.0, 13.0]). Twelve had positive nasopharyngeal Polymerase chain reaction (PCR) for viral infection (11 rhinoviruses and 1 respiratory syncytial virus (RSV)). Nine were in the pediatric intensive care unit (PICU) and among them five required continuous positive airway pressure (CPAP). Mean (±SD) days on systemic steroids before drawing blood sample were 2.5 ± 1.6. Twelve had history of environmental allergies with 917 (274, 1396) IU/mL total IgE (median (IQR)). Compared with controls, IL-1RA and IL-10 levels were significantly increased and TNF-α significantly decreased in asthma subjects (p < .05 for all). RNA-seq analysis revealed 852 differentially expressed genes in subjects with asthma. Pathway analysis found upregulated genes and pathways involved in innate immune responses in subjects with asthma. Significantly reduced genes included pathways associated with T helper cell differentiation and activation. CONCLUSIONS: In acute asthma exacerbation, innate immune pathways remained increased while adaptive immune responses related to T helper cells are blunted and are independent of trigger or asthma severity. Our novel findings highlight the need to identify new therapies to target persistent innate immune responses to improve outcomes in acute asthma.

Elexacaftor-Tezacaftor-Ivacaftor Therapy for Chronic Sinus Disease in Cystic Fibrosis.

Importance: Cystic fibrosis (CF) is a multiorgan genetic disease with progressive upper and lower airway involvement. The effects of CF transmembrane conductance regulator (CFTR) modifier therapies on CF-related upper airway disease, specifically chronic rhinosinusitis (CRS), are not characterized. Objective: To determine the outcome of elexacaftor-tezacaftor-ivacaftor (ETI) on CRS as measured by changes in sinus computed tomography (CT) metrics and on clinical parameters in individuals with CF. Design, Setting, and Participants: This prospective longitudinal cohort study was conducted at the CF center of a tertiary care hospital between October 1, 2019, and July 31, 2021. A total of 64 participants with CF were included in the analysis. Intervention: Sinus CT was obtained within 1 month of initiation of ETI therapy (baseline), and within 1 month of 1 year of ETI therapy. Images were independently analyzed by pulmonology, radiology, and otolaryngology physicians, using the Lund-Mackay and Sheikh-Lind scoring systems. Percent predicted forced expiratory volume in 1 second (ppFEV1), body mass index (BMI), and microbiologic data collected at initiation of ETI therapy and 3-month intervals for 1 year were also measured. Main Outcomes and Measures: The study hypothesis was that ETI therapy will improve CRS as measured by changes in sinus CT at initiation and 1 year after ETI therapy and clinical parameters in individuals with CF. Results: Among the 64 participants (39 [60.9%] female; median age, 18.5 [IQR, 16.0-28.5] years; 64 [100%] White), improvement in CRS was noted by improvements in sinus CT scans using both sinus CT scoring systems after 1 year of ETI therapy. The reduction in the median total score using the Lund-Mackay sinus CT scoring system (from 5.8 [IQR, 5.0-7.0] to 3.3 [IQR, 2.6-4.2]) and the Sheikh-Lind scoring system (from 3.8 [IQR, 3.0-5.0] to 2.2 [IQR, 2.0-2.5]) was noted. Increases in ppFEV1 and BMI were also observed by 3 months of ETI therapy with persistent improvement through 1 year of treatment. Similarly, after 1 year of ETI therapy, participants with CF had reductions in positivity for Pseudomonas aeruginosa and Staphylococcus aureus in oropharyngeal cultures. Conclusion and Relevance: This cohort study found that use of ETI therapy was associated with improved CRS outcomes in participants with CF as quantified by improved sinus CT scans measured by 2 radiographic scoring systems and was also associated with improved clinical outcomes. Despite improvement in CT scan scores, most people with CF continue to have scores that indicate severe sinus disease.

Impact of Elexacaftor-Tezacaftor-Ivacaftor on lung disease in cystic fibrosis.

