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Bronchopulmonary dysplasia (BPD) is the most common serious morbidity of preterm birth. Short-term respiratory outcomes for infants with the most severe forms of BPD are highly variable. The mechanisms that explain this variability remain unknown and may be mediated by racial disparities.To determine the association of maternal race with death and length of hospital stay in a multicenter cohort of infants with severe BPD.This multicenter cohort study included preterm infants enrolled in the BPD Collaborative registry from January 1, 2015, to July 19, 2021, involving 8 BPD Collaborative centers located in the US. Included patients were born at less than 32 weeks' gestation, had a diagnosis of severe BPD as defined by the 2001 National Institutes of Health Consensus Criteria, and were born to Black or White mothers.Maternal race: Black vs White.Death and length of hospital stay.Among 834 registry infants (median [IQR] gestational age, 25 [24-27] weeks; 492 male infants [59%]) meeting inclusion criteria, the majority were born to White mothers (558 [67%]). Death was observed infrequently in the study cohort (32 [4%]), but Black maternal race was associated with an increased odds of death (adjusted odds ratio, 2.1; 95% CI, 1.2-3.5) after adjusting for center. Black maternal race was also significantly associated with length of hospital stay (adjusted between-group difference, 10 days; 95% CI, 3-17 days).In a multicenter severe BPD cohort, study results suggest that infants born to Black mothers had increased likelihood of death and increased length of hospital stay compared with infants born to White mothers. Prospective studies are needed to define the sociodemographic mechanisms underlying disparate health outcomes for Black infants with severe BPD.

Objective Our objective was to test the hypothesis that in‐hospital respiratory viral infections (RVI) would be significantly lower in a cohort of patients with established bronchopulmonary dysplasia (BPD) exposed to a severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection prevention protocol when compared to historical controls. Study Design On April 1, 2020, we implemented a universal infection prevention protocol to minimize the risk of nosocomial SARS‐CoV‐2 transmission in a dedicated BPD intensive care unit. We performed a retrospective cohort study and included patients with established BPD, as defined by the 2019 Neonatal Research Network criteria, admitted to our center who underwent real‐time polymerase‐chain‐reaction RVI testing between January 1, 2015 and March 31, 2021. We excluded patients readmitted from home. We compared the proportion of positive tests to the number of tests performed and the distribution of viral respiratory pathogens in the pre‐ and post‐SARS‐CoV‐2 eras. Results Among 176 patients included in the study, 663 RVI tests were performed and 172 (26%) tests were positive. The median number of tests performed, measured in tests per patient per month, in the SARS‐CoV‐2 era was not significantly different compared to the pre‐SARS‐CoV‐2 era (0.45 vs. 0.34 tests per patient per month, p = .07). The proportion of positive RVI tests was significantly lower in the SARS‐CoV‐2 era when compared to the pre‐SARS‐CoV‐2 era (0.06 vs. 0.30, p

To determine the in-hospital outcomes for patients with established bronchopulmonary dysplasia (BPD) referred late for severe disease. Retrospective cohort study of patients with established BPD referred to our center after 36 weeks PMA. Among 71 patients with BPD referred to our center after 36 weeks PMA between 2010 and 2018, the median PMA was 47 weeks (IQR, 42, 53) and the median respiratory severity score was 8.1 (IQR 4.5, 11.0) on admission. Survival in this cohort was 92%. Most survivors were discharged home without the need for positive pressure respiratory support (77%) or pulmonary vasodilators (89%). For survivors, we observed a significant improvement in median z-scores for length (−6.7 vs −3.3, p

To describe characteristics, outcomes, and risk factors for death or tracheostomy with home mechanical ventilation in full-term infants with chronic lung disease (CLD) admitted to regional neonatal intensive care units.This was a multicenter, retrospective cohort study of infants born ≥37 weeks of gestation in the Children's Hospitals Neonatal Consortium.Out of 67,367 full-term infants admitted in 2010-2016, 4886 (7%) had CLD based on receiving respiratory support at either 28 days of life or discharge. 3286 (67%) were still hospitalized at 28 days receiving respiratory support, with higher mortality risk than those without CLD (10% vs. 2%, p 0.001). A higher proportion received tracheostomy (13% vs. 0.3% vs. 0.4%, p 0.001) and gastrostomy (30% vs. 1.7% vs. 3.7%, p 0.001) compared to infants with CLD discharged home before 28 days and infants without CLD, respectively. The diagnoses and surgical procedures differed significantly between the two CLD subgroups. Small for gestational age, congenital pulmonary, airway, and cardiac anomalies and bloodstream infections were more common among infants with CLD who died or required tracheostomy with home ventilation (p 0.001). Invasive ventilation at 28 days was independently associated with death or tracheostomy and home mechanical ventilation (odds ratio 7.6, 95% confidence interval 5.9-9.6, p 0.0001).Full-term infants with CLD are at increased risk for morbidity and mortality. We propose a severity-based classification for CLD in full-term infants. Future work to validate this classification and its association with early childhood outcomes is necessary.

