TY - JOUR
T1 - Interstitial lung disease in newborns
AU - Nogee, Lawrence M.
N1 - Funding Information:
L.M.N. has received funding from the US National Institutes of Health (NHLBI) ( U01HL134745 ), American Thoracic Society , and the Eudowood Board , and is a co-investigator on a study sponsored by United Therapeutics Corporation . None of the sponsors had any role in the preparation of this manuscript.
Publisher Copyright:
© 2017 Elsevier Ltd
PY - 2017/8
Y1 - 2017/8
N2 - 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.
AB - 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.
KW - Alveolar capillary dysplasia
KW - Genetic basis of disease
KW - Lung development
KW - Mutation
KW - Neuroendocrine cell hyperplasia of infancy
KW - Surfactant
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U2 - 10.1016/j.siny.2017.03.003
DO - 10.1016/j.siny.2017.03.003
M3 - Review article
C2 - 28363760
AN - SCOPUS:85016716873
SN - 1744-165X
VL - 22
SP - 227
EP - 233
JO - Seminars in Fetal and Neonatal Medicine
JF - Seminars in Fetal and Neonatal Medicine
IS - 4
ER -