BACKGROUND: In people with cystic fibrosis (pwCF), the impact of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies, such as Elexacaftor-Tezacaftor-Ivacaftor (ETI), on structural changes in the lungs is unclear. OBJECTIVE: To determine the impact of ETI on clinical parameters and on structural lung disease as measured by the changes in the chest computed tomography (CT) scans in pwCF. METHODS: Percent predicted forced expiratory volume in one second (ppFEV1), body mass index (BMI), and microbiologic data were collected at initiation and 3-month intervals for 1 year. Chest CT scans before starting ETI therapy (baseline) and at 1-year on ETI therapy were compared by two pulmonologists independently. RESULTS: The sample size was 67 pwCF, 30 (44.8%) males, median age of 25 (16, 33.5) years. Significant increases in ppFEV1 and BMI observed by 3 months of ETI therapy persisted throughout 1 year of ETI therapy (p < 0.001 at all-time points for both). After 1 year on ETI, pwCF had significant reductions in Pseudomonas aeruginosa (-42%) and MRSA (-42%) positivity. None of the pwCF had worsening of chest CT parameters during 1 year of ETI therapy. Comparing chest CT findings at baseline and at 1-year follow-up, bronchiectasis was present in 65 (97%) pwCF and at 1-year follow-up decreased in 7 (11%). Bronchial wall thickening 64 (97%), decreased in 53 (79%). Mucous plugging in 63 (96%), absent in 11 (17%), and decreased in 50 (77%). Hyperinflation/air trapping in 44 (67%), decreased in 11 (18%), absent in 27 (44%) CONCLUSIONS: ETI significantly improved clinical outcomes and lung disease as documented by improvement in chest CT scans.

Clinical change 2 years from start of elexacaftor-tezacaftor-ivacaftor in severe cystic fibrosis.

RATIONALE: Limited published research is available on the impact of elexacaftor/tezacaftor/ivacaftor (ETI) beyond the initial few months postdrug initiation, especially for those who initiated therapy via individual investigational new drug application. The experiences of patients with cystic fibrosis (CF) experiencing severe lung disease were reviewed for significant improvements in clinical symptoms and quality of life. OBJECTIVES: To examine clinical outcomes 2 years post-ETI in patients with CF and advanced lung disease. METHODS: This single center institutional review board-approved, retrospective chart review assessed clinical markers (percent predicted forced expiratory volume in 1 s, weight, sweat chloride), quality of life and computed tomography scans in patients with advanced lung disease who met criteria for compassionate use/expanded access program due to high risk of death or transplant need within 2 years. RESULTS: Eighteen identified patients (ages 15-49 years) initiated drug between July and September 2019. Clinical markers indicated that therapy was well tolerated, not discontinued by any participant, and lab values did not indicate medical concern or discontinuation. Monitoring results indicated the safety of modulator therapy as there were no adverse clinical occurrences and all patients presented universal stabilization. There were no deaths and no transplants by the end of the study. CONCLUSIONS: This study focused on patients with CF eligible for modulator therapy and were initiated due to advanced lung disease. Initiation of modulator therapy was deemed safe and resulted in objective positive changes in nutrition, cough, FEV1 , subjective reports of clinical status, level of activity, and a reduction in burden of treatment.

Impact of elexacaftor-tezacaftor-ivacaftor on bacterial colonization and inflammatory responses in cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is a multisystem disease with progressive deterioration. Recently, CF transmembrane conductance regulator (CFTR) modulator therapies were introduced that repair underlying protein defects. Objective of this study was to determine the impact of elexacaftor-tezacaftor-ivacaftor (ETI) on clinical parameters and inflammatory responses in people with CF (pwCF). METHODS: Lung function (FEV1 ), body mass index (BMI) and microbiologic data were collected at initiation and 3-month intervals for 1 year. Blood was analyzed at baseline and 6 months for cytokines and immune cell populations via flow cytometry and compared to non-CF controls. RESULTS: Sample size was 48 pwCF, 28 (58.3%) males with a mean age of 28.8 ± 10.7 years. Significant increases in %predicted FEV1 and BMI were observed through 6 months of ETI therapy with no change thereafter. Changes in FEV1 and BMI at 3 months were significantly correlated (r = 57.2, p < 0.01). There were significant reductions in Pseudomonas and Staphylococcus positivity (percent of total samples) in pwCF through 12 months of ETI treatment. Healthy controls (n = 20) had significantly lower levels of circulating neutrophils, interleukin (IL)-6, IL-8, and IL-17A and higher levels of IL-13 compared to pwCF at baseline (n = 48). After 6 months of ETI, pwCF had significant decreases in IL-8, IL-6, and IL-17A levels and normalization of peripheral blood immune cell composition. CONCLUSIONS: In pwCF, ETI significantly improved clinical outcomes, reduced systemic pro-inflammatory cytokines, and restored circulating immune cell composition after 6 months of therapy.

