Using an Innovative Model to Improve Performance of the Infant Hip Examination.

OBJECTIVES: Determine if a new teaching bundle targeting developmental dysplasia of the hip screening improved interns' examination skills across multiple pediatric residency programs. METHODS: This multicenter prospective cohort study included interns across 6 pediatric residency sites within the Academic Pediatric Association's Better Outcomes through Research for Newborns Network in 2022. Participants underwent a baseline hip examination assessment on models using a checklist derived from textbook descriptions of Galeazzi, Ortolani, and Barlow maneuvers before receiving a teaching bundle. Repeat testing occurred after instruction. Data were analyzed using t-test for continuous and χ2 test for categorical variables. Semistructured focus groups provided qualitative feedback regarding the teaching bundle. RESULTS: We enrolled 117 of 155 interns across 6 sites (76%) for participation in the teaching bundle. Only 2% of participants (n = 2) identified a positive Galeazzi sign at baseline, whereas 88% (n = 103, P < .001) did so on the postinstructional assessment. Although 27% of participants (n = 32) correctly identified a positive Barlow sign at baseline, 69% (n = 81, P < .001) did so on the postinstructional assessment. The ability to correctly detect a positive Ortolani sign increased from 22% (n = 26) to 92% (n = 108, P < .001). Fifteen interns participated in the semistructured focus groups, with resultant themes reinforcing the limited experience of the infant hip examination before this intervention and the positive impact of the teaching bundle. CONCLUSIONS: Most participants in this study did not have strong infant hip examination skills at entry into residency. A standardized teaching bundle significantly improved skills in examination technique and identifying abnormalities.

Congenital lymphatic dysplasia and severe bone disease in a term neonate with a novel homozygous PIEZO1 variant.

We report a patient with nonimmune fetal hydrops and multiple pathologic fractures. RNA analysis revealed a novel PIEZO1 variant. This report is the first to elucidate PIEZO1's role as a critical regulator of bone mass and strength.

Military Family Perspectives on Enrollment and Engagement in the WIC Program.

INTRODUCTION: One in four U.S. service members endorses food insecurity. The Supplemental Nutrition Program for Women, Infants, and Children (WIC) is an invaluable, underutilized resource that can increase access to nutritious food for families with children under 5 years of age. Our research sought to evaluate military family perceptions and engagement with the WIC program. MATERIALS AND METHODS: We conducted a mixed-method study, recruiting from a convenience sample of military families who applied for financial assistance for child care. An 18-question survey and follow-up focus groups assessed participant demographics and experiences with WIC. We utilized descriptive statistics ordinal logistic regression analyses for quantitative data analysis. For qualitative data, descriptive content analysis with constant comparison and inductive and deductive coding of interviews identified emerging themes. This study was approved by the Uniformed Services University of the Health Sciences Institutional Review Board. RESULTS: Among the 399 survey respondents, 25% were currently enrolled in WIC and 39% had been previously enrolled. Service members and their partners learned about WIC from a variety of sources, and there was no significant association between the branch of service or rank and WIC enrollment. There were 40 total participants in 10 completed focus groups. Six themes emerged: (1) Military-specific factors create unique circumstances related to WIC engagement; (2) the WIC program facilitates access to formula supplementation and nutritious foods for military families; (3) lack of program awareness and misinformation are top barriers to WIC engagement in military families; (4) stigma impacts WIC program enrollment and engagement; (5) logistics of enrollment can impact WIC engagement among military families; and (6) the military can support WIC enrollment and engagement through standardization, education, and leadership commitment. CONCLUSIONS: Our findings suggest that unique circumstances related to military family life create a profound need for programs addressing food support, such as WIC. Interventions to improve WIC enrollment among military families need to be rooted in broad outreach efforts, not targeted at specific ranks, branches, or ages. Specific recommendations include increasing information dissemination, universally screening military families for WIC, decreasing logistical burdens, and involving military leadership.

"Hole" Exome Sequences: The Importance of Phenotyping to Fill the Gaps in Whole Exome Sequencing.

BACKGROUND: Whole exome sequencing (WES) is commonly used for patients with nonspecific clinical features and conditions with genetic heterogeneity. However, a nondiagnostic exome does not exclude a genetic diagnosis, so history and physical examination is crucial to selecting appropriate genetic testing. CASES: We report three patients with three recognizable phenotypes: a seven-year-old female with classic Rett syndrome; a 28-year-old male with neuropathy, ataxia, and retinitis pigmentosa; and a 16-year-old male with mosaic, segmental, paternal uniparental disomy 14 who had nondiagnostic WES. CONCLUSIONS: Despite recognizable phenotypes they had diagnostic delays due to incorrect selection of genetic testing. This case series highlights the limitations of WES and reinforces the importance of utilizing patient history and physical examination to select initial testing. We will discuss appropriate testing for these patients and a consistent diagnostic algorithm that can be applied when approaching patients with unknown or uncertain clinical presentations.

Massive fetomaternal haemorrhage warranting novel use of tandem isovolumetric partial exchange transfusion and therapeutic hypothermia.

