An Official American Thoracic Society Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy
Geoffrey Kurland,Robin R. Deterding,James S. Hagood,Lisa R. Young,Alan S. Brody,Robert G. Castile,Sharon D. Dell,Leland L. Fan,Aaron Hamvas,Bettina C. Hilman,Claire Langston,Lawrence M. Nogee,Gregory J. Redding +12 more
TLDR
Recommendations were formulated using a systematic approach 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.Abstract:
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. Recommendations were formulated using a systematic approach. Outcomes considered important included the accuracy of the diagnostic evaluation, complications of delayed or incorrect diagnosis, psychosocial complications affecting the patient’s or family’s quality of life, and death.Results: No controlled clinical trials were identified. Therefore, observational evidence and clinical experience informed judgments. These guidelines: (1) describe the clinical characteristics of neonates and infants (<2 yr of age) with diffuse lung disease (DLD); (2) list the common causes of DLD that should be e...read more
Citations
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European protocols for the diagnosis and initial treatment of interstitial lung disease in children
Andrew Bush,Steve Cunningham,Jacques de Blic,Angelo Barbato,Annick Clement,Ralph Epaud,Meike Hengst,Nural Kiper,Andrew G. Nicholson,Martin Wetzke,Deborah Snijders,Nicolaus Schwerk,Matthias Griese +12 more
TL;DR: Proposals for a staged investigation of chILD, starting from when the condition is suspected to defining the diagnosis, using pathways dependent on the clinical condition and the degree of illness of the child are reported.
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Diseases of Pulmonary Surfactant Homeostasis
TL;DR: The pulmonary surfactant system and the genetic causes of acute and chronic lung disease caused by disruption of alveolar homeostasis are considered.
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LungMAP: The Molecular Atlas of Lung Development Program
Maryanne E. Ardini-Poleske,Robert Clark,Charles Ansong,James P. Carson,Richard A. Corley,Gail H. Deutsch,James S. Hagood,Naftali Kaminski,Thomas J. Mariani,S. Steven Potter,Gloria S. Pryhuber,David Warburton,Jeffrey A. Whitsett,Scott M. Palmer,Namasivayam Ambalavanan +14 more
TL;DR: A brief description of research conducted by the consortium, database, and portal development and upcoming features that will enhance the LungMAP experience for a community of users are presented.
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Genotype–Phenotype Correlations for Infants and Children with ABCA3 Deficiency
Jennifer A. Wambach,Alicia Casey,Martha P. Fishman,Daniel J. Wegner,Susan E. Wert,F. Sessions Cole,Aaron Hamvas,Aaron Hamvas,Lawrence M. Nogee +8 more
TL;DR: Frameshift or nonsense ABCA3 mutations are predictive of neonatal presentation and poor outcome, whereas missense, splice site, and insertion/deletions are less reliably associated with age of presentation and prognosis.
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All the “RAGE” in lung disease: The receptor for advanced glycation endproducts (RAGE) is a major mediator of pulmonary inflammatory responses
TL;DR: The receptor for advanced glycation endproducts (RAGE) is a pro-inflammatory pattern recognition receptor (PRR) that has been implicated in the pathogenesis of numerous inflammatory diseases and is an important mediator in allergic airway inflammation and asthma.
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