Impact of type of health care provider on long term asthma control.

OBJECTIVE: Asthma prevalence is high and adherence to asthma guidelines is still less than adequate. The main objective of this study was to determine if there were significant differences in outcome measures if asthma care was provided per guidelines either by physicians (pediatric pulmonologists) or specialty trained advance practice nurses (APNs). METHODS: This was a three-year, prospective cohort study of children referred by their primary care providers to a tertiary care center for better asthma control. Patients were provided asthma care per NAEPP guidelines including asthma education. Results were compared over time and between patients followed by physicians or APNs. Alpha level of significance was ≤0.05. RESULTS: The sample included 471 children, ages 2-17 years (mean = 6.4 ± 2.4 years). Physicians and APN's provided asthma care. Of the 471 children enrolled in the study, 176 (37%) were followed for the full three-year study period. At the initial visit, physician group reported more short courses of oral steroids and more unscheduled visits to PCP for acute asthma care in the past 6 months compared to those followed by APNs (<0.05 for all). Among the total cohort and both subgroups, there were significant improvements in mean Asthma Control Test (ACT), acute care need and mean days/month with asthma symptoms over a three-year period (p < 0.05). There was significantly more improvement in use of oral steroids and urgent care visits in physician group (p < 0.05). CONCLUSION: When asthma guidelines are followed, improvements in asthma control are achieved in children in both the MD and APN groups.

Impact of guideline adherence and race on asthma control in children.

BACKGROUND: Asthma control in African Americans (AA) is considered more difficult to achieve than in Caucasian Americans (CA). The aim of this study was to compare asthma control over time among AA and CA children whose asthma is managed per NAEPP (EPR-3) guidelines. METHODS: This was a one-year prospective study of children referred by their primary care physicians for better asthma care in a specialty asthma clinic. All children received asthma care per NAEPP guidelines. Results were compared between CA and AA children at baseline and then at three-month intervals for one year. RESULTS: Of the 345 children, ages 2-17 years (mean = 6.2 ± 4), 220 (63.8%) were CA and 125 (36.2%) were AA. There were no significant differences in demographics other than greater pet ownership in CA families. At baseline, AA children had significantly more visits to the Emergency Department for acute asthma symptoms (mean = 2.3 [Formula: see text] compared to CA (1.4 ± 2.3, P = 0.003). There were no other significant differences in acute care utilization, asthma symptoms (mean days/month), or mean asthma control test (ACT) scores at baseline. Within 3-6 months, in both groups, mean ACT scores, asthma symptoms and acute care utilization significantly improved (P < 0.05 for all) and change over time in both groups was comparable except for a significantly greater decrease in ED visits in AA children compared to CA children (P = 002). CONCLUSION: Overall, improvement in asthma control during longitudinal assessment was similar between AA and CA children because of  consistent use of NAEPP asthma care guidelines.

Impact of asthma severity on long-term asthma control.

Background: Asthma is a common childhood disease with significant morbidity. Severe asthma accounts for just 4-6% of patients, but this group is more difficult to treat and is responsible for up to 40% of asthma expenses.Objective: The relationship between asthma severity and control is not well characterized. The main objective of this study was to determine impact of asthma severity on asthma control over time.Methods: This was a three year, prospective observational cohort study at a tertiary care children's hospital. Results were compared over time and between patients with severe and non-severe persistent asthma. Intervention included therapy based on severity and control, accompanied by a NAEPP (EPR-3) guidelines based structured asthma education program.Results: The sample included 471 children referred from primary care offices with the diagnosis of persistent asthma, mean age 6.4 ± 2.4 years. Forty-one children (8.7%) had severe persistent asthma and 430 (91.3%) children had non-severe persistent asthma (mild-moderate persistent). Our sample size decreased over the three-year period and the number of patients completing the third year were 176 (38%) and among them 20 (11.4%) had severe asthma. At the initial visit, children with severe persistent asthma had significantly more acute care needs, more daily symptoms, and lower mean Asthma Control Test™ scores compared to children with non-severe persistent asthma. Differences between groups decreased within six months with significant improvements in most indicators persisting throughout three-year follow up in both groups (p < 0.05).Conclusion: Asthma control improves independent of severity if asthma guidelines are followed.

