Journal of Pediatrics最新文献

Objective: To understand parents' experiences of ethical challenges in the care of children with chronic and serious medical conditions and what resources they access for support.

Study design: We recruited English-speaking parents of children with complex and serious medical conditions via family advocacy groups to complete an electronic survey from October 2022 through February 2023. We queried respondents' experiences with specific ethical challenges in the care of their child, whether their concerns persisted, and what resources they accessed. Respondents also reported demographics, educational backgrounds, financial resources, and their child's medical needs.

Results: 218 parents completed surveys. Parents reported experiencing each of the 15 presented ethical challenges with varying frequency. More than half of parents reported residual distress in 6/15 ethical challenges. Demographic variables (gender, relationship status, time since medical challenge, and ethnicity) were not associated with level of distress related to ethical challenges. When facing challenges, respondents most commonly communicated with family members, friends, doctors, and other parents of children with medical problems, accessed the internet, and relied on their instincts or prayer/meditation.

Conclusions: Parents of children with medical complexity experience ethical challenges in the care of their children, although covering a broader range of experiences than typically considered by clinicians to have ethical dimensions. Many of these challenges leave residual distress. The resources that families report accessing to navigate these challenges are ones that typically do not have training, background, or specificity to medical ethics.

Objective: To investigate the effects of intrauterine and perinatal exposure to chikungunya virus (CHIKV) on neurodevelopment in infants and toddlers.

Study design: We conducted a cohort study comparing children with intrauterine or perinatal exposure to maternal CHIKV infection with unexposed controls in Rio de Janeiro, Brazil. Neurodevelopment was assessed with General Movement Assessments (GMA) in the first six months of life, and the Bayley-III Scales of Infant and Toddler Development and Modified Checklist for Autism in Toddlers (M-CHAT) for older children. Developmental delay (DD) was defined as a Bayley score less than 70 and risk of DD as a score less than 85.

Results: Among 60 children exposed to intrauterine or perinatal CHIKV, 20 (33 %) had laboratory confirmation of CHIKV infection by RT-PCR or IgM serology and 40 did not; 44 exposed children (15 infected and 29 uninfected) had GMA performed, with 19% having suboptimal or abnormal results. At 11 to 42 months of age, 35 exposed children and 78 unexposed controls had Bayley-III assessments. Compared with controls, exposed children had higher rates of DD (7 [20%] vs. 2 [3%], p=0.004) driven by the language domain, and greater risk of DD driven by motor and cognitive domains scores (10 [29%] vs. 10 [13%], p=0.03 and 8 [23%] vs. 5 [6%], p=0.02, respectively). Eight of 35 (23%), CHIKV exposed children screened positive for autism spectrum disorder. CHIKV-exposed uninfected children had 2 (9.5%) cases of DD and 5 (23.8%) cases of ASD.

Conclusions: Abnormal neurodevelopmental results were seen in both infected and uninfected children with intrauterine or perinatal CHIKV exposure. Infant neurodevelopment monitoring should be considered following exposure to maternal CHIKV infection in pregnancy to facilitate early interventions and to mitigate neurodevelopmental sequelae.

Objective: To identify and characterize how race and ethnicity influence the relationship between autism and weight status, across all categories of weight from underweight to severe obesity.

Study design: We developed a propensity score-matched cross-sectional dataset of children with and without parent-reported autism in the National Survey of Children Health (NSCH, 2016-2022) and Adolescent Brain and Cognition Development Study (ABCD, 2016-2018). We included non-Hispanic Asian, non-Hispanic Black, non-Hispanic White, and Hispanic children aged 6 to 17 years. Prevalence ratios for autistic and non-autistic children were estimated with multinomial regression models across Centers for Disease Control-defined categories for underweight, healthy weight, overweight, mild-to-moderate obesity, and severe obesity, based on parent-reported height and weight (NSCH) and measured heights and weights (ABCD).

Results: Prevalence disparities across racial and ethnic groups were evident and the pattern of prevalence ratios (autistic:non-autistic) showed remarkably consistent U- or J-shaped prevalence ratios. Prevalence ratios were elevated in underweight and severe obesity for autistic Asian, Black, White, and Hispanic children compared with their non-autistic peers of the same race or ethnicity, with the exception of underweight prevalence where autistic and non-autistic Asian children did not differ.

