Children's Hospital & Medical Center

Bicuspid aortic valve (BAV) is the most common congenital heart lesion that can be associated with significant valve dysfunction in childhood. Surgical interventions for this condition often include pulmonary autograft (Ross procedure) or aortic valve replacement (mechanical - mAVR - or bioprosthetic - bAVR). Aortic valve repair (Rpr) was a common primary intervention for BAV with aortic stenosis at our institution during the study period. This single-center cohort study included 150 adults with BAV and aortic stenosis who underwent 197 surgical interventions during childhood. We investigated important clinical demographic differences, long-term freedom from repeat aortic valve intervention, and long-term freedom from adverse events based upon the type of definitive aortic valve surgery. We hypothesized that Ross and mAVR would demonstrate superior temporal durability, but that mAVR would have more associated adverse events, and that Ross would require more lifetime sternotomies. Ross and mAVR demonstrated superior temporal durability when compared to other aortic valve interventions by pairwise comparison (Rpr vs mAVR p < 0.001; Rpr vs Ross p < 0.001; Rpr vs bAVR p = 0.075; mAVR vs Ross p = 0.924; mAVR vs bAVR p = 0.026; Ross vs bAVR p = 0.001). Ross demonstrated superior freedom from adverse events when compared directly to bAVR (Rpr vs mAVR p = 0.755; Rpr vs Ross p = 0.033; Rpr vs bAVR p = 0.816; mAVR vs Ross p = 0.040; mAVR vs bAVR p = 0.847; Ross vs bAVR p = 0.006). Ross procedure was not associated with an increased number of lifetime sternotomies compared to mAVR. Concurrent Konno procedure was not an independent predictor of worse outcomes.

Diet during early infancy, as well as dietary patterns during childhood and parental feeding styles influence children’s eating behaviours and long-term health. While extensive data exist on the timing of complementary feeding introduction and recommended food frequencies during infancy and childhood, data on portion sizes during this period remains limited in Europe, despite their clear relevance for evaluating dietary patterns and supporting evidence-based guidance. This longitudinal study, secondary to the European Childhood Obesity Project (EU CHOP), aims to describe portion sizes consumed by infants and children aged 6 months to 8 years across five European countries. Dietary intake was recorded using 3-day food diaries and analysed by trained personnel following standardized procedures at multiple infant and childhood ages (6, 7, 8, 9, 12, 24, 36, 48, 60, 72 and 96 months). Portions sizes were calculated for 33 food groups and expressed as percentiles in the overall sample and stratified by country. A total of 1018 3-day food records were available at 6 months, with sample size gradually declining over the 11 follow-up time points to 400 records at 8 years. The results revealed a wide variation in portion sizes across food groups, ages and countries. Food portion sizes vary across food groups, age and countries. These findings provide reference percentiles representing typical portion sizes when foods are consumed. The data can support guidance provided to parents by healthcare professionals (pediatricians, nurses, and dietitian-nutritionists) and assist public health authorities in defining appropriate portion sizes and mitigating risks associated with both overeating and undereating, while respecting children’s hunger and satiety cues. These results also have practical applications in school meal planning and dietary assessment methodologies in research.

Objective To examine the utility of implementing a neurorehabilitation intervention in pediatric psychology and neuropsychology training programs for building clinical competencies in neurorehabilitation. Method Using a convergent mixed methods approach, we surveyed neuropsychological and pediatric psychology trainees who completed training for the Teen Online Problem-Solving program (TOPS). The survey asked about training and competency on a 5-point Likert scale. Open-ended responses to “How has completing TOPS training changed how you practice as a psychologist?” were used for inductive thematic analyses. We surveyed site leaders and supervising psychologists at three hospitals (two in the United States and one in Canada) that integrated TOPS into their psychology training programs to provide case studies. Results 30 trainees completed the survey. Quantitatively, they indicated the TOPS program was a valuable part of training and improved understanding of neurological sequelae. Trainees endorsed that TOPS increased confidence and comfort working with adolescents and families and enhanced competency in delivering evidence-based interventions for neurodevelopmental disorders. Qualitative analyses yielded five themes: (1) enhanced clinical skills, (2) better tailoring to patients’ needs, (3) greater confidence delivering and adapting interventions, (4) equipping families with a helpful tool, and (5) informed feedback and recommendations for families. Conclusion Our mixed-methods analysis suggests that integrating evidence-based neurorehabilitation programs, such as TOPS, into neuropsychology and pediatric psychology training can build competencies, confidence, and interdisciplinary collaboration in neurorehabilitation.

