TY - JOUR
T1 - Children's interstitial and diffuse lung disease
AU - Cunningham, Steven
AU - Jaffe, Adam
AU - Young, Lisa R
PY - 2019/6/18
Y1 - 2019/6/18
N2 - Children’s interstitial and diffuse lung disease (chILD) is a rare heterogenous group of conditions, with symptoms often overlapping more common conditions, impeding diagnosis and frustrating parents and clinicians alike. Clinical collaborations are improving diagnostic precision, disease phenotyping, mechanistic understanding and interventional therapeutic capability; however with over 200 conditions, chILD requires greater aligned international collaboration to address the knowledge gaps. Genetics play an increasing role in diagnosis, and thus may help align current classification systems. Empiric therapeutics are few in number, of no proven benefit and with significant side effects, particularly in infants. Novel therapeutics, postulated for a number of chILD conditions based on mechanistic observations are in development and represent real hope for those conditions to which they apply. Broader therapeutics against the downstream effects of chILD (i.e. fibrosis) are under consideration for chILD conditions, but require adequately validated biological outcome measures, something which the chILD community urgently needs to address.
AB - Children’s interstitial and diffuse lung disease (chILD) is a rare heterogenous group of conditions, with symptoms often overlapping more common conditions, impeding diagnosis and frustrating parents and clinicians alike. Clinical collaborations are improving diagnostic precision, disease phenotyping, mechanistic understanding and interventional therapeutic capability; however with over 200 conditions, chILD requires greater aligned international collaboration to address the knowledge gaps. Genetics play an increasing role in diagnosis, and thus may help align current classification systems. Empiric therapeutics are few in number, of no proven benefit and with significant side effects, particularly in infants. Novel therapeutics, postulated for a number of chILD conditions based on mechanistic observations are in development and represent real hope for those conditions to which they apply. Broader therapeutics against the downstream effects of chILD (i.e. fibrosis) are under consideration for chILD conditions, but require adequately validated biological outcome measures, something which the chILD community urgently needs to address.
U2 - 10.1016/S2352-4642(19)30117-8
DO - 10.1016/S2352-4642(19)30117-8
M3 - Review article
JO - The Lancet Child & Adolescent Health
JF - The Lancet Child & Adolescent Health
SN - 2352-4642
ER -