PMID- 35657736 OWN - NLM STAT- MEDLINE DCOM- 20221109 LR - 20231102 IS - 2325-6621 (Electronic) IS - 2329-6933 (Print) IS - 2325-6621 (Linking) VI - 19 IP - 11 DP - 2022 Nov TI - Association of Neonatal Hospital Length of Stay with Lung Function in Primary Ciliary Dyskinesia. PG - 1865-1870 LID - 10.1513/AnnalsATS.202202-116OC [doi] AB - Rationale: Primary ciliary dyskinesia (PCD), an inherited lung disease, is characterized by abnormal ciliary function leading to progressive bronchiectasis. There is wide variability in respiratory disease severity at birth and later in life. Objectives: To evaluate the association between neonatal hospital length of stay (neonatal-LOS) and supplemental oxygen duration (SuppO(2)) with lung function in pediatric PCD. We hypothesized that longer neonatal-LOS and SuppO(2) are associated with worse lung function (i.e., forced expiratory volume in 1 second percent predicted [FEV(1)pp]). Methods: We performed a secondary analysis of the Genetic Disorders of Mucociliary Clearance Consortium prospective longitudinal multicenter cohort study. Participants enrolled, during 2006-2011, were <19 years old with a confirmed PCD diagnosis and followed annually for 5 years. The exposure variables were neonatal-LOS and SuppO(2), counted in days since birth. The outcome, FEV(1)pp, was measured annually by spirometry. The associations of neonatal-LOS and SuppO(2) with FEV(1)pp were evaluated with a linear mixed-effects model with repeated measures and random intercepts, adjusted for age and ciliary ultrastructural defects. Results: Included were 123 participants (male, 47%; mean enrollment age, 8.3 yr [range, 0 to 18 yr]) with 578 visits (median follow-up, 5 yr). The median neonatal-LOS was 9 d (range, 1 to 90 d), and median SuppO(2) was 5 d (range, 0 to 180 d). Neonatal-LOS was associated with worse lung function (-0.27 FEV(1)pp/d [95% confidence interval, -0.53 to -0.01]; P = 0.04). SuppO(2) was not associated with lung function. Conclusions: Neonatal-LOS is associated with worse lung function in pediatric PCD, independent of age and ultrastructural defects. Future research on the mechanisms of neonatal respiratory distress and its management may help us understand the variability of lung health outcomes in PCD. FAU - Wee, Wallace B AU - Wee WB AUID- ORCID: 0000-0003-4291-3513 AD - Respiratory Medicine. AD - Child Health Evaluative Sciences, Hospital for Sick Children. AD - Department of Pediatrics, Faculty of Medicine, and. AD - IHPME, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada. FAU - Leigh, Margaret W AU - Leigh MW AUID- ORCID: 0000-0002-6420-2459 AD - Pediatric Pulmonology, University of North Carolina, Chapel Hill, North Carolina. FAU - Davis, Stephanie D AU - Davis SD AD - Pediatric Pulmonology, University of North Carolina, Chapel Hill, North Carolina. FAU - Rosenfeld, Margaret AU - Rosenfeld M AD - Department of Pediatrics, University of Washington School of Medicine and Seattle Children's Research Institute, Seattle, Washington. FAU - Sullivan, Kelli M AU - Sullivan KM AD - Department of Medicine and. FAU - Sawras, Michael G AU - Sawras MG AD - Child Health Evaluative Sciences, Hospital for Sick Children. FAU - Ferkol, Thomas W AU - Ferkol TW AD - Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri. FAU - Knowles, Michael R AU - Knowles MR AD - Department of Medicine and. AD - Department of Pathology and Laboratory Medicine, Marsico Lung Institute, University of North Carolina School of Medicine, Chapel Hill, North Carolina. FAU - Milla, Carlos AU - Milla C AD - Center for Excellence in Pulmonary Biology, Stanford University School of Medicine, Stanford, California. FAU - Sagel, Scott D AU - Sagel SD AUID- ORCID: 0000-0001-6172-4465 AD - Department of Pediatrics, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado; and. FAU - Zariwala, Maimoona A AU - Zariwala MA AUID- ORCID: 0000-0003-1619-1393 AD - Department of Pathology and Laboratory Medicine, Marsico Lung Institute, University of North Carolina School of Medicine, Chapel Hill, North Carolina. FAU - Pullenayegum, Eleanor AU - Pullenayegum E AD - Child Health Evaluative Sciences, Hospital for Sick Children. FAU - Dell, Sharon D AU - Dell SD AUID- ORCID: 0000-0003-2169-9407 AD - Child Health Evaluative Sciences, Hospital for Sick Children. AD - Department of Pediatrics, Faculty of Medicine, and. AD - IHPME, Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada. AD - Division of Respiratory Medicine, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada. LA - eng GR - R01 HL071798/HL/NHLBI NIH HHS/United States GR - U54 HL096458/HL/NHLBI NIH HHS/United States GR - UL1 TR002535/TR/NCATS NIH HHS/United States GR - UM1 HG006504/HG/NHGRI NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't PL - United States TA - Ann Am Thorac Soc JT - Annals of the American Thoracic Society JID - 101600811 SB - IM MH - Child MH - Humans MH - Infant, Newborn MH - Male MH - *Ciliary Motility Disorders MH - Cohort Studies MH - Hospitals MH - *Kartagener Syndrome/diagnosis MH - Length of Stay MH - Lung MH - Prospective Studies MH - Infant MH - Child, Preschool MH - Adolescent PMC - PMC9667809 OTO - NOTNLM OT - lung function OT - neonatal OT - pediatric OT - primary ciliary dyskinesia EDAT- 2022/06/04 06:00 MHDA- 2022/11/04 06:00 PMCR- 2023/11/01 CRDT- 2022/06/03 12:53 PHST- 2022/06/04 06:00 [pubmed] PHST- 2022/11/04 06:00 [medline] PHST- 2022/06/03 12:53 [entrez] PHST- 2023/11/01 00:00 [pmc-release] AID - 10.1513/AnnalsATS.202202-116OC [doi] PST - ppublish SO - Ann Am Thorac Soc. 2022 Nov;19(11):1865-1870. doi: 10.1513/AnnalsATS.202202-116OC.