Management of Neonatal Hyperinsulinemic Hypoglycemia: Trends Over Nine Years.

Background: With increasing use of diazoxide for hyperinsulinemic hypoglycemia (HH), reporting of serious side effects of diazoxide such as pulmonary hypertension (PHT) increased.

Methods: Charts of all children diagnosed with HH during the study period and evaluated by Pediatric Endocrinology division of the Hasbro Children's Hospital were reviewed. We analyzed diazoxide use among infants with HH with focus on infants born small for gestational age (SGA) and preterm infants.

Increase in the Incidence of Type 2 Diabetes in Young Children During the COVID-19 Pandemic.

Objective: To describe the incidence of new onset type 2 diabetes (T2D) among children younger than 11 years old diagnosed during the COVID-19 pandemic (March 1, 2020-December 31, 2021) compared to those diagnosed during the pre-pandemic period (January 1, 2017-February 29, 2020) and to compare the metabolic parameters of those with T2D diagnosed before age 11 years with those diagnosed after age 11 years.

Methods: We conducted a retrospective cohort study of youth <21 years with new onset T2D treated at Hasbro Children's Hospital between January 1, 2017 and December 31, 2021. Patients diagnosed at age <11 years were compared to those ≥11 years.

Hypolipidemia due to Familial Hypobetalipoproteinemia in Adolescents.

Background/objective: Individuals with heterozygous familial hypobetalipoproteinemia (h-FHBL) due to loss-of-function mutation in the apolipoprotein B gene are typically asymptomatic with mild liver dysfunction, which is often detected incidentally. About 5% to 10% of those with h-FHBL develop steatohepatitis which occasionally progress to cirrhosis especially in the presence of alcohol use, excess calorie consumption, or liver injury. We report 3 patients with hypobetalipoproteinemia, 2 with confirmed h-FHBL, and 1 with suspected h-FHBL.

Change in Body Mass Index in Youth in the First 5 Years After Type 1 Diabetes Mellitus Diagnosis.

Objective: Examine body mass index (BMI) trajectories in American youth with type 1 diabetes (T1D) over the first 5 years following diagnosis.

Methods: Retrospective record review of BMI trajectories in youth with T1D diagnosed in 2015 to 2016.

Results: Near the time of diabetes diagnosis, 35.

The Evaluation and Management of Methimazole-Induced Agranulocytosis in the Pediatric Patient: A Case Report and Review of the Literature.

Introduction: Agranulocytosis is a rare, but serious complication of methimazole (MMI) use for Graves' disease (GD). Treatment requires discontinuation of MMI, and the use of propylthiouracil (PTU) is also contraindicated. Few reports exist about the optimal alternative treatment regimens for the management of thyrotoxicosis in these medically complex patients in the pediatric population.

Persistent hypercalcemia mimicking hypophosphatasia after discontinuation of a ketogenic diet: a case report.

Objectives: Hypercalcemia has been reported as an uncommon complication of the ketogenic diet (KD). Here we present a toddler whose hypercalcemia persisted for 2 months after stopping the KD.

Case Presentation: A 2 year 11-month-old child with global developmental delay, infantile spasms, neuromuscular weakness with limited mobility, tracheostomy and ventilator dependence, and oropharyngeal dysphagia with G-tube dependence presented with hypercalcemia in the setting of recurrent vomiting.

The Rising Incidence of Hyperinsulinemic Hypoglycemia: Connection With Maternal Health.

Objective: Due to a perceived rise in hyperinsulinemic hypoglycemia (HH) cases over time, notably during the COVID-19 pandemic, institutional experiences between 2013 and 2021 were reviewed to evaluate trends, characteristics, and outcomes in children with HH.

Methods: Charts of all children diagnosed with HH during the study period and evaluated by Pediatric Endocrinology were reviewed. HH was defined per Pediatric Endocrine Society guidelines.

Incidence, Severity, and Presentation of Type 2 Diabetes in Youth During the First and Second Year of the COVID-19 Pandemic.

Objective: To describe the evolving impact of the coronavirus disease 2019 pandemic on the incidence and presentation of new-onset pediatric type 2 diabetes.