The Small Baby Program at Nationwide Children's Hospital was launched in 2004 in response to a need for better care for infants born extremely preterm. Standardization of care, decreased variability, multidisciplinary support, and robust research and quality improvement have allowed us to greatly improve our outcomes. In addition to the numerous medical and technological advances during this time, a strong commitment to kangaroo care and family-centered care have been integral to the growth and success of our program. The following review of the program aims to highlight the above areas while detailing the specific processes that have contributed to its ongoing success. Key areas of focus have been on respiratory management, neurodevelopmental care, and nutritional optimization. The implementation and continued refinement of the Small Baby Program has allowed us to improve the survival of extremely preterm infants, decrease certain morbidities, and improve long-term neurodevelopmental outcomes.

Objective Our objective was to test the hypothesis that in-hospital respiratory viral infections (RVI) would be significantly lower in a cohort of patients with established bronchopulmonary dysplasia in the SARS-CoV-2 era when compared to historical controls. Study Design On April 1, 2020, we implemented a universal infection prevention bundle to minimize the risk of nosocomial SARS-CoV-2 transmission in a dedicated BPD intensive care unit. We performed a retrospective cohort study and included patients with established BPD, as defined by the 2019 Neonatal Research Network criteria, admitted to our center who underwent real-time polymerase-chain-reaction RVI testing between January 1, 2015 and March 31, 2021. We excluded patients re-admitted from home. We compared to number of tests performed, the proportion of positive tests, and the distribution of viral respiratory pathogens in the pre- and post-SARS-CoV-2 eras. Results Among 176 patients included in the sudy, 663 RVI tests were performed and 172 (26%) tests were positive. The median number of tests performed, measured in tests per patient per month, in the SARS-CoV-2 era was not significantly different compared to the pre-SARS-CoV-2 era (0.45 vs 0.34 tests per patient per month, P = 0.07). The proportion of positive RVI tests was significantly lower in the SARS-CoV-2 era when compared to the pre-SARS-CoV-2 era (0.06 vs 0.30, P

To test the hypothesis that elevated respiratory severity indices will identify patients with severe bronchopulmonary dysplasia (BPD) at the greatest risk for adverse in-hospital outcomes.This was a retrospective cohort study. A modified respiratory severity score (mean airway pressure × fraction of inspired oxygen) and a modified pulmonary score (respiratory support score × fraction of inspired oxygen + sum of medication scores) were calculated in a consecutive cohort of patients ≥36 weeks of postmenstrual age with severe BPD admitted to a referral center between 2010 and 2018. The association between each score and the primary composite outcome of death/prolonged length of stay (75th percentile for cohort) was assessed using area under the receiver operator characteristic curve (AUROC) analysis and logistic regression. Death and the composite outcome death/tracheostomy were analyzed as secondary outcomes.In 303 patients, elevated scores were significantly associated with increased adjusted odds of death/prolonged length of stay: aOR 1.5 (95% CI 1.3-1.7) for the modified respiratory severity score and aOR 11.5 (95% CI 5.5-24.1) for the modified pulmonary score. The modified pulmonary score had slightly better discrimination of death/prolonged length of stay when compared with the modified respiratory severity score, AUROC 0.90 (95% CI 0.85-0.94) vs 0.88 (95% CI 0.84-0.93), P = .03. AUROCs for death and death/tracheostomy did not differ significantly when comparing the modified respiratory severity score with the modified pulmonary score.In our referral center, the modified respiratory severity score or the modified pulmonary score identified patients with established severe BPD at the greatest risk for death/prolonged length of stay, death, and death/tracheostomy.

