Journal of Pediatrics最新文献

This multi-institutional, descriptive study of 19 children with neurofibromatosis 1 examines the link between optic pathway gliomas (OPGs) and central precocious puberty (CPP). We report that CPP can arise without OPG chiasmal involvement and that prior OPG chemotherapy does not prevent the development of CPP.

Objective: To determine the accuracy of the Parent's Observation of Social Interactions (POSI) when deployed for universal autism screening within a large healthcare network.

Study design: Retrospective analysis of electronic health record (EHR) data from children screened for autism spectrum disorder (ASD) using POSI at 18- and 24- month pediatric well child care (WCC) visits across Southern California Permanente Medical Group facilities throughout the 2022 calendar year. Data on ASD diagnoses placed in the EHR problem list were analyzed one year later (until end of 2023) to calculate sensitivity, specificity, and positive/negative likelihood ratio (LR+ and LR-) values.

Results: At 18-month WCC, 8014 (26.4%) out of 30,375 children had elevated POSI scores of >3 (positive screen), and the sensitivity and specificity were 77.9% and 76.1% respectively. At 24-month WCC, 5988 children (21.4%) out of 27,975 had positive POSI screens, and the sensitivity and specificity were 76.5% and 81.4% respectively. Likelihood ratio for positive screen (LR+) was 3.3 at 18 months and 4.2 at 24 months. Likelihood ratio for negative screen (LR-) was 0.29 at both 18 and 24-month time points.

Conclusion: The POSI screen for ASD had adequate sensitivity and specificity (both >76%) when systematically deployed in a primary care setting at 18- and 24-month WCC visits. Children diagnosed with ASD were 3.3 and 4.2 times more likely to have screened positive at 18 and 24 months, compared with children who were not diagnosed with ASD. Conversely, children not diagnosed with ASD were 3.4 times more likely to have screened negative than children with ASD at 18 and 24 months.

Objectives: To examine pediatrician diagnostic skill development of dermatology image-based cases via a web-based tool and to determine case-level variables that were associated with diagnostic error.

Study design: This was a multi-center, prospective, cross-sectional study. A convenience sample of pediatric trainees and attendings were eligible for participation. Using a web-based tool, physicians practiced 334 pediatric dermatology image-based cases until they achieved a performance standard. Participants identified whether the case was concerning, the morphologic category, and the specific diagnosis. After every case, participants received corrective feedback and their progress towards the performance standard.

Results: Among 185 participants, there was a significant improvement in diagnostic performance in classifying concerning vs. non-concerning (+19.2% [95% CI 17.7, 20.6]), morphologic category (+17.9% [95% CI 16.5, 19.3]), and specific diagnosis (+25.2% [95% CI 23.4, 26.7]). The median number of cases required to achieve the performance standard was 142 (IQR 96, 209; min 58, max 330), with a median time to achievement of 57.3 minutes (IQR 38.7, 84.3). Based on 38,502 case interpretations, children with darker versus lighter skin color had a lower odds of correct identification of "concerning" (OR=0.87; 95% CI 0.83, 0.93), morphologic category (OR=0.91; 95% CI 0.85, 0.97), and specific disease (OR=0.96; 95% CI 0.90; 0.99). Fewer than 60% of participants correctly identified bullous variations of diseases, psoriasis, herpes infections, and non-specific viral infections.

Conclusions: The deliberate practice of dermatologic presentations in the context of an education intervention significantly and efficiently improved pediatrician diagnostic ability. The specific diagnostic challenges identified also provide opportunity for targeted learning opportunities in these areas.

Objective: To use a pre-COVID-19 birth cohort database to describe the clinical heterogeneity of severe acute respiratory infections (SARI) cases of the past seasons as a basis to investigate further distribution of clinical phenotypes in the era of immunization for respiratory syncytial virus (RSV).

Study design: Infants with RSV-SARI were identified from a 2014 through 2019 birth cohort in a tertiary care center in Lyon, France, and their medical records extensively reviewed. Using the criteria of the World Health Organization severity definitions, we classified SARI as having nutritional (inability to feed), respiratory (SpO₂<93%), or neurological (failure to respond, apneas) impairments.

Results: We described three phenotypes: nutritional impairment, respiratory impairment, and neurological (with respiratory and nutritional) impairment. Respiratory impairment could overlap with nutritional impairment, or not. There was a significant difference in age categories distribution regarding nutritional impairment (greater proportion in 1.5- to 3-month-old, p=0.01) and neurological impairment (greater proportion in <1.5-month-old, p=0.002).

Conclusions: The present study highlights the presence of three potentially overlapping phenotypes as a new way to consider and describe RSV disease. In order to adapt care and healthcare policies in the RSV-immunization era, it is necessary to investigate further and monitor the residual SARI burden of each phenotype.

