Novel Model of Childhood Appetitive Traits in Children with Obesity.

Background/objectives: Appetitive traits have a central role in energy intake and development of obesity. Child Eating Behavior Questionnaire (CEBQ) is a validated psychometric tool to measure appetitive traits in children. This study sought to assess the CEBQ factor structure in children with obesity.

Setmelanotide for the treatment of acquired hypothalamic obesity: a phase 2, open-label, multicentre trial.

Background: Hypothalamic obesity resulting from hypothalamic damage might affect melanocortin signalling. We investigated the melanocortin-4 receptor agonist setmelanotide for treatment of hypothalamic obesity.

Methods: This phase 2, open-label, multicentre trial was done in five centres in the USA.

Prediction of resting energy expenditure for adolescents with severe obesity: A multi-centre analysis.

Background And Objectives: Resting energy expenditure (REE) assessments can help inform clinical treatment decisions in adolescents with elevated body mass index (BMI), but current equations are suboptimal for severe obesity. We developed a predictive REE equation for youth with severe obesity and obesity-related comorbidities and compared results to previously published predictive equations.

Methods: Data from indirect calorimetry, clinical measures, and body composition per Dual x-ray absorptiometry (DXA) were collected from five sites.

Bardet-Biedl syndrome: A clinical overview focusing on diagnosis, outcomes and best-practice management.

Bardet-Biedl syndrome (BBS) is a genetic disorder characterized by early-onset obesity, polydactyly, genital and kidney anomalies, developmental delay and vision loss due to rod-cone dystrophy. BBS is an autosomal recessive disorder with >20 implicated genes. The genotype-phenotype relationship in BBS is not clear, and there may be additional modifying factors.

Diazoxide choline extended-release tablet in people with Prader-Willi syndrome: results from long-term open-label study.

Objective: This study assessed the effect of 1-year administration of diazoxide choline extended-release tablet (DCCR) on hyperphagia and other complications of Prader-Willi syndrome (PWS).

Methods: The authors studied 125 participants with PWS, age ≥ 4 years, who were enrolled in the DESTINY PWS Phase 3 study and who received DCCR for up to 52 weeks in DESTINY PWS and/or its open-label extension. The primary efficacy endpoint was Hyperphagia Questionnaire for Clinical Trials (HQ-CT) score.

Risk Factors for Progression to Type 2 Diabetes in a Pediatric Prediabetes Clinic Population.

Background: Pediatric type 2 diabetes (T2D) is increasing in prevalence, yet it is unclear what definition of pediatric prediabetes predicts progression to T2D. Strategies are needed to better identify at risk individuals who could benefit from early intervention.

Methods: Retrospective chart review of a pediatric prediabetes clinic over 7 years.

Prevalence of genetic causes of obesity in clinical practice.

Background: While obesity is common in the United States, monogenic obesity is rare, accounting for approximately 5% of individuals with obesity. New targeted therapies for genetic forms of obesity are available but there is limited guidance on who requires testing. The aims of this study were to evaluate the prevalence of potentially clinically significant variants among individuals in Pediatric Endocrinology or Medical Weight Center clinics at a single center and to identify clinical characteristics that may make genetic obesity more likely.

Phentermine/Topiramate for the Treatment of Adolescent Obesity.

Background: Antiobesity medication may be useful for the treatment of pediatric obesity, yet few safe and effective options exist. We evaluated phentermine/topiramate (PHEN/TPM) for weight management in adolescents with obesity.

Methods: This 56-week, randomized, double-blind trial enrolled adolescents 12 to less than 17 years of age with obesity.

Diazoxide Choline Extended-Release Tablet in People With Prader-Willi Syndrome: A Double-Blind, Placebo-Controlled Trial.

Context: Prader-Willi syndrome (PWS) is a rare neurobehavioral-metabolic disease caused by the lack of paternally expressed genes in the chromosome 15q11-q13 region, characterized by hypotonia, neurocognitive problems, behavioral difficulties, endocrinopathies, and hyperphagia resulting in severe obesity if not controlled.

Objective: The primary end point was change from baseline in hyperphagia using the Hyperphagia Questionnaire for Clinical Trials (HQ-CT). Other end points included Global Impression Scores, and changes in body composition, behaviors, and hormones.

Approach to the Patient With Hypothalamic Obesity.

Hypothalamic obesity (HO) is defined as abnormal weight gain due to physical destruction of the hypothalamus. Suprasellar tumors, most commonly craniopharyngiomas, are a classic cause of HO. HO often goes unnoticed initially as patients, families, and medical teams are focused on oncologic treatments and management of panhypopituitarism.

Prolonged lapses between pediatric and adult care are associated with rise in HbA1c and inpatient days among patients with type 1 diabetes.

Aim: To quantify the association between the duration of the pediatric-to-adult care transfer with glycemic control among patients with type 1 diabetes (T1D).

Methods: This retrospective cohort study included patients with T1D who completed transfer between pediatric and adult diabetes clinics at a single academic medical center between 2004 and 2020. The primary exposure was time from the last pediatric to first adult diabetes care encounter.

The Efficacy, Safety, and Pharmacology of a Ghrelin O-Acyltransferase Inhibitor for the Treatment of Prader-Willi Syndrome.

Context: Acylated ghrelin (AG) stimulates appetite and is elevated compared to its unacylated (UAG) counterpart in Prader-Willi syndrome (PWS). GLWL-01 is a selective, reversible inhibitor of ghrelin O-acyltransferase (GOAT), the enzyme that converts UAG into AG.

Objective: This work aimed to assess the efficacy, pharmacokinetics, pharmacodynamics, and safety of GLWL-01 in the treatment of PWS patients.

