4.2 Article

Interstitial lung disease in newborns

Journal

SEMINARS IN FETAL & NEONATAL MEDICINE
Volume 22, Issue 4, Pages 227-233

Publisher

ELSEVIER SCI LTD
DOI: 10.1016/j.siny.2017.03.003

Keywords

Lung development; Alveolar capillary dysplasia; Surfactant; Mutation; Genetic basis of disease; Neuroendocrine cell hyperplasia of infancy

Categories

Funding

  1. US National Institutes of Health (NHLBI) [U01HL134745]
  2. American Thoracic Society
  3. Eudowood Board
  4. United Therapeutics Corporation

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The term 'interstitial lung disease' (ILD) refers to a group of disorders involving both the airspaces and tissue compartments of the lung, and these disorders are more accurately termed diffuse lung diseases. Although rare, they are associated with significant morbidity and mortality, with the prognosis depending upon the specific diagnosis. The major categories of ILD in children that present in the neonatal period include developmental disorders, growth disorders, surfactant dysfunction disorders, and specific conditions of unknown etiology unique to infancy. Whereas lung histopathology has been the gold standard for the diagnosis of ILD, as many of the disorders have a genetic basis, non-invasive diagnosis is feasible, and characteristic clinical and imaging features may allow for specific diagnosis in some circumstances. The underlying mechanisms, clinical, imaging, and lung pathology features and outcomes of ILD presenting in newborns are reviewed with an emphasis on genetic mechanisms and diagnosis. (C) 2017 Elsevier Ltd. All rights reserved.

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