Functional MRI for bilingual epilepsy surgery patients: serving a diverse pediatric cohort.

Objective: Functional MRI (fMRI) helps with the identification of eloquent cortex to assist with function preservation in patients who undergo epilepsy surgery. Language and memory tasks can even be used effectively in clinically involved pediatric patients. Most pediatric studies report on English speaking-only cohorts from English-dominant countries, yet languages other than English (LOEs) are increasingly prevalent in countries such as the US.

Over 100 Fontan Patients on Systemic Ventricular Assist Device Support: An ACTION Update.

As the Fontan population grows, understanding successful strategies for ventricular assist device (VAD) support of the failing Fontan circulation is needed. We performed a retrospective analysis of patients with Fontan circulation and systemic VAD support in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. Competing outcomes and Kaplan-Meier estimated survival methods were used.

Concordance and discordance of anticoagulation assays in children supported by ECMO: The truth is out there.

Introduction: Extracorporeal membrane oxygenation (ECMO) provides critical support to patients in severe cardiac and respiratory failure, but it requires anticoagulation to prevent complications like bleeding and thrombosis. Heparin, the primary anticoagulant utilized, is monitored by activated partial thromboplastin time (aPTT) and anti-Factor Xa (AntiXa) levels. Discordance between the two assays complicates its titration and the impact on patient outcomes is not well-established.

Socioeconomic and Ethnic Disparities in Timing and Outcome of Interarytenoid Injection Augmentation.

Objectives: To investigate the relationship between social determinants of health and timeliness of management, adherence to follow-up, and outcomes of treatment with interarytenoid injection augmentation (IAIA).

Methods: Retrospective cohort study of all pediatric patients treated with IAIA at a large pediatric institution's multidisciplinary aerodigestive clinic between August 2022 and February 2024. Retrieved demographic factors, dates of referral, consultation, treatment, and follow-up, as well as objective measures of dysphagia and aspiration via videofluoroscopic swallow study (VFSS) reports, using dysphagia outcome and severity scale (DOSS) scores and the greatest unsafe thickness, or 'aspiration score'.

Management of Persistent Hypernasality After Pharyngeal Flap by Revision Palatoplasty With Use of MRI to Aid Surgical Decision Making.

Objective: Describe surgical decision making and outcomes in a series of patients with persistent VPI after pharyngeal flap placement that were all treated with revision palatoplasty.

Design: Retrospective, case series.

Participants: Five patients with nonsyndromic cleft palate and persistent hypernasality following a pharyngeal flap.

Consensus Guideline for Care of Patients in the Prehospital and Aerospace Settings with Exposures to Hydrazine and Hydrazine Derivatives.

Objectives: Hydrazine (HZ) and Hydrazine Derivative (HZ-D) exposures pose health risks to people in industrial and aerospace settings. Several recent systematic reviews and case series have highlighted common clinical presentations and management strategies. Given the low frequency at which HZ and HZ-D exposures occur, a strong evidence base on which to develop an evidence-based guideline does not exist at this time.

Evaluation of a Digital Home Weight Monitoring Program for Infants With Cleft Palate.

Objective: Evaluate the effectiveness of a digital home monitoring program for infants with cleft palate with or without cleft lip (CP ± L), compared to monitoring through in-person clinic visits.

Design: Retrospective cohort study.

Setting: One metropolitan pediatric hospital.

Does machine learning improve prediction accuracy of the Endoscopic Third Ventriculostomy Success Score? A contemporary Hydrocephalus Clinical Research Network cohort study.

Purpose: This Hydrocephalus Clinical Research Network (HCRN) study had two aims: (1) to compare the predictive performance of the original ETV Success Score (ETVSS) using logistic regression modeling with other newer machine learning models and (2) to assess whether inclusion of imaging variables improves prediction performance using machine learning models.

Methods: We identified children undergoing first-time ETV for hydrocephalus that were enrolled prospectively at HCRN sites between 200 and 2020. The primary outcome was ETV success 6 months after index surgery.

Outcomes Following Fecal Diversion for Intractable Hirschsprung Associated Enterocolitis: A Study From the Pediatric Colorectal and Pelvic Learning Consortium.

Background: Hirschsprung associated enterocolitis (HAEC) is a challenging problem in a subset of children with Hirschsprung disease (HD). In refractory cases, fecal diversion may be required. The aim of this study was to characterize patients who require fecal diversion for HAEC management and examine their long-term outcomes.

Outcome of Surgery for Hypothalamic Hamartoma-Related Epilepsy: A Systematic Review and Individual Participant Data Meta-Analysis.

Background And Objectives: There is a paucity of data directly comparing the outcome of surgical techniques available for the treatment of hypothalamic hamartomas (HHs). This study aims to evaluate the safety and efficacy of commonly used surgical approaches in the treatment of HH-related epilepsy.