Conclusions: The largely consistent pattern of prevalence ratios comparing autistic and non-autistic children for underweight and severe overweight in the 4 major racial and ethnic groups in the US suggests that health care and other providers should be aware of these risks in autistic children, actively monitor their weight status, and intervene early to prevent excess weight loss or weight gain.

Objective: To describe the clinical course and outcome of 33 patients with Kawasaki disease (KD) treated with cyclosporine (CSA) for coronary artery abnormalities (CAA) or treatment resistance.

Study design: Single-center, retrospective study of patients with KD treated from 2013 through 2023 for CAA or treatment resistance. Demographics, laboratory studies, medications, adverse events, and echocardiographic data were analyzed.

Results: Of the 33 KD patients treated with CSA, 25 patients received CSA for CAA and 8 for treatment resistance. Four patients received CSA intravenously initially, followed by oral therapy. Since 2014, all patients received CSA orally only. The target 2-hour post-dose level (300-600 ng/ml) was best achieved by dividing 5 mg/kg/day every 12 hours. We developed a standardized treatment protocol based on our experiences with this patient population. The patients treated for CAA all showed improved coronary artery Z-scores at both the 2-week and 6-week follow-up. Two patients experienced significant adverse events. One patient had posterior reversible encephalopathy syndrome, while the other developed EBV-associated lymphoproliferative disorder. Discontinuation of CSA led to complete recovery in both cases.

Conclusions: CSA was generally well tolerated in patients with KD and CAA or treatment resistance. Our study contributes to the limited literature on CSA use in KD, providing dosing guidance and advocating for cautious monitoring.

Objective: To assess variability among data elements collected among existing neonatal hypoxic-ischemic encephalopathy (HIE) data registries worldwide and to determine the need for future harmonization of standard common data elements.

Study design: This was a cross-sectional study of data elements collected from current or recently employed HIE registry data forms. Registries were identified by literature search and email inquiries to investigators worldwide. Data elements were categorized by group consensus.

Results: A total of 1,281 data elements were abstracted from 22 registries based in 14 countries, including 3 middle-income countries. Registries had a median of 106.5 distinct data elements per registry (range 59-458). The most commonly collected data were related to pregnancy, therapeutic hypothermia, and short-term hospital outcomes. The least consistently collected data were laboratory values other than acid/base status values. Only 4 variables were consistently collected in every registry. Five registries included neurodevelopmental follow-up fields and 5 others linked their data to a separate follow-up registry.

Conclusion: Many HIE registries are collecting patient data around the world, but there is considerable variability in the number, type, and format of data collected. Future attempts to develop standard common data elements to harmonize data collection globally will be crucial to facilitate worldwide collaboration and to optimize management and outcome of neonatal HIE.

Objectives: To determine whether airway and parenchymal function identifies subgroups of infants born preterm according to the predominant pulmonary pathophysiology, and whether these subgroups have different risks for respiratory disease during infancy.

Study design: We prospectively enrolled a cohort of 125 infants born preterm with planned clinical follow-up after NICU discharge. The study included monthly questionnaires for wheeze and visits to a physician or care provider for any respiratory illness. In addition, infant lung function testing near 5-months corrected-age included measures of airways and parenchymal function using forced expiratory flows, alveolar volume (V_A), and the carbon monoxide transfer constant (D_L/V_A). Phenotypes were defined using two approaches: an a priori defined phenotypes based on FEF₇₅ and D_L/V_A z-scores, and an unbiased approach to classifying infants using k-means clustering. Results We identified four pulmonary physiologic phenotypes that distinguished participants with predominantly decreased airway and/or parenchymal function. Although the worst physiologic phenotypes were associated with a lower gestational age (GA) at birth, these phenotypes had a better predictive value than GA, sex, and diagnosis of BPD for increased respiratory morbidity during infancy (AUC = 0.71 vs 0.63 for respiratory illness and 0.69 vs 0.63 for wheeze).

