Introduction: Newborn screening (NBS) for cystic fibrosis (CF) was implemented in all US states and DC by 2010. This hypothesis generating study was designed to form the basis of additional research and to plan quality improvement initiatives. The aims were to describe the outcomes of infants with CF born during the first 9 years of universal NBS. Methods: We included participants in the CF Foundation Patient Registry born 2010-2018 with age at first CF event (first sweat test, clinic visit or hospitalization) by age 365 days. We assessed age of center-reported diagnosis, age at first CF event, demographics and outcomes for three consecutive 3-year cohorts born in 2010-2012, 2013-2015, and 2016-2018. Results: In 6354 infants, median age at diagnosis was earlier than median age at first CF event, which decreased from 1st cohort to 3rd cohort. Weight-for-age (WFA) was < 10th percentile in about 40% of infants at the first CF Center visit. Median WFA z-score at 1-2 years was > 0 but height-for-age (HFA) z-score was < 0 through age 5-6 years. The second cohort had a higher HFA z-score than the first cohort at age 5-6 years. Pseudomonas aeruginosa infection rates decreased over time. About 1/3 of infants were hospitalized in the first year of life across cohorts. Conclusion: Over 9 years of CF NBS, median age at first CF event decreased. CF NBS had positive health impacts but improving nutritional deficits and reducing infant hospitalizations remain targets for improvement.

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.

Pulmonary physiology is a core element of pediatric pulmonology care and research. This article reviews some of the notable publications in physiology that were published in Pediatric Pulmonology in 2020.

Wheezing is a common outcome of preterm birth. This article will review the mechanisms, epidemiology, and treatment of wheezing in preterm children with and without a history of bronchopulmonary dysplasia

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.

Background: Diagnostic sweat testing is required for infants with positive newborn screening (NBS) tests for cystic fibrosis (CF). Infants have “quantity not sufficient” (QNS) sweat volumes more often than older children. A comprehensive study of QNS sweat volumes in infants has not previously been reported. Methods: We surveyed US CF Centers to obtain QNS rates in all infants who had sweat testing at < 14 days and < 3 months of age. We then calculated QNS rates reported to the Cystic Fibrosis Foundation Patient Registry (CFFPR) 2010-2018 in 10-day increments from 1 to 60 days of life. We compared QNS sweat tests rates in preterm (< 37 weeks gestational age) versus term infants. We assessed age at sweat test and proportion of infants who did not have a sweat test reported by 60 days of age. Results: Thirty-nine of 144 (27%) of CF Centers reported a mean QNS rate of 10.5 % (range, 0-100) in infants < 14 days old. CFFPR data showed highest QNS rates in the youngest infants and in those born < 37 weeks gestation. The median age at sweat testing decreased over time, but > 22% of infants did not have a sweat test reported by 60 days. Conclusion: Higher QNS rates are seen in the youngest infants with CF, but > 80% of infants < 2 weeks of age have adequate sweat volumes. Sweat testing should not be delayed in infants with a positive CF NBS test.