Development and validation of clinical criteria to identify familial chylomicronemia syndrome (FCS) in North America.

Background: Familial chylomicronemia syndrome (FCS) is an ultrarare inherited disorder. Genetic testing is not always feasible or conclusive. European clinicians developed a "FCS score" to differentiate between FCS and multifactorial chylomicronemia syndrome (MCS), a more common condition with overlapping features.

Beyond the Guidelines: Perspectives on Management of Pediatric Patients with Hypertriglyceridemia.

Purpose Of Review: To provide a comprehensive overview of hypertriglyceridemia (HTG) in youth, identifying gaps in categorizing triglyceride (TG) levels and management strategies, and exploring new therapies for TG reduction.

Recent Findings: Non-fasting TG levels as important cardiovascular (CV) risk indicators, with HTG's pathophysiology involving genetic and secondary factors affecting TG metabolism. Emerging treatments, including those affecting the lipoprotein lipase complex and inhibiting proteins like apoC3 and ANGPTL3, show promise.

Exploring the Gut Microbiota: Key Insights Into Its Role in Obesity, Metabolic Syndrome, and Type 2 Diabetes.

The gut microbiota (GM), comprising trillions of microorganisms in the gastrointestinal tract, is a key player in the development of obesity and related metabolic disorders, such as type 2 diabetes (T2D), metabolic syndrome (MS), and cardiovascular diseases. This mini-review delves into the intricate roles and mechanisms of the GM in these conditions, offering insights into potential therapeutic strategies targeting the microbiota. The review elucidates the diversity and development of the human GM, highlighting its pivotal functions in host physiology, including nutrient absorption, immune regulation, and energy metabolism.

Rising tide: The global surge of type 2 diabetes in children and adolescents demands action now.

Childhood-onset obesity has emerged as a major public healthcare challenge across the globe, fueled by an obesogenic environment and influenced by both genetic and epigenetic predispositions. This has led to an exponential rise in the incidence of type 2 diabetes mellitus in children and adolescents. The looming wave of diabetes-related complications in early adulthood is anticipated to strain the healthcare budgets in most countries.

Precocious Puberty and GnRH Analogs: Current Treatment Practices and Perspectives among US Pediatric Endocrinologists.

Introduction: Gonadotropin releasing hormone analogs (GnRHas) are used for treatment of precocious puberty. Over the last decade, several new formulations have been approved.

Methods: The Drugs and Therapeutics Subcommittee of the Pediatric Endocrine Society (PES) undertook a review to ascertain the current treatment options, prescribing behaviors, and practices of GnRHas among pediatric endocrinologists practicing within the USA.

Altered lipid metabolism and the development of metabolic-associated fatty liver disease.

Purpose Of Review: An increasing amount of research has underscored the significant role of lipoproteins in the pathogenesis of metabolic-associated fatty liver disease (MAFLD). This comprehensive review examines the intricate relationship between lipoprotein abnormalities and the development of MAFLD.

Recent Findings: Atherogenic dyslipidemia seen in insulin resistance states play a significant role in initiating and exacerbating hepatic lipid accumulation.

Invited Mini Review Metabolic Bone Disease of Prematurity: Overview and Practice Recommendations.

Metabolic bone disease of prematurity (MBDP) is defined by undermineralization of the preterm infant skeleton arising from inadequate prenatal and postnatal calcium (Ca) and phosphate (PO4) accretion. Severe MBDP can be associated with rickets and fractures. Despite advances in neonatal nutrition, MBDP remains prevalent in premature infants due to inadequate mineral accretion ex-utero.

Children and Adolescents With Hybrid Diabetes: A Management Conundrum.

Objective: Hybrid diabetes (HD); ie, insulin resistance with positive diabetes-associated autoantibodies (DAAs) is increasing in children. We aimed to compare the characteristics of children with HD with those with type 1 diabetes (T1DM) and type 2 diabetes (T2DM) at diagnosis and after 2 years.

Methods: A retrospective review of patients aged 0 to 19 years, with C-peptide and 4 DAA measurements available, who were diangosed with new-onset diabetes from 2016 to 2020 were included in the analysis.

Lipid Disorders and Metabolic-Associated Fatty Liver Disease.

Dyslipidemia has been linked metabolic-associated fatty liver disease (MAFLD). Several genes and transcription factors involved in lipid metabolism can increase susceptibility to MAFLD. Multiple parallel 'hits' have been proposed for developing hepatic steatosis, NASH, and MAFLD, including insulin resistance and subsequent free fatty acid excess, de novo lipogenesis, and excessive hepatic triglyceride and cholesterol deposition in the liver.

Adrenal Insufficiency in Peroxisomal Disorders: A Single Institution Case Series.

Introduction: There are two major categories of peroxisomal disorders (PDs): peroxisomal biogenesis disorders (PBDs) due to defects in peroxisomal (PEX) genes and deficiency of other peroxisomal enzymes (such as D-bifunctional enzyme deficiency due to HSD17B4). PDs are characterized by abnormal elevations of very-long-chain fatty acids (VLCFA). We aimed to evaluate the clinical phenotype of adrenal insufficiency in patients with PD and to assess any genotype-phenotype correlations with adrenal insufficiency.

Hyponatremia after craniotomy in children: a single-institution review.

