Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease.

Background: Childhood interstitial lung disease (chILD) encompasses a group of rare heterogeneous respiratory conditions associated with significant morbidity and mortality. Reports suggest that many patients diagnosed with chILD continue to have potentially progressive or fibrosing disease into adulthood. Over the last decade, the spectrum of conditions within chILD has widened substantially, with the discovery of novel entities through advanced genetic testing.

The US national registry for childhood interstitial and diffuse lung disease: Report of study design and initial enrollment cohort.

Introduction: Childhood interstitial and diffuse lung disease (chILD) encompasses a broad spectrum of rare disorders. The Children's Interstitial and Diffuse Lung Disease Research Network (chILDRN) established a prospective registry to advance knowledge regarding etiology, phenotype, natural history, and management of these disorders.

Methods: This longitudinal, observational, multicenter registry utilizes single-IRB reliance agreements, with participation from 25 chILDRN centers across the U.

Pulmonary Complications in Patients With Fontan Circulation: JACC Review Topic of the Week.

The Fontan operation has resulted in significant improvement in survival of patients with single ventricle physiology. As a result, there is a growing population of individuals with Fontan physiology reaching adolescence and adulthood. Despite the improved survival, there are long-term morbidities associated with the Fontan operation.

The lung in inborn errors of immunity: From clinical disease patterns to molecular pathogenesis.

In addition to being a vital organ for gas exchange, the lung is a crucial immune organ continuously exposed to the external environment. Genetic defects that impair immune function, called inborn errors of immunity (IEI), often have lung disease as the initial and/or primary manifestation. Common types of lung disease seen in IEI include infectious complications and a diverse group of diffuse interstitial lung diseases.

Neuroendocrine Cell Hyperplasia of Infancy. Clinical Score and Comorbidities.

Neuroendocrine cell hyperplasia of infancy (NEHI) is an important form of children's interstitial and diffuse lung disease for which the diagnostic strategy has evolved. The prevalence of comorbidities in NEHI that may influence treatment has not been previously assessed. To evaluate a previously unpublished NEHI clinical score for assistance in diagnosis of NEHI and to assess comorbidities in NEHI.

Postinfectious Bronchiolitis Obliterans in Children: Diagnostic Workup and Therapeutic Options: A Workshop Report.

Bronchiolitis obliterans (BO) is a rare, chronic form of obstructive lung disease, often initiated with injury of the bronchiolar epithelium followed by an inflammatory response and progressive fibrosis of small airways resulting in nonuniform luminal obliteration or narrowing. The term BO comprises a group of diseases with different underlying etiologies, courses, and characteristics. Among the better recognized inciting stimuli leading to BO are airway pathogens such as adenovirus and mycoplasma, which, in a small percentage of infected children, will result in progressive fixed airflow obstruction, an entity referred to as postinfectious bronchiolitis obliterans (PIBO).

Home Oxygen Therapy for Children. An Official American Thoracic Society Clinical Practice Guideline.

Background: Home oxygen therapy is often required in children with chronic respiratory conditions. This document provides an evidence-based clinical practice guideline on the implementation, monitoring, and discontinuation of home oxygen therapy for the pediatric population.

Methods: A multidisciplinary panel identified pertinent questions regarding home oxygen therapy in children, conducted systematic reviews of the relevant literature, and applied the Grading of Recommendations, Assessment, Development, and Evaluation approach to rate the quality of evidence and strength of clinical recommendations.

Perception of Pulmonary Function in Children with Asthma and Cystic Fibrosis.

Under-perception of pulmonary dysfunction may delay appropriate treatment, while over-perception may result in unnecessary treatments. To evaluate the ability of patients with asthma or cystic fibrosis and their subspecialty caregivers to assess changes in lung function based on their subjective clinical impressions. Patients were asked to qualitatively describe how they felt compared to their prior visit (same/better/worse) and to quantitatively estimate their forced expiratory volume in 1 s (FEV) after being reminded of their FEV at the prior visit.

Airway microbiota across age and disease spectrum in cystic fibrosis.

Our objectives were to characterise the microbiota in cystic fibrosis (CF) bronchoalveolar lavage fluid (BALF), and determine its relationship to inflammation and disease status.BALF from paediatric and adult CF patients and paediatric disease controls undergoing clinically indicated bronchoscopy was analysed for total bacterial load and for microbiota by 16S rDNA sequencing.We examined 191 BALF samples (146 CF and 45 disease controls) from 13 CF centres.

Obesity and Airway Dysanapsis in Children with and without Asthma.

Rationale: For unclear reasons, obese children with asthma have higher morbidity and reduced response to inhaled corticosteroids.

Objectives: To assess whether childhood obesity is associated with airway dysanapsis (an incongruence between the growth of the lungs and the airways) and whether dysanapsis is associated with asthma morbidity.

Methods: We examined the relationship between obesity and dysanapsis in six cohorts of children with and without asthma, as well as the relationship between dysanapsis and clinical outcomes in children with asthma.

Antiinflammatory effects of bromodomain and extraterminal domain inhibition in cystic fibrosis lung inflammation.