Comparison of Two Therapies on Asthma Control in Children.

Background: Childhood asthma carries significant morbidity. Aim/Objectives: Aim of the study was to compare efficacy of 2 commonly used therapies for asthma control in children with asthma. Methods: This was a 1-year, prospective cohort study at a tertiary care children's hospital. Patients were referred by their primary care physicians (PCPs) for asthma control. All patients were on low-dose inhaled corticosteroids (ICSs) at baseline. They were either switched to medium-dose ICS (ICS group) or medium-dose ICS and long-acting beta agonist (ICS+LABA group). Results were compared over time and between both groups. Results: Our cohort included 163 children (ages 2-18 years) with mean age of 5.62 ± 3.61 years. Mean Asthma Control Test (ACT) score at baseline was 15.9 ± 5.4. Mean ACT and percent predicted forced expiratory volume in one second improved (P < 0.0001 for both) in both groups. Median emergency department visits, short courses of oral steroids, and unscheduled PCP visits for acute asthma significantly decreased (P < 0.001 for all) in both groups. Similarly, days/month with wheezing, nighttime cough, and missed school days significantly decreased in both groups (P < 0.001 for all). Patients in ICS group were more likely to fail to achieve asthma control compared to patients in ICS+LABA group. Conclusion: Our study suggests that in children with uncontrolled asthma on low-dose ICS, switching to either medium-dose ICS or medium-dose ICS+LABA resulted in better symptom control, ACT improvement, and less asthma exacerbations over time. ICS+LABA had the additional benefit of less risk of treatment failure when compared to medium-dose ICS.

Gaps in health insurance coverage and emergency department use among children with asthma.

Background: Gaps in health insurance coverage may complicate asthma management and increase emergency department (ED) use. Using two nationally-representative surveys, we characterize the prevalence of coverage gaps among children with asthma, and describe their association with ED visits in this population. Methods: De-identified data were obtained from the 2016 National Survey of Children's Health (NSCH) and National Health Interview Survey (NHIS). Among children with asthma, we classified coverage over the past year as: (1) continuous private, (2) continuous public, (3) gap in coverage, and (4) continuously uninsured. The primary outcome was all-cause ED visits in the past year (both surveys). Secondary outcomes included unmet health care needs (NSCH), asthma-related ED visits or hospitalizations (NHIS) and asthma exacerbations (NHIS). Results: The analysis included 3739 (NSCH) and 854 (NHIS) children with asthma, representing a population of 5.5 million children in the US. Estimated prevalence of coverage gaps was 5% in the NSCH and 3% in the NHIS. On multivariable ordinal logistic regression using NSCH data, coverage gaps were associated with increased all-cause ED use (OR = 2.5; 95% CI: 1.3, 4.7, p = 0.005), compared to continuous private coverage. Further analysis confirmed higher odds of unmet health care needs, asthma exacerbations, and asthma-related ED visits among children with coverage gaps. Conclusions: Children with asthma who experience insurance coverage gaps have increased ED use, possibly related to poorer access to appropriate health care. Protecting insurance coverage continuity may reduce ED use and improve clinical outcomes in this population.

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.

Erratum to: Racial differences in pet ownership in families of children with asthma.

In the article by Sheikh SI, et al, "Racial differences in pet ownership in families of children with asthma" in World Journal of Pediatrics 2016;12(3):343-346 (doi: 10.1007/s12519-016-0027-9), the last author's name was incorrectly listed as "Don Hayes". His name should have read "Don Hayes Jr".

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.

Role of CFTR mutation analysis in the diagnostic algorithm for cystic fibrosis.