Objectiv: To evaluate the associations between complex hip surgery and subsequent hospitalizations in children with intellectual disability, including a subset of children with cerebral palsy.

Study design: We: conducted a retrospective cohort study using linked administrative, health, and disability data from Western Australia. Children born between 1983 and 2009 who underwent complex hip surgery by end 2014 were included (intellectual disability, n=154; subset with cerebral palsy, n=91). A self-controlled case series analysis using Poisson regression was used to estimate the age-adjusted associations of complex hip surgery on all-cause hospitalizations and when the principal diagnosis was lower respiratory tract infection (LRTI) or epilepsy, for periods following the individual's first major hip surgery, compared with the year before surgery.

Results: Age adjusted incidence of all-cause hospitalizations decreased after surgery (year 1: incidence rate ratio [IRR] 0.87 [95% CI, 0.74-1.02]; year 6: IRR 0.57 [95% CI, 0.46-0.72]). The incidence of hospitalizations for LRTI increased (year 1: IRR,1.03 [95% CI, 0.72-1.51]; year 6: IRR 2.08 [95% CI, 1.18-3.68]). The incidence of hospitalizations for epilepsy decreased (year 1: IRR 0.93 [95% CI, 0.57, 1.54]; year>6: IRR 0.72 [95% CI, 0.34-1.55]) after surgery. A similar pattern was observed for the subset of children with or without cerebral palsy.

Conclusion: Complex hip surgeries are associated with fewer hospitalizations overall but not respiratory hospitalizations for children with intellectual disability. Fewer hospitalizations suggest benefits for better musculoskeletal alignment.

Objective: To assess how medical complexity and neighborhood opportunity jointly affect cognitive, motor, and language Bayley's Scales of Infant Development. Secondary objectives involved identifying the factors contributing to developmental disparities across diverse racial and ethnic groups.

Study design: Electronic health records from a Southern California high-risk infant follow-up clinic were analyzed for 440 infants from 2014 through 2023 who had either had neonatal intensive care unit stays, prematurity, very low birthweight, or developmental delay risk. Medical complexity was categorized using the Pediatric Medical Complexity Algorithm (PMCA) into complex chronic (CC), noncomplex chronic (NCC), or non-chronic (NC). Neighborhood opportunity was assessed using the Child Opportunity Index 2.0 (COI). Developmental progress was tracked from ages 4 to 35.6 months.

Results: Of the cohort, 56% were male, and 67% were born prematurely, with 143 NC, 115 NCC, and 182 CC cases. Developmental scores showed a progressive decline with increased medical complexity. CC infants had lower cognitive (β= -15.20, p<0.001, 95% CI: -18.75, -11.7), motor (β= -20.50, p<0.001, 95% CI: -24.25, -16.8), and language scores (β=-11.88, p<0.001, 95% CI = -15.13 to -8.6) compared with NC infants. Lower COI was linked with decreased language scores (β= -0.07, p = 0.005, 95% CI: 0.01, 0.12) but not cognitive or motor scores.

Conclusions: In high-risk infants, the adverse effects of medical complexities on developmental outcomes exceeded those of prematurity and additionally varied according to child neighborhood opportunity.

Objective: To determine if chorioamnionitis is associated with an increased risk of adverse 2-year outcomes among infants with hypoxic-ischemic encephalopathy (HIE).

Study design: This cohort study included all infants with moderate to severe HIE treated with therapeutic hypothermia and enrolled on the High-dose Erythropoietin for Asphyxia and Encephalopathy Trial. Clinical chorioamnionitis (CC) was defined as a diagnosis made by a treating obstetrician and histologic chorioamnionitis (HC) was defined as placental inflammation observed on histology. We used proportional odds regression to determine the associations between CC, HC, and an ordinal 2-year neurodevelopmental outcome measure: no neurodevelopmental impairment (NDI), mild NDI, moderate NDI, severe NDI, or death.

Results: Of 500 infants, 65 (13%) were exposed to CC. Of 317 infants with placental data available, 125 (39%) were exposed to HC. Infants exposed to CC (odds ratio 0.57, 95% CI 0.34-0.95) and those exposed to HC (odds ratio 0.62, 95% CI 0.40-0.96) had a lower severity of primary outcome than unexposed infants. Infants exposed to chorioamnionitis also had lower frequencies of sentinel events (CC: P = .001; HC: P = .005), central pattern magnetic resonance imaging brain injury (CC: P = .02; HC: P = .02), and electroencephalogram background abnormalities (CC: P = .046; HC: P = .02), compared with unexposed infants.

Conclusions: Infants with HIE who were exposed to chorioamnionitis had lower severity of 2-year outcomes than unexposed infants. Our findings suggest that chorioamnionitis may lead to a lower severity of brain dysfunction than other pathophysiologic mechanisms of encephalopathy.