Significant morbidity in cystic fibrosis (CF) results from chronic lung inflammation, most commonly due to infection. Recent data suggest that IL-17 contributes to pathological inflammation in the setting of abnormal mucosal immunity, and type 17 immunity-driven inflammatory responses may represent a target to block aberrant inflammation in CF. Indeed, transcriptomic analysis of the airway epithelium from CF patients undergoing clinical bronchoscopy revealed upregulation of IL-17 downstream signature genes, implicating a substantial contribution of IL-17-mediated immunity in CF lungs.

A novel surfactant protein C gene mutation associated with progressive respiratory failure in infancy.

Mutations of the Surfactant Protein C (SPC) gene (SFTPC) have been associated with childhood interstitial lung disease (chILD) with variable age of onset, severity of lung disease, and outcomes. We report a novel mutation in SFTPC [c.435G->A, p.

Site of Bronchoalveolar Lavage Via Flexible Bronchoscopy and Fluid Return in Children.

Background: Despite its widespread use as a diagnostic tool, the procedure for bronchoalveolar lavage (BAL) via flexible bronchoscopy is not standardized in children. Our objective was to examine the dissimilarities in fluid return between the different lobes in children undergoing flexible bronchoscopies with BAL.

Methods: We conducted a review of all pediatric flexible bronchoscopies with BAL conducted at a single institution over a 2-year period.

Subjective and Objective Assessments of Flow-Volume Curve Configuration in Children and Young Adults.

Rationale: Analysis of maximal expiratory flow-volume curves (MEFVCs) allows for determination of airway obstruction, but quantitative methods to describe these curves are not commonly used.

Objectives: We sought to determine the variability in MEFVC concavity assessment by pulmonary physicians, whether objective indices of concavity can be substituted for subjective expert impression, and whether MEFVC concavity correlates with clinical outcomes.

Methods: A survey of 37 MEFVCs (plus 3 duplicates) was sent to pulmonologists for quantitative assessment of MEFVC concavity.

Diffuse Lung Disease in Biopsied Children 2 to 18 Years of Age. Application of the chILD Classification Scheme.

Rationale: Children's Interstitial and Diffuse Lung Disease (chILD) is a heterogeneous group of disorders that is challenging to categorize. In previous study, a classification scheme was successfully applied to children 0 to 2 years of age who underwent lung biopsies for chILD. This classification scheme has not been evaluated in children 2 to 18 years of age.

Surfactant Administration During Pediatric Extracorporeal Membrane Oxygenation.

Administering surfactant during pediatric extracorporeal membrane oxygenation (ECMO) may influence important clinical variables but has been insufficiently described. Ninety-six courses of ECMO from our center were retrospectively assessed, and 89 surfactant doses were identified during 37 ECMO courses. Surfactant administration was associated with a respiratory indication for ECMO and increased durations of ECMO and positive pressure ventilation.

Official American Thoracic Society technical standards: flexible airway endoscopy in children.

Background: Flexible airway endoscopy (FAE) is an accepted and frequently performed procedure in the evaluation of children with known or suspected airway and lung parenchymal disorders. However, published technical standards on how to perform FAE in children are lacking.

Methods: The American Thoracic Society (ATS) approved the formation of a multidisciplinary committee to delineate technical standards for performing FAE in children.

Dexmedetomidine for infant pulmonary function testing.

For the last thirty years, oral chloral hydrate has been used for sedation of infants for lung function testing. Recently, however, availability of chloral hydrate became severely limited in the United States after two manufacturers discontinued manufacturing in 2012. Due to these limitations and the recent and ongoing shortage of chloral hydrate, other medications have been proposed for lung function testing, including midazolam and propofol.

Comparison of two aspiration techniques of bronchoalveolar lavage in children.

Background: Although bronchoalveolar lavage (BAL) via flexible bronchoscopy is an essential diagnostic tool, its technique is not standardized in children. Our objective was to compare two different aspiration techniques of BAL in children (continuous wall suction vs. handheld syringe suction) in regards to the percentage of fluid recovered and the odds of performing a technically acceptable procedure (i.

A survey of training in pediatric flexible bronchoscopy.

Objective: There is currently no evidence-based method for defining competency in pediatric flexible bronchoscopy (FB). Based on expert opinion, guidelines using numbers of procedures have been published in defining competency for pediatric FB. The purpose of this study was to formally survey the opinion of USA pediatric pulmonology training directors about the assessment of competency and training experiences in pediatric FB in their programs.

An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy.

Background: There is growing recognition and understanding of the entities that cause interstitial lung disease (ILD) in infants. These entities are distinct from those that cause ILD in older children and adults.

Methods: A multidisciplinary panel was convened to develop evidence-based guidelines on the classification, diagnosis, and management of ILD in children, focusing on neonates and infants under 2 years of age.

Radiation exposure from diagnostic imaging in the pediatric intensive care unit.

Objective: To determine the range of radiation exposure from diagnostic imaging in children requiring mechanical ventilation.

Design: Prospective, observational.

Setting: Tertiary pediatric critical care unit.