Novel findings regarding the role of the endocannabinoid system in pediatric functional gastrointestinal disorders.

Introduction: Pain constitutes the chief complaint of some functional gastrointestinal disorders (FGIDs). The endocannabinoid (EC) and peroxisome proliferator-activated receptors (PPARs) agonist systems have not been explored as possible contributors.

Objective: To determine if EC and PPAR agonist abnormalities occur in adolescents with FGID.

13 C-Spirulina Gastric Emptying Breath Test Normative Values in Healthy Children: A Multicenter Study With Comparison to Symptomatic Children.

Introduction: Diagnosing pediatric gastroparesis presents a significant challenge due to lack of normative data in children. We aimed to determine normative values for gastric emptying using the nonradioactive 13 C-Spirulina gastric emptying breath test ( 13 C-GEBT) in a large cohort of healthy children (HC) and examine differences compared to children with gastroparesis-like symptoms (GLS).

Methods: A total of 301 HC ages 7-18 years completed the 13 C-GEBT with 10 different breath samples × 4 hours.

Neurocognitive passport: A novel feedback tool for pediatric patients with sickle cell disease.

Sickle Cell Disease (SCD) is associated with neurocognitive impairment, and routine cognitive and academic screening is recommended. Traditional reports following testing are often not health literate, limiting the usefulness of evaluations. This study investigated whether a small "passport" summarizing results and recommendations would be helpful in increasing understanding of results and follow-through on recommendations.

Diagnostic characteristics of pediatric cyclic vomiting syndrome.

Objectives: Cyclic vomiting syndrome (CVS) remains a diagnostic challenge due to its nonspecific presentation despite consensus-based diagnostic criteria. There is a need for improved, evidence-based diagnostic criteria. We hypothesized that symptoms differ quantitatively between children with CVS versus other vomiting conditions and that current diagnostic criteria are not sufficiently sensitive for diagnosing CVS.

Variants Are Associated with Atrial Dysfunction in Neonates with Hypoplastic Left Heart Syndrome.

: variants are the most well-known genetic risk factor (10%) for hypoplastic left heart syndrome (HLHS) and are associated with decreased cardiac transplant-free survival. encodes for α-myosin heavy chain (α-MHC), a contractile protein expressed in the neonatal atria. We therefore assessed atrial function in HLHS patients with variants.

Reduction in ACE2 expression in peripheral blood mononuclear cells during COVID-19 - implications for post COVID-19 conditions.

Background: Severe COVID-19 is uncommon, restricted to 19% of the total population. In response to the first virus wave (alpha variant of SARS-CoV-2), we investigated whether a biomarker indicated severity of disease and, in particular, if variable expression of angiotensin converting enzyme 2 (ACE2) in blood might clarify this difference in risk and of post COVID -19 conditions (PCC).

Methods: The IRB-approved study compared patients hospitalized with severe COVID-19 to healthy controls.

Evaluating threshold for donor fraction cell-free DNA using clinically available assay for rejection in pediatric and adult heart transplantation.

Background: The aims of the study were to assess the performance of a clinically available cell-free DNA (cfDNA) assay in a large cohort of pediatric and adult heart transplant recipients and to evaluate performance at specific cut points in detection of rejection.

Methods: Observational, non-interventional, prospective study enrolled pediatric and adult heart transplant recipients from seven centers. Biopsy-associated plasma samples were used for cfDNA measurements.

Impact of Underweight, Overweight, and Obesity on Health-Related Quality of Life in Children with Tetralogy of Fallot Variants.

Traditional cardiovascular risk factors put patients with congenital heart disease (CHD) at increased risk for cardiovascular morbidity and mortality. The aim of this study was to evaluate whether body mass index (BMI) is associated with health-related quality of life (HRQoL) in patients with variants of Tetralogy of Fallot (TOF). Patients and parents of children with variants of TOF-CHD were asked to fill out the PedsQL 4.

A multicenter registry study on percutaneous electrical nerve field stimulation for pediatric disorders of gut-brain interaction.

Objectives: Percutaneous electrical nerve field stimulation (PENFS) has demonstrated promise in single-center trials for pediatric abdominal pain-related disorders of gut-brain interaction (DGBI). Our aim was to explore efficacy of PENFS as standard therapy for DGBI in a registry involving multiple pediatric gastroenterology referral centers.

Methods: This was a multicenter, prospective open-label registry of children (8-18 years) undergoing PENFS for DGBI at seven tertiary care gastroenterology clinics.

Carboplatin, paclitaxel, and pembrolizumab followed by pembrolizumab maintenance for primary treatment of incompletely resected epithelial ovarian cancer.

Objective: Incompletely resected epithelial ovarian cancer represents a poor prognostic subset of patients. Novel treatment strategies are needed to improve outcomes for this population. We evaluated a treatment strategy combining platinum-based chemotherapy with pembrolizumab followed by pembrolizumab maintenance therapy in the first-line treatment after incomplete resection of epithelial ovarian cancer patients.