A newborn male infant was pale, hypotonic, and had respiratory distress after delivery. Venous cord blood gas revealed a severe metabolic acidosis. His initial examination was consistent with moderate encephalopathy and laboratory testing uncovered severe congenital anaemia (haematocrit 0.127 L/L). He met the clinical criteria for therapeutic hypothermia (TH) and required red blood cell transfusions, but due to the severity of his anaemia, an exchange transfusion was favoured to prevent transfusion-associated circulatory overload. There are no previous reports of these procedures completed in tandem, but the benefits were perceived to outweigh the risks. During the 72 hours of TH, the infant received an isovolumetric partial exchange transfusion and tolerated both treatments without any adverse clinical events.Kleihauer-Betke testing detected a massive chronic fetomaternal haemorrhage with 475 mL (164 mL/kg) of blood. A brain MRI completed prior to discharge was normal. At 6 months of age, he is growing and developing normally.

Evaluating WIC Engagement and Food Insecurity Among Active Duty Military Families.

INTRODUCTION: Nearly a quarter of active duty service members identified as food insecure in a 2022 Department of Defense report. Food insecurity impacts military readiness, retention, and recruitment. The Supplemental Nutrition Program for Women, Infants, and Children (WIC) is a federal food supplementation program that can mitigate food insecurity for service members with children less than 5 years of age. To date, there is a lack of standardized screening for WIC eligibility or enrollment for service members and their families. This project sought to evaluate WIC awareness and enrollment as well as the prevalence of food insecurity at Walter Reed National Military Medical Center. MATERIALS AND METHODS: A 26-question survey was developed to assess WIC awareness, source of WIC information, food insecurity, and nutritional insecurity. Our team developed and utilized a novel WIC screening algorithm to rapidly screen families for WIC eligibility. These tools were administered to families presenting for care at the Walter Reed National Military Medical Center pediatrics and obstetric outpatient clinics during the month of July 2022. This study was approved by the institutional review board at Walter Reed. RESULTS: A total of 108 (25%) of the 432 surveyed participants were eligible for WIC, with odds of WIC eligibility increasing for lower-ranking and younger service members. Of the 432 participants, 354 (81.9%) were aware of WIC. Enlisted service members were more likely than officers to know about WIC (P = 0.03), and of the 354 participants aware of WIC, a higher proportion of enlisted rank respondents learned about WIC from a military source (P = 0.01). Among the 108 participants eligible for WIC, only 38 (35.2%) reported being enrolled in WIC. Among WIC-eligible respondents who knew about WIC, being enrolled in the WIC program was not associated with rank, branch of service, sponsor gender, or sponsor age. CONCLUSIONS: Despite proven efficacy, WIC remains an underutilized resource for eligible military families. Our results show that a standardized screening approach at Walter Reed National Military Medical Center increased identification of WIC-eligible active duty service members by 180%, with approximately $150,000 a year in increased food supplementation benefits. Military healthcare and readiness leaders should embrace efforts to increase knowledge of, referral to, and enrollment in WIC to increase family health, well-being, and military family readiness.

Predicting the RSV Surge: Pediatric RSV Patterns of the COVID Pandemic.

Historically, respiratory syncytial virus (RSV) infection trends have been predictable. The COVID-19 pandemic and associated precautions impacted RSV disease patterns. RSV infection trends during the first year of the COVID-19 pandemic may have predicted the 2022 surge in pediatric RSV infections. A continued emphasis on increased viral testing will allow for early recognition and preparation for future public health crises.

Potential Neurodevelopmental Effects of Pediatric Intensive Care Sedation and Analgesia: Repetitive Benzodiazepine and Opioid Exposure Alters Expression of Glial and Synaptic Proteins in Juvenile Rats.

Sedatives are suspected contributors to neurologic dysfunction in PICU patients, to whom they are administered during sensitive neurodevelopment. Relevant preclinical modeling has largely used comparatively brief anesthesia in infant age-approximate animals, with insufficient study of repetitive combined drug administration during childhood. We hypothesized that childhood neurodevelopment is selectively vulnerable to repeated treatment with benzodiazepine and opioid. We report a preclinical model of combined midazolam and morphine in early childhood age-approximate rats. DESIGN: Animal model. SETTING: Basic science laboratory. SUBJECTS: Male and female Long-Evans rats. INTERVENTIONS: Injections of morphine + midazolam were administered twice daily from postnatal days 18-22, tapering on postnatal days 23 and 24. Control groups included saline, morphine, or midazolam. To screen for acute neurodevelopmental effects, brain homogenates were analyzed by western blot for synaptophysin, drebrin, glial fibrillary acidic protein, S100 calcium-binding protein B, ionized calcium-binding adaptor molecule 1, and myelin basic proteins. Data analysis used Kruskal-Wallis with Dunn posttest, with a p value of less than 0.05 significance. MEASUREMENTS AND MAIN RESULTS: Morphine + midazolam and morphine animals gained less weight than saline or midazolam (p ≤ 0.01). Compared with saline, morphine + midazolam expressed significantly higher drebrin levels (p = 0.01), with numerically but not statistically decreased glial fibrillary acidic protein. Similarly, morphine animals exhibited less glial fibrillary acidic protein and more S100 calcium-binding protein B and synaptophysin. Midazolam animals expressed significantly more S100 calcium-binding protein B (p < 0.001) and 17-18.5 kDa myelin basic protein splicing isoform (p = 0.01), with numerically increased synaptophysin, ionized calcium-binding adaptor molecule 1, and 21.5 kDa myelin basic protein, and decreased glial fibrillary acidic protein. CONCLUSIONS: Analysis of brain tissue in this novel rodent model of repetitive morphine and midazolam administration showed effects on synaptic, astrocytic, microglial, and myelin proteins. These findings warrant further investigation because they may have implications for critically ill children requiring sedation and analgesia.