Research Design And Methods: Retrospective medical record review of youth with new-onset type 2 diabetes comparing the prepandemic period (1 January 2017-29 February 2020) with the first (1 March 2020-31 December 2020) and second pandemic year (1 January 2021-31 December 2021).

Results: The annualized incidence of type 2 diabetes increased nearly threefold during the pandemic versus prior, with a 61% increase in the 2nd versus 1st year.

Severe Vitamin D Deficiency in Youth with Autism Spectrum Disorder During the COVID-19 Pandemic.

Background: Decrease in sunlight exposure during periods of social distancing during the COVID-19 pandemic increased the risk of severe manifestations of vitamin D deficiency (VDD) in a particular "high-risk" population. Our objective was to highlight the importance of vitamin D screening in youth with autism spectrum disorder (ASD) and restrictive eating.

Case Presentation: We describe 3 adolescent male patients with ASD who developed severe manifestations of VDD and hypocalcemia in late 2020 during the COVID-19 pandemic.

Bacteremia in a Newborn with Hypocalcemic Seizures and Vitamin D Deficiency.

Infants with neonatal hypocalcemia often present with seizures, and neonatal hypocalcemia can be due to parathyroid (PTH) insufficiency or resistance. Causes of hypocalcemia with PTH elevation include increased phosphate load, vitamin D deficiency (VDD) or defects in metabolism, renal dysfunction, hypomagnesemia, genetic mutations resulting in end-organ resistance to PTH, or critical illness. Hypocalcemia has also been shown to be associated with Gram-negative bacteremia and sepsis in adults.

Self-reported insulin pump prescribing practices in pediatric type 1 diabetes.

Introduction: Disadvantaged and minority youth with type 1 diabetes are less likely to be on insulin pump therapy compared to the majority population. Little is known about how pediatric endocrinology providers determine eligibility for insulin pump. We aimed to identify provider factors influencing the decision to initiate insulin pump therapy.

The Incidence of Erosive Esophagitis as a Complication of Pediatric Diabetic Ketoacidosis.

Introduction: Gastrointestinal (GI) symptoms commonly occur during diabetic ketoacidosis (DKA) and typically resolve with treatment. However, GI complications can persist after DKA resolves. The incidence of upper GI bleeding during DKA in adults has been described, with erosive esophagitis one of the most common lesions.

NOVEL MUTATIONS IN AN INFANT WITH MICROCEPHALIC PRIMORDIAL DWARFISM, DILATED CARDIOMYOPATHY, SUBCLINICAL HYPOTHYROIDISM, AND EARLY DEATH: EXPANDING THE PHENOTYPE OF MUTATIONS.

Objective: Microcephalic primordial dwarfism (MPD) is a group of clinically and genetically heterogeneous disorders which result in severe prenatal and postnatal growth failure. X-ray repair cross-complementing protein 4 () is a causative gene for an autosomal recessive form of MPD. The objective of this report is to describe novel mutations in a female infant with MPD, dilated cardiomyopathy, and subclinical hypothyroidism.

EARLY-ONSET GONADOBLASTOMA IN A 13-MONTH-OLD INFANT WITH 46,XY COMPLETE GONADAL DYSGENESIS IDENTIFIED WITH PRENATAL TESTING: A CASE OF CHROMOSOME 9p DELETION.

Objective: Individuals with 46,XY complete gonadal dysgenesis (CGD) are at high risk of developing gonadal neoplasms. Chromosome 9p monosomy with deletion of the gene, a key transcription factor in testicular development, is one of the known causes of 46,XY CGD. Noninvasive prenatal testing (NIPT) is being increasingly used, and can identify disorders of sexual development (DSDs).

Graves' Thyrotoxicosis Leading to Adrenal Decompensation and Hyperandrogenemia in a Pediatric Patient with Salt-Wasting Congenital Adrenal Hyperplasia.

