Pediatric pulmonology 2021 year in review: Rare and diffuse lung disease.

The field of rare and diffuse pediatric lung disease is experiencing rapid progress as diagnostic and therapeutic options continue to expand. In this annual review, we discuss manuscripts published in Pediatric Pulmonology in 2021 in (1) children's interstitial and diffuse lung disease, (2) congenital airway and lung malformations, and (3) noncystic fibrosis bronchiectasis including primary ciliary dyskinesia. These include case reports, descriptive cohorts, trials of therapies, animal model studies, and review articles.

Multidisciplinary feeding treatment for a patient with interstitial lung disease.

Children with underlying medical and developmental conditions often present with complications that include feeding difficulties. Severe feeding difficulties may meet the criteria for the psychiatric diagnosis of Avoidant Restrictive Food Intake Disorder (ARFID). ARFID is a disruption in feeding/eating which may include weight loss/faltering growth, nutrient deficiencies, and/or reliance on a nutritionally complete formula to meet caloric needs.

Pediatric pulmonology 2020 year in review: Rare and diffuse lung disease.

Pediatric Pulmonology publishes original research, review articles, and case reports on topics related to a wide range of children's respiratory disorders. Here we review some of the most notable manuscripts published in 2020 in this journal on (1) children's interstitial lung disease (chILD), (2) congenital airway and lung anomalies, and (3) primary ciliary dyskinesia and other non-cystic fibrosis bronchiectasis. The articles reviewed are discussed in context with published works from other journals.

Care recommendations for the respiratory complications of esophageal atresia-tracheoesophageal fistula.

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity.

Novel Application of Aptamer Proteomic Analysis in Cystic Fibrosis Bronchoalveolar Lavage Fluid.

Purpose: Biomarkers are needed in cystic fibrosis (CF) to understand disease progression, assess response to therapy, and enrich enrollment for clinical trials. Aptamer-based proteomics have proven useful in blood samples. The aim is to evaluate proteins in bronchoalveolar lavage fluid (BALF) in CF children compared to controls and identify endotypes during CF exacerbations.

Analysis of Risk Factors for Abnormal Pulmonary Function in Pediatric Cancer Survivors.

Background: Childhood cancer survivors are at increased risk for pulmonary-related morbidity and mortality. The Children's Oncology Group Long-Term Follow-Up (COG-LTFU) guidelines recommend pulmonary function testing after treatment with bleomycin, busulfan, carmustine, lomustine, thoracic radiation, bone marrow transplant, or pulmonary surgery. The aim of this analysis was to determine the prevalence of pulmonary function abnormalities in a pediatric survivor cohort.

Impulse oscillometry: interpretation and practical applications.

Simple spirometry and body plethysmography have been routinely used in children aged > 5 years. New techniques based on physiologic concepts that were first described almost 50 years ago are emerging in research and in clinical practice for measuring pulmonary function in children. These techniques have led to an increased understanding of the pediatric lung and respiratory mechanics.

Bronchoalveolar lavage fluid cytokine profiles in neuroendocrine cell hyperplasia of infancy and follicular bronchiolitis.

Background: Neuroendocrine Cell Hyperplasia of Infancy (NEHI) and Follicular Bronchiolitis (FB) are rare pediatric diffuse lung diseases with poorly understood pathogenesis and similar clinical presentations. We sought to determine if cellular and cytokine profiles in bronchoalveolar lavage fluid (BALF) from subjects with NEHI and FB would differ from pediatric disease controls.

Methods: BALF was obtained from forty-one subjects classified into four disease groups: NEHI, Cystic Fibrosis (CF), other airway disease controls (DC), and FB during clinically indicated procedures.

Abnormal infant pulmonary function in young children with neuroendocrine cell hyperplasia of infancy.

Rationale: Lung function in children with neuroendocrine cell hyperplasia of infancy (NEHI) and correlations with future clinical outcomes are needed to guide clinical management.

Objective: To compare results of infant pulmonary function tests (IPFTs) in children with NEHI to disease control (DC) subjects and to correlate NEHI IPFTs with future outcomes.

Methods: We performed a retrospective, single center study of IPFT in subjects diagnosed by lung biopsy (NEHI) or clinically (NEHI syndrome) and in DC subjects evaluated for cancer or pre-hematopoietic stem cell transplantation (HSCT).

New coding in the International Classification of Diseases, Ninth Revision, for children's interstitial lung disease.

The term "children's interstitial lung disease" (chILD) refers to a heterogeneous group of rare and diffuse lung diseases associated with significant morbidity and mortality. These disorders include neuroendocrine cell hyperplasia of infancy, pulmonary interstitial glycogenosis, surfactant dysfunction mutations, and alveolar capillary dysplasia with misalignment of pulmonary veins. Diagnosis can be challenging, which may lead to a delay in recognition and treatment of these disorders.

Autoimmune polyendocrine syndrome type 1: Utility of KCNRG autoantibodies as a marker of active pulmonary disease and successful treatment with rituximab.

Autoimmune polyendocrine syndrome type 1 (APS-1), also known as Autoimmune Polyendocrinopathy Candidiasis and Ectodermal Dysplasia (APECD) is a disorder caused by mutations in the autoimmune regulator (AIRE) gene. In some APS-1 patients, significant pulmonary disease is observed. Autoantibodies directed against the potassium channel regulatory protein (KCNRG), found in epithelial cells of terminal bronchioles, have been suggested as a marker for pulmonary disease in APS-1 patients.

Use of flexible bronchoscopy in pediatric patients receiving extracorporeal membrane oxygenation (ECMO) support.

Introduction: Critically ill children treated with extracorporeal membrane oxygenation (ECMO) support frequently have respiratory complications amenable to evaluation by flexible bronchoscopy (FB). The safety and efficacy of FB in this setting has not been well described in children.

Methods: Retrospective analysis of 153 FBs in 79 children treated with ECMO at a single institution from 2000 to 2008.