BACKGROUND: The cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation identification is being used with increased frequency to aid in the diagnosis of cystic fibrosis (CF) in those suspected with CF. Aim of this study was to identify diagnostic outcomes when CFTR mutational analysis was used in CF diagnosis. CFTR mutational analysis results were also compared with sweat chloride results. METHODS: This study was done on all patients at our institution who had CFTR mutation analysis over a sevenyear period since August 2006. RESULTS: A total of 315 patients underwent CFTR mutational analysis. Fifty-one (16.2%) patients had two mutations identified. Among them 32 had positive sweat chloride levels (≥60 mmol/L), while seven had borderline sweat chloride levels (40-59 mmol/L). An additional 70 patients (22.3%) had only one mutation identified. Among them eight had positive sweat chloride levels, and 17 had borderline sweat chloride levels. Fifty-five patients (17.5%) without CFTR mutations had either borderline (n=45) or positive (n=10) sweat chloride results. Three patients with a CF phenotype had negative CFTR analysis but elevated sweat chloride levels. In eighty-three patients (26.4%) CFTR mutational analysis was done without corresponding sweat chloride testing. CONCLUSIONS: Although CFTR mutation analysis has improved the diagnostic capability for CF, its use either as the first step or the only test to diagnose CFTR dysfunction should be discouraged and CF diagnostic guidelines need to be followed.

Improved quality-of-life of caregivers of children with asthma through guideline-based management.

OBJECTIVE: The quality of life (QOL) of caregivers of children with asthma may be related to children's responses to asthma management. AIM: To evaluate change in QOL over time of caregivers of children with asthma through guideline-based management. DESIGN: This was a 3-year prospective cohort study of children with asthma referred to our pediatric asthma center. Families completed Pediatric Asthma Caregiver's Quality of Life Questionnaire (PACQLQ), the Asthma Control Test™ (ACT), and reported the number of days/month of albuterol use and wheezing at each clinic visit. RESULTS: We enrolled 143 children, ages 7-17 years (mean = 10.6 ± 2.9), 56.6% male, 70.6% Caucasian. Patients were managed by the same MD (n = 65,45.5%) or APN (n = 78,54.5%) over time. The mean total PACQLQ significantly increased over the 3-year period (F = 67.418, p < .001). Total scores at the first visit were 4.8 ± 1.6, which improved to 6.1 ± 1 at the 3-month follow-up visit. This improvement was sustained at the 1, 2, and 3-year clinic visits. PACQLQ emotional function (F = 60.798, p < .001) and activity limitation (F = 41.517, p < .001) domains significantly improved as well. PACQLQ scores were significantly associated with improved ACT scores (r = .37 to .47, p < .05), fewer days/month of albuterol use (r = -.25 to -.36., p < .05), and wheezing (r = -.28 to -.33, p < .05). There were no significant differences in PACQLQ, or asthma clinical outcome measures between MD and APN providers. CONCLUSION: Use of National Asthma Education and Prevention Program (NAEPP) guidelines significantly improved QOL of caregivers of children with asthma and in asthma-related symptoms. Improvements over time were independent of type of providers.

Pretransplant Panel Reactive Antibodies and Lung Transplant Outcomes in Children.

Background Pre-lung transplant (LTx) panel reactive antibody (PRA) levels are associated with adverse outcomes in adult LTx recipients, but their impact in pediatric LTx recipients is unknown. Methods The United Network for Organ Sharing registry was queried from 2004 to 2013 to compare survival between pediatric LTx recipients with PRA class I and II levels = 0 versus > 0. Results Overall, 333 pediatric LTx recipients had data on class I or II PRA and were included in the analysis. Univariate analysis demonstrated that PRA > 0 was not associated with survival benefit for class I (hazard ratio [HR] = 0.985; 95% confidence interval [CI]: 0.623, 1.555; p = 0.947) or class II (HR = 1.080; 95% CI: 0.657, 1.774; p = 0.762) PRA. Multivariate Cox models confirmed no significant association with mortality hazard for both class I (HR = 1.230; 95% CI: 0.641, 2.363; p = 0.533) and class II (HR = 0.847; 95% CI: 0.359, 1.997; p = 0.704) PRA. Multivariate logistic regression models identified no association between class I or class II and acute rejection within 3 years of LTx. Conclusions Pretransplant class I and II PRA levels > 0 were not associated with mortality or acute rejection in pediatric LTx recipients.

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.

Racial differences in pet ownership in families of children with asthma.

