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.

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.

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.

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.

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).

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.

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.

Comparison of Two Therapies on Asthma Control in Children.

Childhood asthma carries significant morbidity. Aim of the study was to compare efficacy of 2 commonly used therapies for asthma control in children with asthma. This was a 1-year, prospective cohort study at a tertiary care children's hospital.

Impact of asthma severity on long-term asthma control.

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. The relationship between asthma severity and control is not well characterized.

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

: 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. : De-identified data were obtained from the 2016 National Survey of Children's Health (NSCH) and National Health Interview Survey (NHIS).

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.

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%).

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.

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.

Pretransplant Panel Reactive Antibodies and Lung Transplant Outcomes in Children.

 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.  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.  Overall, 333 pediatric LTx recipients had data on class I or II PRA and were included in the analysis.

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.

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.

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.

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.

Improving pediatric asthma care: A partnership between pediatric primary care clinics and a free-standing Children's Hospital.

Background: Asthma is a common chronic disease of childhood. Providers' adherence to asthma guidelines is still less than optimal.

Objectives: To determine if an Asthma Education Program aimed at primary care practices can improve asthma care within practices and if the results vary by duration of the program.

Screening high-risk children for asthma through a community intervention.

Objective: Many children, particularly those from inner city neighborhoods, have undiagnosed asthma. This study was done to evaluate the effectiveness of an asthma screening, referral and follow-up intervention in an inner city community setting in early identification of children at risk for undiagnosed asthma.

Methods: A descriptive longitudinal cohort design was used to assess children at baseline and at a 2-year follow-up.

Mortality Risk and Pulmonary Function in Adults With Cystic Fibrosis at Time of Wait Listing for Lung Transplantation.

Background: Lung transplantation (LTx) benefit for survival in cystic fibrosis (CF) patients placed on the wait list is not well studied.

Methods: To predict the relationship between initial forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) and the hazard ratio (HR) associated with LTx in CF patients, the United Network for Organ Sharing database was queried from 2005 to 2006 for adult patients with CF. Survival was assessed from wait list entry time until death on wait list, death after LTx, or censoring.

Changes in Pulmonary Function and Controlled Ventilation-High Resolution CT of Chest After Antibiotic Therapy in Infants and Young Children with Cystic Fibrosis.

Background: Infants with cystic fibrosis (CF) develop early progressive lung disease which may be asymptomatic. Infant pulmonary function tests (IPFT) and controlled ventilation-high resolution computed tomography (CV-HRCT) of chest can detect early asymptomatic lung disease. It is not well established that these objective measures can detect changes in lung disease after clinical interventions.

Improvement of hepatic steatosis in cystic fibrosis with ivacaftor therapy.

Treatment of liver disease, including hepatic steatosis, in patients with cystic fibrosis (CF) is limited. With the development of ivacaftor, which corrects the gating defect of the CF transmembrane regulator channel, there is a potential new therapy available for this subgroup of the CF patient population. We present an adolescent with CF who had significant improvement in hepatic steatosis with ivacaftor treatment while hypothesizing on a mechanism of why it occurred.