To determine the in-hospital outcomes for patients with established bronchopulmonary dysplasia (BPD) referred late for severe disease.Retrospective cohort study of patients with established BPD referred to our center after 36 weeks PMA.Among 71 patients with BPD referred to our center after 36 weeks PMA between 2010 and 2018, the median PMA was 47 weeks (IQR, 42, 53) and the median respiratory severity score was 8.1 (IQR 4.5, 11.0) on admission. Survival in this cohort was 92%. Most survivors were discharged home without the need for positive pressure respiratory support (77%) or pulmonary vasodilators (89%). For survivors, we observed a significant improvement in median z-scores for length (-6.7 vs -3.3, p 0.0001) between admission and discharge.Despite presenting relatively late with a high degree of illness severity, nearly all patients in this cohort survived to hospital discharge with improvement in comorbidities.

We performed a point prevalence study on infants with severe BPD collecting data on type and settings of ventilatory support; 187 infants were included from 15 centers, 51% who were on invasive positive pressure ventilation. We found significant center-specific variation in ventilator modes.

Introduction Faculty evaluations are important tools for improving faculty-to-resident instruction, but residents in our pediatric and internal medicine/pediatric residency programs would seldom evaluate individual pediatric faculty hospitalists. Our objectives were to: (1) increase the percentage of completed evaluations of individual pediatric hospitalists to greater than 85%, (2) improve the quality of pediatric hospitalist feedback as measured by resident and faculty satisfaction surveys, and (3) to reduce the resident concern of lack of anonymity of evaluations. Methods Members of the resident inpatient team (pediatric and internal medicine/pediatric residents) completed group-based evaluations of individual pediatric hospitalists. A survey to evaluate this change in process was distributed to the pediatric hospitalists (n = 6) and another survey was distributed to residents, both based on a 5-point Likert-type scale. Surveys were completed before and four months after implementation of the changes. Pre- and post-survey data of resident and hospitalist responses were compared using the Mann-Whitney test and probability proportion test. Results The percent of completed evaluations increased from 0% to 86% in one month and to 100% in two months. Thereafter, the percent of completed evaluations remained at 100% through the end of the data collection period at seven months. Hospitalists reported (n = 6, 100% participation) their satisfaction regarding the feedback they received from residents significantly increased for all survey questions. Resident satisfaction (n = 24, 89% participation in postintervention surveys) increased significantly with regards to the evaluation process. Conclusions For hospitalists, group-based resident evaluations of individual hospitalists led to an increased percentage of completed evaluations, improved the quality and quantity of feedback to hospitalists, and increased satisfaction with evaluations. For residents, these changes led to increased satisfaction with the evaluation process.

Rare variants in the T-box transcription factor 4 gene (

Objective: To identify the number of cumulative medication exposures and most frequently used medications in infants with severe BPD. Study Design: We performed a retrospective cohort study in infants with severe BPD admitted to United States children’s hospitals. We measured cumulative medication exposures in individual subjects and between-center variation after adjustment for infant characteristics. We then identified the specific medications and therapeutic classes with the highest rates of use. Results: In 3252 subjects across 43 hospitals, we identified a median (interquartile range) of 30 (17– 45) cumulative medication exposures per infant. The adjusted mean number of medication exposures varied between centers (p < 0.0001), with a range of 22 – 50. Diuretics and furosemide were the most frequently prescribed therapeutic class and specific medication for the management of severe BPD. Conclusions: Infants with severe BPD are exposed to alarming number of medications of unclear efficacy and safety, with marked variation between center.

Rare variants in the T-box transcription factor 4 gene (TBX4) have recently been recognised as an emerging cause of paediatric pulmonary hypertension (PH). Their pathophysiology and contribution to persistent pulmonary hypertension in neonates (PPHN) are unknown. We sought to define the spectrum of clinical manifestations and histopathology associated with TBX4 variants in neonates and children with PH.We assessed clinical data and lung tissue in 19 children with PH, including PPHN, carrying TBX4 rare variants identified by next-generation sequencing and copy number variation arrays.Variants included six 17q23 deletions encompassing the entire TBX4 locus and neighbouring genes, and 12 likely damaging mutations. 10 infants presented with neonatal hypoxic respiratory failure and PPHN, and were subsequently discharged home. PH was diagnosed later in infancy or childhood. Three children died and two required lung transplantation. Associated anomalies included patent ductus arteriosus, septal defects, foot anomalies and developmental disability, the latter with a higher prevalence in deletion carriers. Histology in seven infants showed abnormal distal lung development and pulmonary hypertensive remodelling.TBX4 mutations and 17q23 deletions underlie a new form of developmental lung disease manifesting with severe, often biphasic PH at birth and/or later in infancy and childhood, often associated with skeletal anomalies, cardiac defects, neurodevelopmental disability and other anomalies.