The use of a simultaneous fascia lata-free flap with cochlear implantation after radical Mastoidectomy.

Patients undergoing cochlear implant after prior radical mastoidectomy are at increased risk of device infection requiring device explant. Various techniques including two-stage operations have been used. We report the novel technique with use of a vascularized fascia lata free flap for a patient undergoing cochlear implantation with radical mastoidectomy.

Music Therapy for Children with Oncology & Hematological Conditions and Their Families: Advancing the Standards of Psychosocial Care.

Diagnosis and treatment of cancer and blood disorders in childhood, adolescence and young adulthood has a significant impact on patients and families. The Psychosocial Standards of Care project, initiated in 2012, resulted in 15 Psychosocial Standards (PSS) that guide the care patients and families receive throughout treatment. As members of the multidisciplinary psychosocial care team, music therapists play an important role in the advancing the PSS.

Current controversies in neonatal resuscitation.

The goal of neonatal bioethics is to help clinicians navigate difficult decisions that arise every day in the care of critically ill newborns. Over the last few decades, there have been vigorous discussions of numerous ethical issues. For some, we have worked out a tentative societal agreement for appropriate responses.

IV Sotalol Use in Pediatric and Congenital Heart Patients: A Multicenter Registry Study.

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age.

A research agenda to support the development and implementation of genomics-based clinical informatics tools and resources.

Objective: The Genomic Medicine Working Group of the National Advisory Council for Human Genome Research virtually hosted its 13th genomic medicine meeting titled "Developing a Clinical Genomic Informatics Research Agenda". The meeting's goal was to articulate a research strategy to develop Genomics-based Clinical Informatics Tools and Resources (GCIT) to improve the detection, treatment, and reporting of genetic disorders in clinical settings.

Materials And Methods: Experts from government agencies, the private sector, and academia in genomic medicine and clinical informatics were invited to address the meeting's goals.

At the Intersection of Cardiology and Oncology: TGFβ as a Clinically Translatable Therapy for TNBC Treatment and as a Major Regulator of Post-Chemotherapy Cardiomyopathy.

Triple-negative breast cancer (TNBC) is a subtype of breast cancer that accounts for the majority of breast cancer-related deaths due to the lack of specific targets for effective treatments. While there is immense focus on the development of novel therapies for TNBC treatment, a persistent and critical issue is the rate of heart failure and cardiomyopathy, which is a leading cause of mortality and morbidity amongst cancer survivors. In this review, we highlight mechanisms of post-chemotherapeutic cardiotoxicity exposure, evaluate how this is assessed clinically and highlight the transforming growth factor-beta family (TGF-β) pathway and its significance as a mediator of cardiomyopathy.

Intrapersonal, interpersonal and environmental correlates of moderate to vigorous physical activity and sedentary time in adolescents with intellectual and developmental disabilities.

Background: Although correlates of physical activity (PA) have been extensively examined in both children and adolescents who are typically developing, little is known about correlates of moderate to vigorous physical activity (MVPA) and sedentary time in adolescents with intellectual and developmental disabilities (IDD). Therefore, we examined intrapersonal, interpersonal and environmental factors and their association with device-based MVPA and sedentary time in adolescents with IDD.

Methods: MVPA and sedentary time was assessed using a hip-worn ActiGraph model wGT3x-BT tri-axial accelerometer across a 7-day period in adolescents with IDD and one of their parents.

Characteristics and predictors of intensive care unit admission in pediatric blunt abdominal trauma.

Background: Pediatric trauma patients sustaining blunt abdominal trauma (BAT) with intra-abdominal injury (IAI) are frequently admitted to the intensive care unit (ICU). This study was performed to identify predictors for ICU admission following BAT.

Methods: Prospective study of children (< 16 years) who presented to 14 Level-One Pediatric Trauma Centers following BAT over a 1-year period.

Single institution review of Mini-ACE® low-profile appendicostomy button for antegrade continence enema administration.

Purpose: Malone antegrade continence enemas (MACE) provide a conduit in which the patient can achieve improved continence, be clean of stool, and gain independence in maintaining bowel function. The Mini-ACE® is a low-profile balloon button that is used to facilitate the administration of antegrade enemas. We sought to describe our practice and short-term outcomes.

