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.

Asthma medication use and health care utilization evolution during pandemic times.

Pediatric asthma care was significantly impacted by the Coronavirus Disease (COVID-19) pandemic, with significant decline in asthma health care utilization noted early in the pandemic. We compared Emergency Department (ED) utilization rates and prescription fill rates of controller and quick relief asthma medications between March and December 2020 versus 2021 in a county-specific pediatric Medicaid population to evaluate for changes later in the pandemic. Our data showed an increase in ED utilization by 46.

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.

Bronchiectasis associated with electronic cigarette use: A case series.

Bronchiectasis (BE) is defined as a permanent, irreversible dilation of the bronchial tree. In the pediatric population, this disease process is most commonly associated with patients with cystic fibrosis (CF). However, BE unrelated to CF is increasingly noted as a cause of chronic respiratory related morbidity worldwide.

Adult survivor with repaired complete pentalogy of Cantrell.

Pentalogy of Cantrell is a very rare condition with very high mortality. We present an adult survivor with a classic pentad who underwent sequential surgical repairs as a neonate, child, and young adult. He required home mechanical ventilation for the first two years of life and subsequently needed noninvasive nocturnal ventilation as an adult.

Decreased right ventricular function in healthy pediatric cystic fibrosis patients versus non-cystic fibrosis patients.

Right ventricular (RV) dysfunction may occur in patients with cystic fibrosis (CF). Tissue Doppler imaging (TDI) and strain and strain rate analysis are new echocardiographic tools that can quantitate RV function. This study aimed to compare the RV function between healthy CF patients and non-CF patients to determine whether differences exist.

A novel exon duplication of the cystic fibrosis transmembrane conductance regulator in a patient presenting with adult-onset recurrent pancreatitis.

Pancreatitis is a rare occurrence in patients with cystic fibrosis (CF) affecting 1.2% of all patients, but it can be the first presenting sign in approximately 15% of adults with pancreatic sufficiency and a milder CF phenotype. We report a case of a woman with recurrent pancreatitis who has one cystic fibrosis-causing mutation (G551D) and the first known description of a pathologic duplication of exon 19 of the CF transmembrane conductance regulator (CFTR).

The role of computerized order sets in pediatric inpatient asthma treatment.

Condition-specific order sets within computerized physician order entry (CPOE) systems are designed to decrease unnecessary practice variation and to promote evidence-based practice. This study quantitatively assessed the relationship between use of a computerized order set and processes of care in inpatient pediatric asthma treatment, and qualitatively assessed user attitudes toward order set use. The study population included 790 patients with a primary diagnosis of asthma admitted to Columbus Children's Hospital between November 1, 2001 and November 30, 2003.