Rationale: Animal models suggest pre-eclampsia (Pre-E) affects alveolar development, but data from humans are lacking. Objective: Assess the impact of Pre-E on airway function, diffusion capacity, and respiratory morbidity in preterm and term infants born from mothers with Pre-E. Methods: Infants born from mothers with and without Pre-E were recruited for this study; term and preterm infants were included in both cohorts. Respiratory morbidity in the first 12 months of life was assessed through monthly phone surveys. Raised volume rapid thoracoabdominal compression and measurement of diffusion capacity of the lung to carbon monoxide (DLCO)) were performed at 6 months corrected age. Results: There were 146 infants in the Pre-E cohort and 143 in the control cohort. The Pre-E cohort was further divided into non-severe (N=41) and severe (N=105) groups. There was no significant difference in DCLO and DLCO/aveolar volume amongst the three groups. Forced vital capacity was similar amongst the three groups, but the non-severe Pre-E group had significantly higher forced expiratory flows that the other two. After adjusting for multiple covariates including prematurity, the severe Pre-E group had a lower risk for wheezing in the first year of life compared to the other two. Conclusions: Pre-E is not associated with reduced DLCO, lower forced expiratory flows, or increased wheezing in the first year of life. These results differ from animal models and highlight the the complex relationships between Pre-E and lung function and respiratory morbidity in human infants.

Objectives: The objective of this study is to compare the Dinakara and Cotes equations in their ability to predict post hematopoietic stem cell transplant (HSCT) pulmonary complications and mortality. Hypothesis We hypothesize the pre-transplant diffusing capacity adjusted for hemoglobin (DLCOHgb) by the Cotes equation in pediatric patients undergoing HSCT will predict morbidity and mortality more accurately than the Dinakara equation. Study-Design: Data was collected retrospectively from chart review of patients who underwent their first HSCT at Riley Hospital for Children using a database maintained by the Pediatric Stem Cell Transplant Program. Patient-Subject Selection: Patients who performed pre-transplant diffusing capacity for carbon monoxide (DLCO) that met ATS criteria, and a hemoglobin recorded within 7 days of their pulmonary function testing were included. Methodology: Paired t-tests and ANOVA models were used to define any differences between the two equations at baseline and when stratifying by hemoglobin level. Logistic regression models were used to determine associations between the Dinakara and Cotes equation with mortality at one- and three-years post-transplant. Results: 90 patients underwent HSCT during the study period, and 69 patients met inclusion criteria. Odds ratios for mortality using DLCO corrected for the Dinakara (1.08 SD 0.98-1.19) and Cotes (1.09 SD 0.97-1.22) were similar (p-value > 0.05). Neither Dinakara or Cotes corrective equation was superior at predicting pulmonary complications. (p-values 0.1388 and 0.5246 respectively) Conclusions: The Dinakara and Cotes equations differed in their calculation of DLCOHgb at lower Hb levels, their ability to predict mortality and pulmonary complications after HSCT was not different.

ABSTRACT Background: The goal of this study was to identify clinical features associated with abnormal infant pulmonary function tests (iPFTs), specifically functional residual capacity (FRC), in infants with cystic fibrosis (CF) diagnosed via newborn screen (NBS). We hypothesized that poor nutritional status in the first 6-12 months would be associated with increased FRC at 12-24 months. Methods: This study utilized a combination of retrospectively and prospectively collected data from ongoing research studies and iPFTs performed for clinical indications. Demographic and clinical features were obtained from the electronic medical record. Forced expiratory flows and volumes were obtained using the raised volume rapid thoracoabdominal technique (RVRTC) and FRC was measured via plethysmography. Results: A total of 45 CF NBS infants had iPFTs performed between 12-24 months. Mean forced vital capacity, forced expiratory volume in 0.5 second, and forced expiratory flows were all within normal limits. In contrast, the mean FRC z-score was 2.18 (95%CI=1.48, 2.88) and the mean respiratory rate (RR) z-score was 1.42 (95%CI=0.95, 1.89). There was no significant association between poor nutritional status and abnormal lung function. However, there was a significant association between higher RR and increased FRC, and a RR cutoff of 36 breaths/min resulted in 92% sensitivity to detect hyperinflation with 32% specificity. Conclusions: These results suggest that FRC is a more sensitive measure of early CF lung disease than RVRTC measurements and that RR may be a simple, non-invasive clinical marker to identify CF NBS infants with hyperinflation.

