Nike I Beckeringh1, Niels W Rutjes2, Joost van Schuppen3, Taco W Kuijpers1. 1. Department of Pediatric Hematology, Immunology and Infectious Diseases, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands. 2. Department of Pediatric Pulmonology, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands. 3. Department of Pediatric Radiology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.
Abstract
Introduction: Pediatric noncystic fibrosis (CF) bronchiectasis has a variety of causes. An early and accurate diagnosis may prevent disease progression and complications. Current diagnostics and yield regarding etiology are evaluated in a pediatric cohort at a tertiary referral center. Methods: Available data, including high-resolution computed tomography (HRCT) characteristics, microbiological testing, and immunological screening of all children diagnosed with non-CF bronchiectasis between 2003 and 2017, were evaluated. Results: In 91% of patients [n = 69; median age 9 (3-18 years)] etiology was established in the diagnostic process. Postinfection (29%) and immunodeficiency (29%) were most common, followed by congenital anomalies (10%), aspiration (7%), asthma (6%), and primary ciliary dyskinesia (1%). HRCT predominantly showed bilateral involvement in immunodeficient patients (85%) and those with idiopathic bronchiectasis (83%). Congenital malformations (71%) were associated with unilateral disease. Completion of the diagnostic process often led to a change of treatment as started after initial diagnosis. Conclusion: Using a comprehensive diagnostic protocol, the etiology of pediatric non-CF bronchiectasis was established in more than 90% of patients. HRCT provides additional diagnostic information as it points to either a more systemic or a more localized etiology. Adequate diagnostics and data analysis allow treatment to be specifically adapted to prevent disease progression. Copyright 2019, Mary Ann Liebert, Inc., publishers.
Introduction: Pediatric noncystic fibrosis (CF) bronchiectasis has a variety of causes. An early and accurate diagnosis may prevent disease progression and complications. Current diagnostics and yield regarding etiology are evaluated in a pediatric cohort at a tertiary referral center. Methods: Available data, including high-resolution computed tomography (HRCT) characteristics, microbiological testing, and immunological screening of all children diagnosed with non-CF bronchiectasis between 2003 and 2017, were evaluated. Results: In 91% of patients [n = 69; median age 9 (3-18 years)] etiology was established in the diagnostic process. Postinfection (29%) and immunodeficiency (29%) were most common, followed by congenital anomalies (10%), aspiration (7%), asthma (6%), and primary ciliary dyskinesia (1%). HRCT predominantly showed bilateral involvement in immunodeficientpatients (85%) and those with idiopathic bronchiectasis (83%). Congenital malformations (71%) were associated with unilateral disease. Completion of the diagnostic process often led to a change of treatment as started after initial diagnosis. Conclusion: Using a comprehensive diagnostic protocol, the etiology of pediatric non-CF bronchiectasis was established in more than 90% of patients. HRCT provides additional diagnostic information as it points to either a more systemic or a more localized etiology. Adequate diagnostics and data analysis allow treatment to be specifically adapted to prevent disease progression. Copyright 2019, Mary Ann Liebert, Inc., publishers.
Authors: Brian S Schwartz; Saba A Al-Sayouri; Jonathan S Pollak; Annemarie G Hirsch; Robert Kern; Bruce Tan; Atsushi Kato; Robert P Schleimer; Anju T Peters Journal: J Allergy Clin Immunol Date: 2022-03-18 Impact factor: 14.290