BACKGROUND: Exposure to household domestic animals such as cats and dogs in early life may have some role in pathogenesis of asthma. Racial differences exist in the prevalence of asthma. We hypothesized that there may also be racial differences in pet ownership in families with asthma. METHODS: A cross sectional study was conducted from June 2011 to December 2014 on 823 of 850 (97%) families of children with asthma for pet ownership. Comparisons among racial groups were done using chi square analysis and one-way analysis of variance. RESULTS: The mean age of the cohort was 6.9±4.4 years. A total of 540 (65.62%) patients were Caucasian, 195 (23.7%) African American, 42 (5.1%) hispanics, and 26 (3.2%) biracial with one Caucasian parent. Pets in the home were reported by 470 (58.5%) households. Significantly fewer African American and hispanic families had pets in the home (26.9% and 44.7%) than biracial and Caucasian families (72% and 69.9%, P<0.001). Likewise, significantly more biracial and Caucasian families were noted to have dogs (52% and 54.4%) or cats (25.4% and 40%) or both cats and dogs (28% and 18%) than African Americans families (20.3%, P<0.001; 7.1%, P<0.001) and (4.6%, P<0.001), respectively. CONCLUSIONS: Among families with asthmatic children, pet ownership is significantly more likely in Caucasian families compared with African-American and Hispanic families, thus there is a racial diversity in pet ownership among families of children with asthma.

Influence of graft ischemic time on survival in children with cystic fibrosis after lung transplantation.

BACKGROUND: The influence of graft ischemic time on survival after lung transplantation (LTx) in children with cystic fibrosis (CF) is not well studied. METHODS: The United Network for Organ Sharing (UNOS) database was queried from May 2005 to September 2013 to examine the impact of ischemic time of <4, 4-6, and >6 hr in pediatric LTx recipients with CF. RESULTS: One hundred and ninety-nine patients with CF under 18 years of age that were first-time LTx recipients from cadaveric donors were included in the analysis. Compared to 4-6 hr, univariate analysis showed a significant increase in mortality hazard with an ischemic time of <4 hr (HR = 2.407; 95%CI: 1.292, 4.485; P = 0.006) but not >6 hr (HR = 1.350; 95%CI: 0.796, 2.290; P = 0.266). A Kaplan-Meier plot demonstrated the highest survival with 4-6 hr (Log-rank test P = 0.018) of ischemic time. Multivariate Cox model confirmed a significantly higher mortality risk with <4 hr (HR = 2.388; 95%CI: 1.169, 4.764; P = 0.014) and not >6 hr (HR = 1.407; 95%CI: 0.760, 2.605; P = 0.278) in relation to 4-6 hr. Sub-analysis examining ischemic time and the hazard of bronchiolitis obliterans syndrome with death as a competing risk found no significant differences in the hazard of this outcome across the three ischemic time categories. CONCLUSIONS: Ischemic time of 4-6 hr was associated with the highest long-term survival in first-time pediatric LTx recipients with CF, with ischemic time <4 hr related to diminished survival. Pediatr Pulmonol. 2016; 51:908-913. © 2016 Wiley Periodicals, Inc.

Environmental exposures and family history of asthma.

BACKGROUND: Asthma pathogenesis is a complex interaction of genetic, ethnic, environmental and social/life style risk factors. AIM: The goal of this study was to identify associations, if any, in children with asthma, between environmental risk factors (exposure to second-hand tobacco smoke (STS), pet ownership, race and a family history of asthma. METHODS: After IRB approval, from June 2011 to December 2014, 823 children with asthma were enrolled in this prospective cross sectional study. At the initial visit, families completed a questionnaire with information on family history of asthma, having a pet at home and exposure to STS by parents at home. Chi square analyses were calculated, with alpha level of significance ≤0.05. RESULTS: History of asthma in parents, siblings or grandparents was reported by 575 (69.8%) patients including father (n = 154, 17.8%) and mother (n = 235, 26.5%). Children with family history of asthma (n = 575) were significantly more likely to have a pet at home and exposure to STS (n = 347, 60.3% and n = 198, 34.4%, respectively) compared to families without a history of asthma (n = 124, 50%, p = 0.006 and n = 44, 17.7%, p < 0.001, respectively). Similarly, asthmatic children with exposure to STS (n = 241) were significantly more likely to have a pet at home and a family history of asthma (n = 153, 63.5% and n = 197, 81.7%, respectively) compared to children with no STS exposure (n = 315, 55.5%, p = 0.034 and n = 371, 65.3%, p < 0.001 respectively). CONCLUSIONS: Significantly more asthmatic children with immediate relatives with a history of asthma have a pet at home and experience STS exposure compared to children without relatives with a history of asthma, suggesting association between life style choices/environmental exposures and family history of asthma.