Maternal Hypertension and Early-Onset Neonatal Neutropenia.

Objective: Maternal hypertension is considered a risk factor for early neonatal neutropenia. We sought to explore this relationship. Study Design: This retrospective cohort study compared initial neutrophil counts in infants born to mothers with preeclampsia with severe features (PSF) and infants born to normotensive mothers using Negative Binomial Regression (NBR) and logistic regression models. Results: Maternal hypertension negatively affected the early neonatal neutrophil count (adjusted NRB coefficient 0.4 [0.2, 0.6], p < 0.0001) but did not increase the risk of neutropenia (OR 2.07 [0.97, 4.41], p = 0.06). The initial neutrophil count and neutropenia risk were not different between PSF subgroups. Gestational age had the greatest impact on neutropenia risk (OR 0.72 [0.64, 0.81], p < 0.0001). Almost all neutropenia resolved within 48 h. Conclusion: Maternal hypertension negatively affects the early neonatal neutrophil count while not increasing the risk of neonatal neutropenia.

Neonatal Intensive Care Unit Boot Camp: A Preparatory Curriculum for Pediatric Residents.

Introduction: Resident training has changed significantly in recent years, resulting in reduced experiences and practice. Because pediatric residents have fewer required intensive care unit (ICU) rotations, we introduced a neonatal ICU (NICU) boot camp (2014-2015) that continues today to prepare residents immediately prior to beginning an NICU rotation. Methods: The NICU boot camp consists of three 1-hour sessions: two interactive lectures with case-based application and one hands-on, integrative learning using simulation. The sessions are designed to cover basic information to assist in daily rounding and decision making while in the NICU. After their NICU rotation, residents complete a 12-item questionnaire. Program evaluation includes direct observation during sessions, faculty debriefing, and a postprogram resident survey. Results: Fifty-seven residents participated; questionnaire responses were available from 46 (80.70%). Combined percentages of very useful and extremely useful responses for the three sessions were 82.61%, 78.26%, and 82.60%, with 86.95% for the overall program; 80.40% agreed that repeating boot camp prior to each NICU rotation would be useful. Analysis of narrative responses revealed that participation in boot camp enhanced residents' readiness and confidence for patient care in the NICU and as stated for each educational objective. Discussion: Program evaluation results support highly effective and sustainable implementation and achievement of educational objectives. Minor refinements continue for enhancing active learning and content materials and for increasing rigor of program evaluation. Results also suggest that our boot camp may benefit other pediatric programs and serve as a model for use in other resident specialty programs.

RAF1 variants causing biventricular hypertrophic cardiomyopathy in two preterm infants: further phenotypic delineation and review of literature.

Noonan syndrome (NS) is an autosomal dominant disorder characterized by distinctive facial features, short neck, short stature, congenital heart defects, pectus deformities, and variable developmental delays. NS is genetically heterogeneous as pathogenic variants in several genes involved in the Ras/mitogen-activated protein kinase pathway have been associated with a NS phenotype. Overall, 50% of patients harbor pathogenic variants in PTPN11, whereas 3-17% of patients have variants in RAF1. We present two premature neonates with progressive biventricular hypertrophy found to have RAF1 variants in the CR2 domain. Molecular testing in patient 1 revealed a missense variant of a highly conserved residue c.782 C>G (p.P261R). This variant has been reported once with fatal outcome. Patient 2 also had a missense variant in a highly conserved neighboring residue c.770 C>T (p.S257L). This variant has been previously reported, most recently associated with the development of pulmonary arterial hypertension. Both our patients had prenatal findings of polyhydramnios, short long bones, hydrops fetalis, and cardiac anomalies with progressive biventricular hypertrophic cardiomyopathy. Both patients had a lethal outcome. Our findings further support the pathogenicity and lethality of p.P261R, and the need to monitor for pulmonary arterial hypertension in p.S257L. In addition, the second patient was presented with progressive hydrocephalus due to aqueductal stenosis. This could be related to the NS phenotype. More cases with this association are needed to confirm this finding.

Enhancing residents' neonatal resuscitation competency through unannounced simulation-based training.

BACKGROUND: Almost half of pediatric third-year residents surveyed in 2000 had never led a resuscitation event. With increasing restrictions on residency work hours and a decline in patient volume in some hospitals, there is potential for fewer opportunities. PURPOSE: Our primary purpose was to test the hypothesis that an unannounced mock resuscitation in a high-fidelity in-situ simulation training program would improve both residents' self-confidence and observed performance of adopted best practices in neonatal resuscitation. METHODS: Each pediatric and medicine-pediatric resident in one pediatric residency program responded to an unannounced scenario that required resuscitation of the high fidelity infant simulator. Structured debriefing followed in the same setting, and a second cycle of scenario response and debriefing occurred before ending the 1-hour training experience. Measures included pre- and post-program confidence questionnaires and trained observer assessments of live and videotaped performances. RESULTS: Statistically significant pre-post gains for self-confidence were observed for 8 of the 14 NRP critical behaviors (p=0.00-0.03) reflecting knowledge, technical, and non-technical (teamwork) skills. The pre-post gain in overall confidence score was statistically significant (p=0.00). With a maximum possible assessment score of 41, the average pre-post gain was 8.28 and statistically significant (p<0.001). Results of the video-based assessments revealed statistically significant performance gains (p<0.0001). Correlation between live and video-based assessments were strong for pre-post training scenario performances (pre: r=0.64, p<0.0001; post: r=0.75, p<0.0001). CONCLUSIONS: Results revealed high receptivity to in-situ, simulation-based training and significant positive gains in confidence and observed competency-related abilities. Results support the potential for other applications in residency and continuing education.