A serum-induced gene signature in hepatocytes is associated with pediatric nonalcoholic fatty liver disease.

Objective: Pediatric nonalcoholic fatty liver disease (NAFLD) is a growing problem, but its underlying mechanisms are poorly understood. We used transcriptomic reporter cell assays to investigate differences in transcriptional signatures induced in hepatocyte reporter cells by the sera of children with and without NAFLD.

Methods: We studied serum samples from 45 children with NAFLD and 28 children without NAFLD.

Influence of the COVID-19 Pandemic on Health-Care Career Interests Among High School Students.

Purpose: This study investigates the influence of the COVID-19 pandemic on high school students' interest in health-care careers.

Methods: A voluntary web-based survey, approved by the Medical College of Wisconsin's Institutional Review Board, was administered across eight high schools in Milwaukee and its suburbs in Wisconsin. The survey collected students' demographic details, opinions on the health-care system's pandemic response, and their interest in health-care careers before and after the pandemic.

Assessment of comorbid symptoms in pediatric autonomic dysfunction.

Purpose: Pediatric patients with autonomic dysfunction and orthostatic intolerance (OI) often present with co-existing symptoms and signs that might or might not directly relate to the autonomic nervous system. Our objective was to identify validated screening instruments to characterize these comorbidities and their impact on youth functioning.

Methods: The Pediatric Assembly of the American Autonomic Society reviewed the current state of practice for identifying symptom comorbidities in youth with OI.

Percutaneous electrical nerve field stimulation improves comorbidities in children with cyclic vomiting syndrome.

Introduction: Children with cyclic vomiting syndrome (CVS) frequently suffer from disabling abdominal pain and comorbidities that impair quality of life. A noninvasive, auricular percutaneous electrical nerve field stimulation (PENFS) device is shown to be effective for abdominal pain in children with disorders of gut-brain interaction. We aimed to determine the effects of PENFS on pain, common comorbidities, and quality of life in pediatric CVS.

Percutaneous Electrical Nerve Field Stimulation for Drug-Refractory Pediatric Cyclic Vomiting Syndrome.

Background: Cyclic vomiting syndrome (CVS) is a disabling condition frequently refractory to pharmacologic therapy. The aim of this study was to evaluate the effects of noninvasive, auricular percutaneous electrical nerve field stimulation (PENFS) as prophylactic therapy for pediatric CVS.

Methods: Children 8-18 years with drug-refractory CVS were prospectively enrolled from a tertiary care CVS clinic.

Higher Weight-Based Doses Are Required to Achieve and Maintain Therapeutic Voriconazole Serum Trough Concentrations in Children.

Objective: Children require weight-based voriconazole doses proportionately larger than adults to achieve therapeutic serum trough concentrations (1-6 mcg/mL). The objective of this quality improvement project was to determine the initial dose, proportion of patients achieving target concentrations with initial dosing, and subsequent therapeutic drug monitoring and dose modifications needed to achieve and maintain therapeutic voriconazole concentrations in children.

Methods: This retrospective study evaluated children aged <18 years treated with voriconazole during the study period.

Does High-Intensity Exercise Cause Acute Liver Injury in Patients with Fontan Circulation? A Prospective Pilot Study.

The Fontan procedure results in chronic hepatic congestion and Fontan-associated liver disease (FALD) characterized by progressive liver fibrosis and cirrhosis. Exercise is recommended in this population, but may accelerate the progression of FALD from abrupt elevations in central venous pressure. The aim of this study was to assess if acute liver injury occurs after high-intensity exercise in patients with Fontan physiology.

Stress Numerical Rating Scale-11: Validation in Pediatric Inpatient and Outpatient Pain Settings.

Stress is a commonly reported issue in pediatric populations of chronic and acute pain. Both outpatient and inpatient settings impose time constraints, which decreases opportunities to measure and address patient stress. The aim of these studies was to evaluate the validity of the Stress Numeric Rating Scale-11 (SNRS-11) in both inpatient and outpatient settings.

Natural History of Pediatric Cyclic Vomiting Syndrome: Progression to Dysautonomia.

Background: The clinical features of pediatric cyclic vomiting syndrome (CVS) often evolve over time. Many patients develop a constellation of chronic symptoms that suggest autonomic nervous system (ANS) dysfunction during adolescence. We aimed to determine the proportion of children with CVS who develop chronic rather than episodic symptoms consistent with ANS dysfunction.

Creating a data dictionary for pediatric autonomic disorders.

Purpose: Whether evaluating patients clinically, documenting care in the electronic health record, performing research, or communicating with administrative agencies, the use of a common set of terms and definitions is vital to ensure appropriate use of language. At a 2017 meeting of the Pediatric Section of the American Autonomic Society, it was determined that an autonomic data dictionary comprising aspects of evaluation and management of pediatric patients with autonomic disorders would be an important resource for multiple stakeholders.

Methods: Our group created the list of terms for the dictionary.