Conclusions: Physiologic pulmonary phenotypes of infants born preterm were associated with differential risks for respiratory morbidities as infants, which may identify heterogeneous risks for long-term respiratory sequelae to individualize therapeutic strategies.

Objective: To characterize the association between maternal ethnicity and infant survival to discharge without major morbidity.

Study design: This is secondary analysis of a prospective cohort of infants born <27 weeks of gestation at NICHD Neonatal Research Network centers from 2006 through 2020. The primary outcome was survival to discharge without major morbidity (sepsis, necrotizing enterocolitis, bronchopulmonary dysplasia grade 3, intracranial hemorrhage grade ≥3, periventricular leukomalacia, and advanced retinopathy of prematurity). Outcomes were compared by ethnicity and adjusted for center, perinatal characteristics, and sociodemographic characteristics.

Results: Of 14,029 subjects, 2155 (15%) were Hispanic, 6116 (44%) non-Hispanic Black, and 5758 (41%) non-Hispanic White. Infants of Hispanic mothers had the lowest survival to discharge without major morbidity (Hispanic 523/2099 [25%], non-Hispanic Black 1701/5940 [29%], non-Hispanic White 1494/5597 [27%], p=0.002). Adjusted odds of survival without major morbidity differed between Hispanic and non-Hispanic Black (aOR 0.80, 95% CI 0.69-0.93), but not between Hispanic and non-Hispanic White infants (aOR 1.07, 95% CI 0.92-1.25). At 2 years, children of non-Hispanic White mothers had the lowest incidence of neurodevelopmental impairment (Hispanic 544/1235 [44%], non-Hispanic Black 1574/3482 [45%], non-Hispanic White 1004/3182 [32%], p<0.001). Odds of impairment were greater for Hispanic than non-Hispanic White children (aOR 1.25, 95% CI 1.05-1.48) but did not differ between Hispanic and non-Hispanic Black children (aOR 0.88, 95% CI 0.74-1.04).

Conclusions: In a multicenter cohort, infants of Hispanic mothers had lower odds of survival to discharge without major morbidity than infants of non-Hispanic Black mothers and similar odds of survival without major morbidity as infants of non-Hispanic White mothers.

Objectives: To report the neurodevelopmental outcomes after a fetoscopic myelomeningocele (MMC) repair and to compare them with children who had an open-hysterotomy repair or a postnatal repair.

Study design: 132 infants were included (prenatal repair: 93 [69 fetoscopic and 24 open-hysterotomy]; postnatal repair: 39). Neurodevelopmental outcomes at or beyond 18 months were evaluated by a developmental pediatrician using the Capute scales (Clinical Adaptive Test [CAT]/ Clinical Linguistic & Auditory Milestone Scale [CLAMS]) and/or during parental interview using Developmental Profile 3 (DP-3) test. Scores were examined against reference values at each gestational age. A "normal" score was defined when >=70%. Neurodevelopmental scores were compared while adjusting for infants' age at the time of testing and the need for hydrocephalus treatment.

Results: After a fetoscopic repair, normal CLAMS results were observed in 25/33 (75.8%) of the cases and normal CAT in 23/33 (69.7%); DP-3 showed normal social-emotional scores in 60/65 (92.3%), normal cognition in 55/65 (84.6%), normal communication in 51/65 (78.5%), normal adaptive behavior in 48/65 (73.8%), normal general development in 39/65( 60%) and normal physical score in 31/65 (47.7%) of the cases. Children who underwent a fetoscopic repair had similar neurodevelopmental outcomes compared with those who had an open fetal surgery repair. There was a significantly higher proportion of children with "normal" CLAMS (25/33 [75.8%] vs 12/39 [30.8%], p<0.01) and "normal" CAT (23/33 [69.7%] vs 16/39 [41.0%], p=0.04) in the fetoscopic group compared with the postnatal repair group.

Conclusion: Children who undergo laparotomy-assisted fetoscopic MMC repair present with normal neurodevelopmental outcomes in two thirds of the cases included in this study at/or beyond 18 months of age and had similar neurodevelopmental outcomes as those who had an open fetal surgery repair.