Neonatal encephalopathy (NE)-including hypoxic-ischemic encephalopathy (HIE)-is associated with significant morbidity and mortality worldwide. While data registries provide hypothesis-generating data, aid in quality improvement, and track management/outcomes over time, we recently demonstrated that only 4 of 1,281 (0.3%) variables were collected by all 22 international NE/HIE registries: birth weight, gestational age, and 1- and 5-minute Apgar scores. In response, our team set out to develop a set of common inpatient data elements to enable future harmonization of NE/HIE registry data. Using a modified Delphi method, a panel of 14 international NE experts voted to separate the 1,281 variables from our previous study into "core elements," "supplemental elements," or those that "do not need to be collected." Based on the anonymous survey results, we created draft lists of core and supplemental common data elements (CDEs). These lists were further revised through group consensus meetings and external feedback from global registry leaders and attendants at 2 international conferences. The final data forms include 164 core elements and 225 supplemental elements stratified into 11 domains: demographics; pregnancy, labor, and delivery; delivery room; transport; acid-base; therapeutic hypothermia; neuromonitoring and seizures; neuroimaging; laboratory values (other than acid-base); hospital course; and discharge. The CDEs are the first of several important steps to further standardize research and reporting of NE/HIE investigations to perform more efficient and powerful clinical research in the field moving forward. Future studies need to establish a clinical and research definition of HIE and develop CDEs for collecting long-term, family-centered follow-up data postdischarge.

HighlightsWhat are the main findings?Not all routine images obtained exhibit sufficient quality and guidelines for image-based 3D model creation and quality assurance are notably absent.Understanding the fundamental tenets of image quality (spatial resolution, contrast, noise) gives rise to the recommendations and guidelines developed here.What is the implication of the main finding?When imaging is performed for the purpose of 3D modeling, image quality may need to be optimized above routine parameters and thin-section images must be archived.Even in the case of suboptimal imaging, 3D models can be created to facilitate surgical planning and patient/family education.Background: Three-dimensional (3-D) models are gaining interest for surgical planning in the field of pediatric surgical oncology. The diverse tumor types in pediatric patients and the non-uniform presentation and anatomy make 3D modeling in pediatric oncology particularly challenging. In addition, centers have variability in technique and experience with these approaches. Guidelines for model creation and quality assurance are notably absent. Objective: We developed national recommendations pertaining to image acquisition and model creation for 3-D renderings based on the existing literature and multidisciplinary expertise. Materials and Methods: The literature was critically appraised, and the authors developed minimum standard guidelines for imaging and modeling pediatric solid tumors. Strength of recommendation scores for each guideline were independently determined by the authors. Results: Twelve specific guidelines were developed and scored with overall strength of recommendation ranging from moderate to very strong. Guidelines focused on image acquisition were uniformly scored very strongly while scores for those pertaining to segmentation and model display were slightly less consistent. Conclusions: We propose minimum standards for image acquisition and modern 3-D modeling of pediatric solid tumors based on literature and expertise. The overall level of agreement among our multidisciplinary team was high.

Alagille syndrome (ALGS) is a rare, autosomal dominant disorder which presents with a broad range of clinical manifestations, including cholestatic pruritus. A unique manifestation of ALGS is the presence of xanthomas in 24%–42% of patients, which can lead to liver transplantation. Maralixibat, an ileal bile acid transporter (IBAT) inhibitor, has demonstrated improvements in both cholestatic pruritus and xanthomas in clinical trials. We report here on the use of maralixibat in two patients with ALGS and unusual manifestations of xanthomatosis, including one patient with airway xanthomas and a second patient with severe, diffuse xanthomas. In both cases, almost complete resolution of severe, debilitating xanthomas and clinically meaningful improvements in pruritus and serum bile acid levels were observed after up to 1 year of treatment with maralixibat. These cases support the utilization of maralixibat for the management of ALGS beyond cholestatic pruritus.