Methods: A systematic review and individual participant data (IPD) meta-analysis was conducted.

Optimal Timing for Safe Bivalving of Fiberglass Casts Is Before the Exothermic Peak.

Introduction: Cast saw injury is a notable source of medicolegal risk. Previous work with plaster casts demonstrated that cast saw injury was minimized by waiting 12 minutes before removal. In this study, we evaluate the safety parameters of fiberglass casting materials.

The FGFR inhibitor Rogaratinib reduces microglia reactivity and synaptic loss in TBI.

Background: Traumatic brain injury (TBI) induces an acute reactive state of microglia, which contribute to secondary injury processes through phagocytic activity and release of cytokines. Several receptor tyrosine kinases (RTK) are activated in microglia upon TBI, and their blockade may reduce the acute inflammation and decrease the secondary loss of neurons; thus, RTKs are potential therapeutic targets. We have previously demonstrated that several members of the Fibroblast Growth Factor Receptor (FGFR) family are transiently phosporylated upon TBI; the availability for drug repurposing of FGFR inhibitors makes worthwhile the elucidation of the role of FGFR in the acute phases of the response to TBI and the effect of FGFR inhibition.

Clinical characteristics and outcome of central nervous system tumors harboring NTRK gene fusions.

Purpose: TRK fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients.

Experimental Design: We conducted an international retrospective cohort study of patients with TRK fusion-driven CNS tumors.

Clinical Actionability of Genetic Findings in Cerebral Palsy: A Systematic Review and Meta-Analysis.

Importance: Single gene variants can cause cerebral palsy (CP) phenotypes, yet the impact of genetic diagnosis on CP clinical management has not been systematically evaluated.

Objective: To evaluate how frequently genetic testing results would prompt changes in care for individuals with CP and the clinical utility of precision medicine therapies.

Data Sources: Published pathogenic or likely pathogenic variants in OMIM genes identified with exome sequencing in clinical (n = 1345) or research (n = 496) cohorts of CP were analyzed.

Duchenne Muscular Dystrophy in Two Half-Brothers Due to Inherited 306 Kb Inverted Insertion of 10p15.1 into Intron 44 of the Dp427m Transcript of the Gene.

Duchenne muscular dystrophy (DMD) is a rare genetic disorder caused by the absence of a fully functional dystrophin protein in myocytes. In skeletal muscle, the lack of dystrophin ultimately results in muscle wasting and the replacement of myocytes with fatty or fibrous tissues. In the heart, cardiomyocytes eventually fail and cause fatal cardiomyopathy.

Focused on the Family: Development of a Family-Based Intervention Promoting the Transition to Adult Health Care for Adolescents with Type 1 Diabetes.

There is minimal evidence for current interventions promoting the transition to adult healthcare for youth with type 1 diabetes (T1D). Few interventions exclusively target modifiable individual and family-based factors that contribute to transition readiness. The purpose of this paper is to describe the development of Behavioral Family Systems Therapy for Diabetes Transition (BFST-DT), a virtual family-based transition readiness intervention for adolescents with T1D.

Atypical Teratoid Rhabdoid Tumor of the Brain in a Young Adult With Down Syndrome: Case Report and Literature Review.

Atypical teratoid/rhabdoid tumor (ATRT) is an aggressive, malignant embryonal tumor with dismal long-term survival despite aggressive multimodal therapy. While this tumor typically presents in infancy or early childhood, there are published case reports of adult-onset ATRT. Making prognostic conclusions or therapeutic decisions for this older patient population remains challenging due to the paucity of these reports.

Efficacy and Complications of Interarytenoid Injection for Dysphagia in Infants 1-Year-Old and Under.

Objective: The objective of this study was to assess the efficacy and complication rates of interarytenoid injection augmentation (IAIA) for the treatment of dysphagia in patients 1 year of age and under and to determine if concurrent feeding therapy (FT) affects outcome.

Study Design: Retrospective case series.

Setting: Tertiary pediatric hospital.

Unhurried Conversations in Health Care Are More Important Than Ever: Identifying Key Communication Practices for Careful and Kind Care.

Unhurried conversations are necessary for careful and kind care that is responsive and responsible to both patients and clinicians. Adequate conceptual development is an important first step in being able to assess and measure this important domain of quality of care. In this article, we expand on a preliminary model to identify the key microlevel communication practices that support an unhurried conversation, defined as an ongoing, mutual accomplishment between patient and clinician that proceeds through a range of verbal and nonverbal communication practices wherein one or more participants (mutually) regulate the sequence, spacing (temporal and spatial), and speed of interaction to make themselves available to the other and remove or suspend distractions from the environment in order to improve care.