To assess the impact of COVID-19 restrictions on cystic fibrosis (CF) pulmonary exacerbations (PEx) we performed a retrospective review of PEx events at our CF Center and compared the rate of PEx in 2019 vs 2020. Restrictions on social interaction due to the COVID-19 pandemic were associated with a lower rate of PEx at our pediatric CF Center, suggesting that these restrictions also reduced exposure to other respiratory viral infection in children with CF.

Introduction: The Lung Clearance Index (LCI) derived from the multiple-breath washout test (MBW), is both feasible and sensitive to early lung disease detection in young children with cystic fibrosis and asthma. The utility of LCI has not been studied in children with sickle cell disease (SCD). We hypothesized that children with SCD, with or without asthma or airway hyper reactivity (AHR), would have an elevated LCI compared to healthy controls. Methods: Children with SCD from a single center between the ages of 6-18 years were studied at baseline health and completed MBW, spirometry, plethysmography and blood draws for serum markers. Results were compared to healthy controls of similar race, age and gender. Results: Control subjects (n=35) had a significantly higher daytime oxygen saturation level, weight and body mass index (BMI) but not height compared to subjects with SCD (n=34). Total Lung Capacity(TLC) z-scores were significantly higher in the healthy controls compared to those with SCD (0.87 (1.13), 0.02 (1.27), p=0.005) while differences in Forced Expiratory Volume in 1 second (FEV1) z-scores approached significance (0.26 (0.97), -0.22 (1.09), p=0.055). There was no significant difference in LCI among the comparison groups (7.29 (0.72), 7.40 (0.69), p=0.514). Conclusion: LCI did not differentiate SCD from healthy controls in children between the ages of 6 and 18 years at baseline health. TLC may be an important pulmonary function measure to follow longitudinally in the pediatric SCD population.

RATIONALE: Outpatient treatment of lower respiratory tract infection (LRTI) in tracheostomy dependent children varies institutionally. The objective of this study was to identify whether only increasing airway clearance (AWC) increased the odds of hospitalization within 28 days of treatment. Our hypothesis was that those treated with antibiotics were less likely to be hospitalized. METHODS: We retrospectively reviewed medical charts of children who were tracheostomy dependent between 2012-2019 and followed at our institution. We recorded recommendations with each sick call, i.e. prescription of antibiotics and/or increase in frequency of airway clearance. Generalized estimating equation models were used to determine whether the recommendation to increase AWC frequency was associated with an increased risk of hospitalization within 4 weeks, as compared to the prescription of oral and/or inhaled antibiotics. RESULTS: Of the eighty -two patients reviewed, there were 283 unique episodes of LRTI. 160 (45%) episodes involved increasing AWC alone and 195 (55%) were given an antibiotic in addition to increasing AWC. Of those who received AWC only, 21.7% were hospitalized within 28 days of treatment, and 13.8% were hospitalized after treatment with increased AWC and oral/inhaled antibiotics, p= 0.08. Those who received only AWC did not have significantly higher odds of hospitalization within 28 days of treatment, compared to those who received an antibiotic: adjusted OR 1.47 (95% CI: 0.75, 2.86); p=.26. CONCLUSIONS: In this retrospective cohort study of pediatric patients with tracheostomy, a recommendation to increase airway clearance only versus initiating an antibiotic was not associated with increased odds of hospitalization.