Introduction: Thyroid hormone is known to accelerate glucocorticoid turnover. In a thyrotoxic state, individuals with adrenal insufficiency are unable to increase endogenous cortisol production to compensate for increased turnover, placing them at risk for symptoms of glucocorticoid deficiency and adrenal crisis. In patients with salt-wasting congenital adrenal hyperplasia (SW-CAH), hyperandrogenemia is a measurable reflection of relative glucocorticoid insufficiency.

Nephrolithiasis: A complication of pediatric diabetic ketoacidosis.

Objective: To determine the frequency of nephrolithiasis as a complication of diabetic ketoacidosis (DKA) in pediatrics.

Methods: We performed a retrospective chart review of patients with DKA admitted to a pediatric hospital between January 2009 and July 2016. We identified patients with nephrolithiasis during admission for DKA.

"Counselor in Your Pocket": Youth and Provider Perspectives on a Mobile Motivational Intervention for Marijuana Use.

Previous interventions for marijuana use have been administered out of the real-life contexts in which use occurs. In 2010, we interviewed youth aged 15-24 years who use marijuana frequently (n = 8) and providers who treat them (n = 6) on the acceptability and utility of a mobile intervention involving momentary self-monitoring of use-related contexts and responsive motivational messaging following clinic-based brief motivational enhancement therapy. Thematic analysis was used to examine youth and provider perspectives on the mobile intervention.

A Pilot Study of Ketamine versus Midazolam/Fentanyl Sedation in Children Undergoing GI Endoscopy.

Background. Ketamine sedation has been found superior by physician report to traditional sedation regimens for pediatric endoscopy. Goal.

Effects of simulation versus traditional tutorial-based training on physiologic stress levels among clinicians: a pilot study.

Introduction: Emotionality and heightened anxiety during medical simulation encounters have been hypothesized to contribute to improved cognition and learning, but the overall stress "dose response curve" of experiential learning remains unclear. We sought to (1) identify the degree and time course of physiologic stress induced in physicians by simulation-based training (SBT), when compared with a traditional tutorial-based interactive-education training (IET) and (2) compare differences in stress responses to simulation activities among pediatric provider groups.

Method: Twenty-seven gastroenterology physicians were randomized among six crisis resource management courses taught by SBT versus IET.

Perceptions of simulation-based training in crisis resource management in the endoscopy unit.

Simulation-based training (SBT) teaches healthcare teams to manage critical events, using crisis resource management skills. The purpose of this study was to assess staff perceptions of using SBT in the endoscopy suite. Nurses and technicians were invited to participate in a training program that used simulation of endoscopy-based scenarios to teach crisis resource management skills.

Fellow perceptions of training using computer-based endoscopy simulators.

Background: Integrating procedural training by using computer-based endoscopic simulators (CBES) into gastroenterology fellowships may facilitate technical skill development, while posing no additional risk to patients.

Objective: The aim of our study was to survey pediatric gastroenterology fellows about their experiences with and perceptions of CBES as compared with actual procedures, prior to and after exposure to both types of endoscopic learning.

Design And Setting: All first-year trainees at Children's Hospital Boston (2003-2008) were invited to complete a written, pretraining questionnaire and then perform at least 10 each of CBES endoscopies and colonoscopies prior to performing actual procedures.

Nurse reports of adverse events during sedation procedures at a pediatric hospital.

The purpose of our study was to examine the reliability of nurse reports of adverse events related to procedural sedation in children. A descriptive, correlational design was used to analyze for inter-rater agreement between prospective adverse event reporting and that identified on independent review of the medical record. All sedation documentation at a pediatric hospital over one calendar year was reviewed, and inter-rater reliability of reporting was analyzed using K statistics.

Endoscopic sedation in pediatric practice.

Best sedation practices for pediatric endoscopy involve the consideration of many patient factors, including age, medical history, clinical status, and anxiety level, as well as physician access to anesthesia support. A recent survey of pediatric gastroenterologists suggests that endoscopist-administered intravenous (iv) sedation and anesthesiologist-administered propofol represent common sedation regimens in children. Technical advances in ventilatory monitoring are contributing to increased patient safety for all children undergoing gastrointestinal procedures, regardless of sedation type.