Mortality from BPD has improved over the past three decades despite significant trends toward more pronounced prematurity and lower birth weights.

Purpose: Primary Ciliary Dyskinesia (PCD) is a rare disorder of mucociliary clearance leading to recurrent upper and lower respiratory tract infections. PCD is difficult to clinically distinguish from other entities leading to recurrent oto-sino-pulmonary infections, including primary immunodeficiency (PID). Nasal nitric oxide (nNO) is a sensitive and specific diagnostic test for PCD, but it has not been thoroughly examined in PID. Past publications have suggested an overlap in nNO levels among subjects with PCD and PID. We sought to determine if nNO measurements among patients diagnosed with PID would fall significantly above the established PCD diagnostic cutoff value of 77 nL/min. Methods: Children >5 years old and adults with definitive PID or PCD diagnoses were recruited from outpatient subspecialty clinics. Participants underwent nNO testing by standardized protocol using a chemiluminiescence analyzer and completed a questionnaire concerning their chronic otosino-pulmonary symptoms, including key clinical criteria specific to diagnosed PCD (neonatal *

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Children with chronic neurologic and chest wall diseases are at increased risk of postoperative respiratory complications. These complications include acute respiratory failure, atelectasis, pneumonia, need for reintubation, and need for tracheostomy and can carry significant negative impacts on patient outcomes, including mortality, and increased healthcare resource utilization. 1 As such, careful examination of risk factors in these complex children should be initiated in order to plan appropriate preoperative and postoperative interventions that help mitigate postoperative respiratory complications. Such interventions may preoperatively include initiation of respiratory support devices such as non-invasive ventilation (NIV) and airway clearance techniques and/or a plan

Background: Despite improved health, shorter stature is common in cystic fibrosis (CF). We aimed to describe height velocity (HV) and contribution of height-related genetic variants to height(HT) in CF. Methods: HV cohort – Standard deviation scores (-Z) for HT, mid-parental height-adjusted HT (MPAH), and HV were generated using our Pediatric Center’s CF Foundation registry data. HV-Z was compared to population means at each age (5–17y), the relationship of HV-Z with HT-Z assessed, and HT-Z compared to MPAH-Z. GRS cohort- HT genetic risk-Z (HT-GRS-Z) were determined for pancreatic exocrine sufficient (PS) and insufficient (PI) youth and adults from our CF center and their relationships with HT-Z assessed. Results: HV cohort: Average HV-Z was normal across ages in our cohort but was 1.5x-lower (p<0.01) for each SD decrease in HT-Z. MPAH-Z was lower than HT-Z (p< 0.001). GRS cohort: HT-GRS-Z more strongly correlated with HT-Z and better explained height variance in PS (rho=0.42;R 2 =0.25) vs. PI (rho=0.27;R 2 =0.11). Conclusions: Despite shorter stature compared to peers and mid-parental height, youth with CF generally have normal linear growth in mid- and late-childhood. PI tempered the heritability of height. These results suggest that, in CF, final height is determined early in life in CF and genetic potential is attenuated by other factors.

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Background: Infant respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD) remain important respiratory complications of preterm births. This study aims at establishing the incidence and impact of respiratory complications and extreme low birth weight (ELBW) on the healthcare utilization of a population of preterm infants.Methods: Retrospective cohort study using administrative healthcare databases of the province of Quebec (Canada). The main outcome measures of healthcare utilization, hospital admissions, hospital mortality and use of prescription drugs were studied in 55 033 subjects born prematurely with or without respiratory complications such as BPD or RDS between 1999 and 2009.Results: Preterm infants with BPD and RDS had significantly higher hospital readmissions per person-year. This trend persisted for the entire duration of the 10-year follow-up. Diagnoses of childhood asthma, attention deficit hyperactivity disorder and cerebral palsy were more frequent in BPD subjects, but were not influenced by birth weight. Extreme low birth weight carried odds ratio of 38.0 [33.5, 43.2] and 3.5 [3.2, 3.9] respectively for the occurrence of BPD and RDS, and was associated with greater mortality, longer hospital stay and more medical visits per person-year. Except for anxiolytics and sedatives, bronchopulmonary dysplasia subjects were not more likely to have been prescribed neurological and psychiatric medications.Conclusions: Birth weight is a major determinant in the occurrence of respiratory complications following a preterm birth. The impacts of BPD and extreme low birth weight following a preterm birth have lasting consequences on respiratory health and healthcare utilization.

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