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.

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 longterm 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, longterm data are available for 44 subjects.

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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 interfacility transfer rates using the U.S. 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 2007-2012, there was no significant change among each 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

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.

Background: Impaired glycogen release with fasting results in hypoglycemia in the glycogen storage diseases. A waxy-maize extended release cornstarch was introduced in the United States in 2012 to maintain glucose concentrations during the overnight period, but no studies have assessed the long-term safety and efficacy of this product in the ketotic forms of GSD. Objective: To assess long-term safety and efficacy of modified cornstarch in patients with ketotic forms of GSD. Design: An open label overnight trial of extended release cornstarch was performed. Subjects who had a successful trial (defined as optimal metabolic control lasting 2 or more hours more than with traditional cornstarch) were given the option of continuing into the long-term observational phase. Participants were assessed biochemically at baseline and after 12 months. Results: A total of 16 subjects participated in the open label trial. Efficacy was demonstrated in 100% of the subjects with GSD 0, III, VI, and IX. Of the patients who entered the longitudinal phase, long-term data are available for all subjects. The mean duration of overnight fasting on traditional cornstarch prior to the study for the cohort was 4.9 hours and 9.6 hours 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 dramatically prolongs the overnight fast duration, maximizes safety from hypoglycemic events, reduces the possibility of sleep deprivation, and improves the quality of life of patients by eliminating the need to awaken without fail for middle of the night therapy without sacrificing metabolic control.

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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.