Changes in physical activity across a 6-month weight loss intervention in adolescents with intellectual and developmental disabilities.

Background: Adolescents and young adults with intellectual and developmental disabilities (IDD) have high rates of obesity and low levels of physical activity. This analysis examined changes in light, moderate-to-vigorous physical activity (MVPA) and sedentary time, and the association between changes in MVPA and weight loss in adolescents and young adults with IDD and overweight and obesity participating in a 6-month multi-component weight loss intervention.

Methods: Adolescents and young adults with IDD and overweight or obesity (body mass index ≥ 85 percentile, n = 110, age ~16 years, 52.

Whole-genome sequencing-based phylogeny, antibiotic resistance, and invasive phenotype of Escherichia coli strains colonizing the cervix of women in preterm labor.

Background: Escherichia coli is a major neonatal pathogen and the leading cause of early-onset sepsis in preterm newborns. Maternal E. coli strains are transmitted to the newborn causing invasive neonatal disease.

Safety of Epicutaneous Immunotherapy in Peanut-Allergic Children: REALISE Randomized Clinical Trial Results.

Background: Treatment options for peanut allergy are limited. In previous clinical trials, epicutaneous immunotherapy with a patch containing 250-μg peanut protein (Viaskin Peanut 250 μg [VP250]) was well tolerated and statistically superior to placebo in desensitizing peanut-allergic children.

Objective: To examine the safety of VP250 in children, using a study design approximating potential real-world use.

Organ-Specific Comorbidities Are Associated With Distinct Complications After Liver Transplantation for Biliary Atresia.

Although transplant outcomes for biliary atresia (BA) have improved, there are few data to predict the risk of specific posttransplant complications. We therefore defined the impact of comorbidities in BA on posttransplant outcomes. Patients enrolled in the Society of Pediatric Liver Transplantation registry from 2011 to 2019 (n = 1034) were grouped by comorbidities of >1.

Kernicterus Spectrum Disorders Diagnostic Toolkit: validation using retrospective chart review.

Background: Kernicterus Spectrum Disorders (KSDs) result from hyperbilirubinemia-induced brain injury. We developed a Toolkit (KSD-TK) to predict the likelihood of KSDs. This study aims to validate the KSD-TK by comparing it to clinical diagnoses made by the Kernicterus Clinic in the Division of Neurology.

Outcomes of pediatric patients with therapy-related myeloid neoplasms.

Long-term outcomes after allogeneic hematopoietic cell transplantation (HCT) for therapy-related myeloid neoplasms (tMNs) are dismal. There are few multicenter studies defining prognostic factors in pediatric patients with tMNs. We have accumulated the largest cohort of pediatric patients who have undergone HCT for a tMN to perform a multivariate analysis defining factors predictive of long-term survival.

Ability of Video Telemetry to Predict Unplanned Hospital Admissions for Single Ventricle Infants.

Background Our Cardiac High Acuity Monitoring Program (CHAMP) uses home video telemetry (HVT) as an adjunct to monitor infants with single ventricle during the interstage period. This study describes the development of an objective early warning score using HVT, for identification of infants with single ventricle at risk for clinical deterioration and unplanned hospital admissions (UHA). Methods and Results Six candidate scoring parameters were selected to develop a pragmatic score for routine evaluation of HVT during the interstage period.

Pooled safety analysis of tisagenlecleucel in children and young adults with B cell acute lymphoblastic leukemia.

Background: Tisagenlecleucel, an anti-CD19 chimeric antigen receptor T cell therapy, has demonstrated efficacy in children and young adults with relapsed/refractory B cell acute lymphoblastic leukemia (B-ALL) in two multicenter phase 2 trials (ClinicalTrials.gov, NCT02435849 (ELIANA) and NCT02228096 (ENSIGN)), leading to commercialization of tisagenlecleucel for the treatment of patients up to age 25 years with B-ALL that is refractory or in second or greater relapse.

Methods: A pooled analysis of 137 patients from these trials (ELIANA: n=79; ENSIGN: n=58) was performed to provide a comprehensive safety profile for tisagenlecleucel.

Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management.