Association of Pseudomonas aeruginosa infection stage with lung function trajectory in children with cystic fibrosis.

Background: Pseudomonas aeruginosa (Pa) infection in cystic fibrosis (CF) is characterized in stages: never (prior to first positive culture) to incident (first positive culture) to chronic. The association of Pa infection stage with lung function trajectory is poorly understood and the impact of age on this association has not been examined. We hypothesized that FEV decline would be slowest prior to Pa infection, intermediate after incident infection and greatest after chronic Pa infection.

Genetic Modifiers of Cystic Fibrosis Lung Disease Severity: Whole-Genome Analysis of 7,840 Patients.

Lung disease is the major cause of morbidity and mortality in persons with cystic fibrosis (pwCF). Variability in CF lung disease has substantial non-CFTR (CF transmembrane conductance regulator) genetic influence. Identification of genetic modifiers has prognostic and therapeutic importance.

Strategies to increase uptake of voluntary medical male circumcision among men aged 25-39 years in Nyanza Region, Kenya: Results from a cluster randomized controlled trial (the TASCO study).

Introduction: Voluntary medical male circumcision (VMMC) for HIV prevention began in Nyanza Region, Kenya in 2008. By 2014, approximately 800,000 VMMCs had been conducted, and 84.9% were among males aged 15-24 years.

Airway Disease in Children with Primary Ciliary Dyskinesia: Impact of Ciliary Ultrastructure Defect and Genotype.

Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary clearance, recurrent respiratory infections, progressive airway damage, and obstructive lung disease. Although the association of ciliary ultrastructure defect/genotype with the severity of airflow obstruction has been well characterized, their association with airway abnormalities on chest computed tomography (CT) has been minimally evaluated. We sought to delineate the association of ciliary defect class/genotype with chest CT scores in children with PCD.

Systemic Corticosteroids in the Management of Pediatric Cystic Fibrosis Pulmonary Exacerbations.

Pulmonary exacerbation (PEx) events contribute to lung function decline in people with cystic fibrosis (CF). CF Foundation PEx guidelines note that a short course of systemic corticosteroids may offer benefit without contributing to long-term adverse effects. However, insufficient evidence exists to recommend systemic corticosteroids for PEx treatment.

Performance and usability of a new mobile application for measuring respiratory rate in young children with acute lower respiratory infections.

Objectives: Respiratory rate (RR) measurement is critical to diagnosing pneumonia in resource-constrained settings, but accurate RR measurement is challenging. The acute lower respiratory illness treatment and evaluation (ALRITE) mobile phone application (app), designed to help healthcare workers (HCWs) manage pediatric respiratory illnesses, includes a semiautomated RR counter. This study aimed to evaluate the accuracy and usability of the ALRITE RR counter and a commercially available RR counter app, RRate, with a reference standard.

CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor.

The chloride channel dysfunction caused by deleterious cystic fibrosis transmembrane conductance regulator (CFTR) variants generally correlates with severity of cystic fibrosis (CF). However, 3 adults bearing the common severe variant p.Phe508del (legacy: F508del) and a deletion variant in an ivacaftor binding region of CFTR (p.

Clinical Outcomes of Antipseudomonal versus Other Antibiotics among Children with Cystic Fibrosis without .

Antibiotic selection for pulmonary exacerbation (PEx) management in children with cystic fibrosis is typically guided by prior respiratory culture results. Although antipseudomonal antibiotics are often used in children with chronic (Pa) airway infection, no data exist to guide antibiotic selection in children who are culture negative for Pa for ≥1 year. To determine among children classified as 1, 2, or 3 years' Pa negative if PEx treatment with at least one oral and/or intravenous antipseudomonal antibiotic is associated with improved clinical outcomes compared with treatment with antibiotics not effective against Pa.

Impact of guideline-recommended dietitian assessments on weight gain in infants with cystic fibrosis.

Background: Cystic fibrosis (CF)-specialized nutrition care strives to meet normal infant growth, but the relationship of dietitian assessments to weight outcomes is unknown. We characterize nutrition management for inadequate weight gain and assess association of dietitian assessments and center-level weight-for-age Z-scores (WAZ).

Methods: We used encounter data from 226 infants across 28 US CF Centers from the Baby Observational Nutritional study between January 2012 through December 2017.

Comparing encounter-based and annualized chronic pseudomonas infection definitions in cystic fibrosis.

Chronic Pseudomonas aeruginosa (Pa) infection is associated with increased morbidity and mortality in people with cystic fibrosis (CF). There is no gold standard definition of chronic Pa infection in CF. We compared chronic Pa definitions using encounter-based versus annualized data in the Early Pseudomonas Infection Control (EPIC) Observational study cohort, and subsequently compared annualized chronic Pa definitions across a range of U.

Influence of gender and sexual identity on adolescent skin health.

Background/objective: Studies have identified dermatologic conditions and relevant skin-related behaviors that distinctly or disproportionately impact sexual and gender minority (SGM) adults compared with their cisgender/heterosexual counterparts, but whether these observations apply to SGM adolescents remains unknown. We aimed to describe the nature and frequency of skin conditions in SGM youth relative to their cisgender/heterosexual peers and explore adolescents' attitudes toward their skin health and accessing dermatologic care.

Methods: SGM and cisgender/heterosexual youth aged 13-21 years seen at Seattle Children's Hospital Adolescent Medicine and Gender clinics from June to December 2019 were invited to participate in this cross-sectional survey study, with subsequent statistical analysis.

Influence of Blood Pressure Percentile Reporting on the Recognition of Elevated Blood Pressures.

