Sedation-free transnasal esophagoscopy to evaluate and monitor esophageal diseases in children with esophageal atresia-tracheoesophageal fistula.

Patients with esophageal atresia and tracheoesophageal fistula (EA-TEF) are at increased risk of conditions including gastroesophageal reflux, peptic esophagitis, gastric metaplasia, anastomotic strictures, eosinophilic esophagitis, and dysphagia. Patients with TEF-EA may need serial endoscopy in their lifetime given the known short- and long-term GI complications. There has been increased interest in pediatric unsedated transnasal endoscopy (TNE) as an endoscopic alternative as it is lower cost, has shorter recovery time, and eliminates potential risks associated with anesthesia. We report on the use of TNE with EA-TEF in four patients: One patient had gastroesophageal reflux disease, one patient had eosinophilic esophagitis and TNE was used for surveillance in two patients. Use of TNE allowed for close endoscopic monitoring and changes in medication management. The third and fourth patients underwent TNE as part of routine EA-TEF screening which is recommended by societal guidelines (Krishnan et al, J Pediatr Gastroenterol Nutr. 2016;63(5):550-570). Unsedated TNE is an alternative endoscopic approach in the management of patients with EA-TEF.

Previous SARS-CoV-2 Infection Is Not Associated With Increased Celiac Disease Autoimmunity in Children and Adolescents.

INTRODUCTION: Recent reports suggest severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infections may increase the risk of celiac disease autoimmunity. This study aims to evaluate potential associations between coronavirus disease 2019 infection and tissue transglutaminase autoantibodies (TGA) immunoglobulin A. METHODS: From 2020 to 2021, cross-sectional screening for SARS-CoV-2 antibodies and TGA was offered to 4,717 children in Colorado through the Autoimmunity Screening for Kids study. Multivariable logistic regression assessed association between previous SARS-CoV-2 infection and TGA positivity. RESULTS: Previous SARS-CoV-2 infection was not associated with TGA positivity (odds ratio 1.02, 95% confidence interval 0.63-1.59; P = 0.95). DISCUSSION: In this large-scale analysis, previous SARS-CoV-2 infection was not associated with celiac disease autoimmunity in Colorado children.

Diagnostic Tools for Early Detection of Biliary Atresia: Is a Newborn Screen Attainable?

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Billing I-AIM: a novel framework for ultrasound billing.

BACKGROUND: Point-of-care ultrasound (POCUS) has an ever-growing footprint in medicine. With this growth POCUS billing and reimbursement has become an area gaining quite a bit of attention as a means of funding and sustaining quality and education programs. Standardization across providers is needed to improve the financial viability of POCUS. RESULTS: We created an institutional collaborative which developed a framework to identify critical POCUS billing and reimbursement checkpoints. The framework, Billing I-AIM, provides a feasible structure to enhance provider-based reimbursement and perform quality improvement efforts across variable POCUS environments. CONCLUSIONS: POCUS billing using the Billing I-AIM technique allows administrative oversight, quality assurance, and educational functions as well. A discussion of the framework and respective application is provided.

Emergency medical services (EMS) versus non-EMS transport among injured children in the United States.

OBJECTIVES: This study aimed to assess the proportions of injured children transported to trauma centers by different transportation modes and evaluate the effect of transportation mode on inter-facility transfer rates using the US national trauma registry. METHODS: We analyzed data from the 2007-2012 National Trauma Data Bank (NTDB) to study trends of EMS versus non-EMS transport. Multivariable logistic regression was used to evaluate the association between transport mode and inter-facility transfer. RESULTS: There were 286,871 pediatric trauma patients in the 2007-2012 NTDB; 45.8% arrived by ground ambulance, 8.6% arrived by air ambulance, and 37.5% arrived by non-EMS. From 2007 to 2012, there was no significant change in transportation mode. Moderate to severely injured patients (ISS>15) comprised 13.3% of arrivals by ground ambulance, 26.7% of arrivals by air ambulance, and 8.3% of arrivals by non-EMS; those who used EMS were significantly less likely to be transferred to another facility than patients who used non-EMS transport. Moderate and severe pediatric patients arriving by non-EMS to adult trauma centers were more often transferred than those arriving at mixed trauma centers (45.8% and 6.8%, respectively). CONCLUSIONS: Over one third of US pediatric trauma patients used non-EMS transport to arrive at trauma centers. Moderate to severely injured children benefit from EMS transport and professional field triage to reach the appropriate trauma facility. Our study suggests that national efforts are needed to increase awareness among parents and the general public of the benefits of EMS transportation and care.

Safety and Efficacy of Chronic Extended Release Cornstarch Therapy for Glycogen Storage Disease Type I.

BACKGROUND: Glycogen storage disease type I (GSD I) causes severe hypoglycemia during periods of fasting since both glycogenolysis and gluconeogenesis are impaired. Primary treatment in North America consists of cornstarch therapy every 3-4 h. Waxy maize extended release cornstarch was introduced for maintaining overnight glucose concentrations, but no studies have assessed long-term safety and efficacy of the product. OBJECTIVE: To demonstrate the safety and efficacy of modified cornstarch in GSD I. DESIGN: An open-label overnight trial of extended release cornstarch was performed. Subjects with a successful trial (optimal metabolic control 2 or more hours longer than with traditional cornstarch) were given the option of continuing into the chronic observational phase. Subjects were assessed biochemically at baseline and after 12 months. RESULTS: Of the 106 subjects (93 GSD Ia/13 GSD Ib), efficacy was demonstrated in 82 patients (88%) with GSD Ia and 10 patients (77%) with GSD Ib. The success rate for extending fasting was 95% for females and 78% for males. Of the patients who entered the longitudinal phase, long-term data are available for 44 subjects. Mean duration of fasting on traditional cornstarch prior to study for the cohort was 4.1 and 7.8 h on the extended release cornstarch (P < 0.001). All laboratory markers of metabolic control have remained stable in the chronically treated patients. CONCLUSION: Extended release cornstarch appears to improve the quality of life of patients with GSD I without sacrificing metabolic control. Avoiding the overnight dose of cornstarch should enhance safety in this population.

Evaluation of glycogen storage disease as a cause of ketotic hypoglycemia in children.

INTRODUCTION: Ketone formation is a normal response when hypoglycemia occurs. Since the majority of children with recurrent hypoglycemia cannot be diagnosed with a known endocrine or metabolic disorder on a critical sample, ketotic hypoglycemia has been described as the most common cause of low blood glucose concentrations in children. Critical samples, however, will miss the ketotic forms of glycogen storage disease (GSD), which present with elevated ketones, hypoglycemia, and normal hormonal concentrations. RESULTS: A total of 164 children (96 boys, 68 girls) were enrolled in the study. Prediction of pathogenicity of DNA changes using computer modeling confirmed pathology in 20 individuals [four GSD 0, two GSD VI, 12 GSD IX alpha, one GSD IX beta, one GSD IX gamma] (12%). Boys were most likely to have changes in the PHKA2 gene, consistent with GSD IX alpha, an X-linked disorder. CONCLUSIONS: Mutations in genes involved in glycogen synthesis and degradation were commonly found in children with idiopathic ketotic hypoglycemia. GSD IX is likely an unappreciated cause of ketotic hypoglycemia in children, while GSD 0 and VI are relatively uncommon. GSD IX alpha should particularly be considered in boys with unexplained hypoglycemia.