Purpose: Hyponatremia after craniotomy can be associated with increased morbidity. However, the incidence of and factors associated with post-craniotomy hyponatremia in children are not known.

Methods: We performed a retrospective cohort study of patients aged 0-21 years who underwent craniotomy in 2017-2019 at a single center to determine the incidence of and to identify risk factors for hyponatremia after craniotomy.

Diagnostic Test Accuracy of Urine C-peptide Creatinine Ratio for the Correct Identification of the Type of Diabetes: A Systematic Review.

: To examine the accuracy of urine c-peptide creatinine ratio (UCPCR) for identifying the type of diabetes in appropriate clinical settings. : Systematic review of test accuracy studies on patients with different forms of diabetes. : Medline, Embase and Cochrane library databases from 1 January 2000 to 15 November 2020.

Pharmacologic Weight Management in the Era of Adolescent Obesity.

Context: Pediatric obesity is a serious health problem in the United States. While lifestyle modification therapy with dietary changes and increased physical activity are integral for the prevention and treatment of mild to moderate obesity in youth, only a modest effect on sustained weight reduction is observed in children and young adults with severe obesity. This underscores the need for additional evidence-based interventions for children and adolescents with severe obesity, including pharmacotherapy, before considering invasive procedures such as bariatric surgery.

Safety and Efficacy of Early Vitamin D Supplementation in Critically Ill Extremely Preterm Infants: An Ancillary Study of a Randomized Trial.

Background: Despite substantial evidence that vitamin D deficiency is highly prevalent among infants born extremely preterm (≤28 weeks' of gestation), several consensus statements do not recommend vitamin D doses >400 IU/day for these infants. Safety remains a concern.

Objective: The study aim was to determine safety and efficacy profiles of enteral vitamin D in Black and White infants randomized to three different vitamin D doses soon after birth.

Treatment of hyperglycemia not associated with NAFLD improvement in children with type 2 diabetes mellitus.

Background And Objectives: Nonalcoholic fatty liver disease (NAFLD) and type 2 diabetes mellitus (T2DM) have become public health problems in the pediatric population. However, the relationship between these two conditions is not well understood. The primary objective of this study was to assess whether treatment of hyperglycemia in obese, treatment-naive children with type 2 diabetes (T2DM) was associated with an improvement of surrogate markers of NAFLD.

Changes in Type 2 Diabetes Trends in Children and Adolescents During the COVID-19 Pandemic.

Context: There is concern that the growing incidence of pediatric type 2 diabetes (T2D) may have been further exacerbated by the COVID-19 pandemic.

Objective: To examine whether trends in new-onset pediatric T2D-inclusive of patients requiring hospitalization and patients managed as outpatients-were impacted during the COVID-19 pandemic, and to compare patient characteristics prior to and during COVID-19.

Methods: A retrospective single-center medical record review was conducted in a hospital which cares for 90% of Alabama's pediatric T2D patients.

Childhood Hypertriglyceridemia: Is It Time for a New Approach?

Purpose Of Review: Hypertriglyceridemia (HTG) is widely prevalent in youth. There is an unmet need for effective medications in the management of HTG in youth. The purpose of this review is to summarize the approach to HTG in acute and chronic settings, and highlight emerging therapies targeted at specific genes, proteins, and enzymes to selectively alter triglyceride (TG) metabolism.

Novel therapeutic targets and agents for pediatric dyslipidemia.

Landmark studies have convincingly demonstrated that atherosclerosis begins in youth. While generally asymptomatic, an increasing number of youth with disorders of lipid and lipoprotein metabolism, such as familial hypercholesterolemia, are being identified through selective and universal screening. While a heart healthy lifestyle is the foundation of treatment for all youth with dyslipidemia, lipid-lowering therapy may be required by some to prevent morbidity and premature mortality, especially when initiated at a young age.

SARS-CoV-2 infection in hospitalized children with type 1 and type 2 diabetes.

Context: While diabetes is a risk factor for severe illness from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in adults, there is conflicting data surrounding the relationship between the virus and diabetic disease process in children.

Objective: This case series aims to illustrate an increase in the incidence of types 1 and 2 diabetes mellitus (T1DM, T2DM) between April - November 2020 at a large tertiary care children's hospital and examine the characteristics and adverse outcomes in these children. In addition, two children with significant complications from coronavirus disease 2019 (COVID-19) and diabetes are highlighted.

Lipoprotein(a) Concentrations Correlate With LDL-C in Children With Type 1 and 2 Diabetes.

Context: Elevated levels of lipoprotein(a) (Lp[a]) is an independent risk factor for atherosclerotic cardiovascular disease especially in patients with diabetes. Adult levels of Lp(a) are thought to be is expressed by the second year of life.

Objective: We hypothesized that Lp(a) would be influenced by low density lipoprotein cholesterol (LDL-C), race, and HbA1C.

Case Studies in Pediatric Lipid Disorders and Their Management.

Context: Identification of modifiable risk factors, including genetic and acquired disorders of lipid and lipoprotein metabolism, is increasingly recognized as an opportunity to prevent premature cardiovascular disease (CVD) in at-risk youth. Pediatric endocrinologists are at the forefront of this emerging public health concern and can be instrumental in beginning early interventions to prevent premature CVD-related events during adulthood.

Aim: In this article, we use informative case presentations to provide practical approaches to the management of pediatric dyslipidemia.