Objectives: To explore the impact of displaying blood pressure (BP) percentiles with BP readings in the electronic health record (EHR) on the recognition of children with elevated blood pressures (EBPs).

Methods: This was a retrospective cohort study of children (ages 1-17), including inpatients and outpatients, with at least 1 EHR noninvasive BP recording. In phase 1, BP percentiles were calculated, stored, and not displayed to clinicians.

Association Between Number of Intravenous Antipseudomonal Antibiotics and Clinical Outcomes of Pediatric Cystic Fibrosis Pulmonary Exacerbations.

Background: Pulmonary exacerbations (PEx) in people with cystic fibrosis (PwCF) are associated with significant morbidity. While standard PEx treatment for PwCF with Pseudomonas aeruginosa infection includes two IV antipseudomonal antibiotics, little evidence exists to recommend this approach. This study aimed to compare clinical outcomes of single versus double antipseudomonal antibiotic use for PEx treatment.

Racial and Language Disparities in Pediatric Emergency Department Triage.

Objective: The aim of this study was to assess the impact race and language have on emergency department (ED) triage scores while accounting for illness severity. We hypothesized that non-White and non-English-speaking patients were assigned lower-acuity triage scores compared with White and English-speaking patients, respectively.

Methods: We used a chart review-based retrospective cohort study design, examining patients aged 0 to 17 years at our pediatric ED from July 2015 through June 2016.

Association of Intensity of Antipseudomonal Antibiotic Therapy With Risk of Treatment-Emergent Organisms in Children With Cystic Fibrosis and Newly Acquired Pseudomonas Aeruginosa.

Background: While Pseudomonas aeruginosa (Pa) eradication regimens have contributed to a decline in Pa prevalence in people with cystic fibrosis (CF), this antibiotic exposure might increase the risk of acquisition of drug-resistant organisms. This study evaluated the association between antipseudomonal antibiotic exposure intensity and acquisition risk of drug-resistant organisms among children with CF and new Pa infection.

Methods: We utilized data from the Early Pseudomonas Infection Control Clinical Trial (EPIC CT), a randomized controlled trial comparing Pa eradication strategies in children with CF and new Pa.

Effect of Concomitant Azithromycin and Tobramycin Use on Cystic Fibrosis Pulmonary Exacerbation Treatment.

Pulmonary exacerbations (PExs) are associated with significant morbidity in people with cystic fibrosis (CF). Severe PExs are treated with intravenous antibiotics, including tobramycin. CF care guidelines recommend continuing chronic maintenance medications during PEx treatment.

Association of Inhaled Antibiotics in Addition to Standard Intravenous Therapy and Outcomes of Pediatric Inpatient Pulmonary Exacerbations.

Considerable morbidity and disease progression in people with cystic fibrosis (CF) result from pulmonary exacerbations (PExs). PEx guidelines note insufficient evidence to recommend for or against the concomitant use of inhaled and intravenous antibiotics. We hypothesize that the addition of inhaled antibiotics for PEx therapy is associated with improvements in lung function and a longer time to next PEx compared with standard intravenous antibiotics alone.

Knowledge acquisition and retention after a high flow training programme in Peru: a quasi-experimental single group pre-post design.

Objective: Respiratory infections remain the leading infectious cause of death in children under 5 and disproportionately affect children in resource-limited settings. Implementing non-invasive respiratory support can reduce respiratory-related mortality. However, maintaining competency after deployment can be difficult.

Genital Shedding of Human Immunodeficiency Virus Type-1 (HIV) When Antiretroviral Therapy Suppresses HIV Replication in the Plasma.

Background: During antiretroviral treatment (ART) with plasma HIV RNA below the limit of quantification, HIV RNA can be detected in genital or rectal secretions, termed discordant shedding (DS). We hypothesized that proliferating cells produce virions without HIV replication.

Methods: ART-naive Peruvians initiating ART were observed for DS over 2 years.

Nasal high flow therapy introduction lowers reintubation risk in a Peruvian paediatric intensive care unit.

Aim: We examined the impact of introducing high-flow nasal oxygen therapy (HFNT) on children under five with post-extubation respiratory failure in a paediatric intensive care unit (PICU) in Peru.

Methods: This quasi-experimental study compared clinical outcomes before and after initial HFNT deployment in the PICU at Instituto Nacional de Salud del Niño in Lima in June 2016. We compared three groups: 29 received post-extubation HFNT and 17 received continuous positive airway pressure (CPAP) from 2016-17 and 12 historical controls received CPAP from 2012-16.

Prevalence and clinical associations of Staphylococcus aureus small-colony variant respiratory infection in children with cystic fibrosis (SCVSA): a multicentre, observational study.

Background: Staphylococcus aureus is the bacterium cultured most often from respiratory secretions of people with cystic fibrosis. Both meticillin-susceptible S aureus and meticillin-resistant S aureus (MRSA) can adapt to form slow-growing, antibiotic-resistant isolates known as small-colony variants that are not routinely identified by clinical laboratories. We aimed to determine the prevalence and clinical significance of S aureus small-colony variants and their subtypes among children with cystic fibrosis.

Comparison between epidural and opioid analgesia for infants undergoing major abdominal surgery.

Background: Epidural analgesia is considered optimal for postoperative pain management after major abdominal surgery. The potential to decrease anesthetic and opioid exposure is particularly desirable for infants, given their vulnerability to respiratory depression and concern for anesthetic neurotoxicity. We reviewed our experience with infants undergoing major abdominal surgery to determine if epidural catheter use decreased anesthetic and opioid exposure and improved postoperative analgesia.