Energy balance in hypothalamic obesity in response to treatment with a once-weekly GLP-1 receptor agonist.

Background/objectives: Hypothalamic obesity (HO) frequently occurs following suprasellar tumors from a combination of decreased energy expenditure and increased energy intake. Glucagon-like peptide-1 receptor agonist (GLP1RA) therapy is associated with increased satiety and energy expenditure. We hypothesized GLP1RA therapy in patients with HO would cause both lower energy intake and increased energy expenditure.

Calcitriol and Levothyroxine Dosing for Patients With Pseudohypoparathyroidism.

Pseudohypoparathyroidism (PHP) is a rare hormone resistance syndrome caused by mutations in . This cross-sectional study investigated whether PHP patients with parathyroid hormone (PTH), thyrotropin (thyroid stimulating hormone; TSH), and free thyroxine (T4) levels at goal required higher doses of levothyroxine and calcitriol than recommended by current guidelines to overcome mineral ion abnormalities due to hormone resistance. Baseline demographic and clinical data of participants enrolled in PHP research studies between 2012-2021 were collected via retrospective chart review.

Racial and Ethnic Disparities in Comorbidities in Youth With Type 2 Diabetes in the Pediatric Diabetes Consortium (PDC).

Background: Type 2 diabetes in the U.S. is more prevalent in youth of minority racial-ethnic background, but disparities in health outcomes have not been examined in this population.

Outcomes of Laparoscopic Peritoneal Dialysis Catheter Placement Using an Optimal Placement Technique.

Background: Peritoneal dialysis (PD) is a widely employed renal replacement modality. A prospective study was conducted to determine the short-term and midterm outcomes and complication rates associated with a standardized optimal laparoscopic peritoneal dialysis catheter placement technique.

Methods: All patients undergoing laparoscopic PD catheter placement by one surgeon using our standardized method over a 5-year period were entered into a prospective database.

MRI measures of hypothalamic injury are associated with glucagon-like peptide-1 receptor agonist treatment response in people with hypothalamic obesity.

Aim: To evaluate whether neuroimaging-delineated regions of hypothalamic injury are associated with a differential treatment response to a glucagon-like peptide-1 receptor agonist (GLP-1RA) in patients with hypothalamic obesity (HO).

Materials And Methods: We performed a prespecified secondary analysis of a randomized, multicentre, double-blind, placebo-controlled trial of people aged 10-25 years with hypothalamic injury and HO randomized to the GLP-1RA exenatide once-weekly (ExQW) or placebo for 36 weeks. Subjects underwent MRI prior to enrolment and the degree of hypothalamic damage was assessed using an integrative hypothalamic lesion score (HLS).

A phase 3 randomized clinical trial using a once-weekly glucagon-like peptide-1 receptor agonist in adolescents and young adults with hypothalamic obesity.

Aim: To evaluate the efficacy, safety and tolerability of a glucagon-like peptide-1 receptor agonist (GLP-1 RA) in patients with hypothalamic obesity (HO).

Materials And Methods: A two-arm, randomized, multicentre, double-blind, placebo-controlled trial was conducted in 10- to 25-year-olds with hypothalamic injury following intracranial tumour and HO. Participants were randomized to once-weekly subcutaneous injections of a GLP-1 RA exenatide 2 mg (ExQW) or placebo for 36 weeks.

Recommendations for Diagnosis and Treatment of Pseudohypoparathyroidism and Related Disorders: An Updated Practical Tool for Physicians and Patients.

Patients affected by pseudohypoparathyroidism (PHP) or related disorders are characterized by physical findings that may include brachydactyly, a short stature, a stocky build, early-onset obesity, ectopic ossifications, and neurodevelopmental deficits, as well as hormonal resistance most prominently to parathyroid hormone (PTH). In addition to these alterations, patients may develop other hormonal resistances, leading to overt or subclinical hypothyroidism, hypogonadism and growth hormone (GH) deficiency, impaired growth without measurable evidence for hormonal abnormalities, type 2 diabetes, and skeletal issues with potentially severe limitation of mobility. PHP and related disorders are primarily clinical diagnoses.

Reoperations for Long-Term Complications Following Laparoscopic Adjustable Gastric Banding: Analysis of Incidence and Causality.

Background: Laparoscopic adjustable gastric banding (LAGB) gained popularity in the early 2000s as a purely restrictive procedure with modest weight loss. The potential for complications requiring reoperation has since become evident. A retrospective review was performed to determine the incidence of long-term complications and predictive factors requiring surgical reintervention after LAGB.

A Clinic-based Approach to Diagnosis and Management of Prediabetes in High-risk Children and Adolescents.

Background: Type 2 diabetes (T2D) in youth is increasing in prevalence. Diabetes screening is recommended for at-risk youth but best-practice strategies for management of pediatric prediabetes are unknown. This study leverages a pediatric prediabetes clinic to assess identification of high-risk patients, the rate of clinic follow-up and progression to T2D in youth over time.

Trends in pediatric obesity management, a survey from the Pediatric Endocrine Society Obesity Committee.

Background In the United States, 18.5% of children are obese. Dietary and lifestyle modifications are key, but often ineffective.

Impaired amygdala-based learning and decreased anxiety in a murine model of pseudohypoparathyroidism type 1A.

Pseudohypoparathyroidism type 1A (PHP1A) is a genetic disorder caused by maternally inherited mutations in the gene Gnas. PHP1A is characterized by multiple hormone impairment, early onset obesity and cognitive impairment. Animal models of PHP1A are needed to investigate the mechanism of cognitive impairment.