hrp0084p3-976 | GH & IGF | ESPE2015

Thyroid Function in Children with Prader-Willi Syndrome, the First 12 Months of GH Therapy

Musthaffa Yassmin M , Hughes Ian P , Harris Mark , Leong Gary

Background: Normal thyroid function is necessary for the optimal growth promoting effects of GH. Changes in the hypothalamic-pituitary-thyroid (HPT) axis following GH have been reported in subjects initially thought to be euthyroid. A specific group of patients, children with Prader-Willi syndrome (PWS), are thought to have a ‘vulnerable’ HPT axis.Objective and hypotheses: To evaluate the impact of paediatric GH therapy on thyroid hormone statu...

hrp0089p3-p165 | Fat, Metabolism and Obesity P3 | ESPE2018

Hidden Hunger in Overweight/obese Indian Adolescents

Jain Vandana , Upadhyaya Babita , Agarwala Anuja

Background and Objective: Overweight/obese children may have unrecognized deficiency of several essential micronutrients owing to their faulty dietary habits. These may impair their physical and mental development. Deficiency of specific minerals and vitamins that co-factors in metabolic and signalling pathways, such as choline, zinc, magnesium, vitamins D and B12 may also predispose to insulin resistance, fatty liver and metabolic syndrome. This study was undertaken to assess...

hrp0095fc5.3 | Adrenals and HPA Axis | ESPE2022

The liver as a site for rAAV-induced ectopic adrenocortical enzyme expression

Graves Lara , Koyyalamudi Sundar , Wotton Tiffany , Srinivasan Shubha , Ginn Samantha , Alexander Ian

Background: The adrenal cortex undergoes constant cellular renewal and any effect from recombinant adeno-associated vectors (rAAV) targeting differentiated cells will be transient as extra-chromosomal DNA will be lost by dividing cells. A novel option is to use rAAV to express ectopic adrenocortical enzymes in a stable organ outside the adrenal gland. Our aim was to correct the biochemical phenotype in a mouse model for congenital adrenal hyperplasia (CAH) wit...

hrp0092rfc4.6 | Fat Metabolism and Obesity Session | ESPE2019

Appetite Suppressing Effects of Glucoregulatory Peptides Devoid of Nausea

Doyle Robert P. , Elfers Clinton T. , Milliken Brandon T. , Sweet Ian , Roth Christian L.

Few treatments for type 2 diabetes (T2D) and obesity achieve meaningful long-term weight-loss and are often accompanied by nausea and vomiting. Thus, there is a critical need for a new generation of obesity medications that provide glycemic control with enhanced hypophagic response without nausea. Our group has developed and tested two new monomeric chimeric peptides against a novel target for obesity treatment concomitant with T2D in the form of dual agonism of the anorectic ...

hrp0086p1-p814 | Syndromes: Mechanisms and Management P1 | ESPE2016

Changes to Thyroid Function (TF) Following Treatment with Growth Hormone (GH) Therapy in Children with Prader-Willi Syndrome (PWS)

Musthaffa Yassmin , Scheermeyer Elly , Hughes Ian , Harris Mark , Crock Patricia , Leong Gary

Background: Normal TF is necessary for optimal growth. Changes in the hypothalamic-pituitary-thyroid (HPT) axis following GH therapy are reported. GH therapy has been suggested to centrally inhibit TSH production as well as peripherally increasing T4 to T3 conversion which increases negative feedback on TSH production. Hypothalamic dysfunction is a feature of PWS, therefore these patients may be at risk of developing central hypothyroidism associated with GH therapy.<p cla...

hrp0086lbp1 | (1) | ESPE2016

46,XY DSD due to Isolated AMH Deficiency Resulting in Persistent Müllerian Duct Syndrome (PMDS) as a Consequence of a Single-Base Deletion in a SF1-Response Element of the AMH promoter

Valeri Clara , di Clemente Nathalie , Marshall Ian , Schteingart Helena , Josso Nathalie , Rey Rodolfo , Picard Jean-Yves

Background: Isolated persistence of Müllerian ducts in an otherwise normally virilised 46,XY newborn, a condition known as PMDS, is a disorder of sex development (DSD) due to a defect limited to AMH-dependent Müllerian duct regression.Objective and hypothesis: We report the case of a patient with PMDS and extremely low serum AMH in whom no mutations were detected in the AMH gene coding sequences. A single base deletion identified in th...

hrp0082p3-d3-655 | Autoimmune Endocrine Disease | ESPE2014

A Conservative Approach to the Management of Endocrine Neoplasia in Carney Complex in an Adolescent Male

Frerichs Carley , Didi Mohammed , Abernethy Laurence , Kenny Simon , Ellis Ian , Blair Joanne

Background: Carney complex (CC) is a rare, dominantly inherited condition due to mutations of the tumour suppressor gene PRKAR1A. Endocrine manifestations include: Cushing’s syndrome (CS) due to primary pigmented nodular adrenocortical disease, pituitary adenomas, testicular neoplasms, thyroid tumours, and ovarian cysts. The management of some of these tumours is controversial.Objective and hypotheses: To describe conservative management of CC.<...

hrp0097p2-249 | Late Breaking | ESPE2023

Longitudinal Improvements in Health-Related Quality of Life among Children and Adolescents enrolled in Canadian Pediatric Weight Management Programs

Ybarra Marina , Rebekah Grace , D. C. Ball Geoff , Buchholz Annick , Hamilton Jill , Zenlea Ian , Morrison Katherine

Background: It is critical to understand patient-reported outcomes in pediatric patients enrolled in pediatric weight management (PWM) clinics. Health-related quality of life (HRQoL) is low in children with obesity. Utilizing data from the CANadian Pediatric Weight Management Registry (CANPWR), we examined (1) changes in HRQoL up to 3 years after enrollment in PWM and (2) factors associated with change in HRQoL over time.Methods:...

hrp0082p3-d1-900 | Pituitary | ESPE2014

Effect of Pubertal Status, Age and Gender on Cortisol Response to Insulin Induced Hypoglycaemia in Children and Adolescents

Giri Dinesh , Mulvey Ian Robert , Gangadharan Arundoss , Saravanai-Prabu K , Blair Jo , Dharmaraj Poonam , Das Urmi , Senniappan Senthil , Didi Mohammed

Background: There is evidence that baseline and peak cortisol response to the low dose short Synacthen test (LDSST) varies with pubertal status and gender in children with asthma on inhaled corticosteroids. There are no published data reporting effects of puberty on cortisol response to the insulin tolerance test (ITT) in childhood and adolescence.Objective and hypotheses: To determine whether pubertal status, age or gender influence basal and peak corti...

hrp0097rfc8.2 | Fat, metabolism and obesity 2 | ESPE2023

Acute rise of leptin after five days of dexamethasone and its association with hunger, fat mass, sleep and fatigue, in children with acute lymphoblastic leukemia

van Hulst Annelienke , Verwaaijen Emm , van den Berg Sjoerd , van Litsenburg Raphaele , Grootenhuis Martha , Fiocco Marta , Neggers Sebastian , van den Heuvel-Eibrink Marry , van den Akker Erica

Background & Aims: Children with acute lymphoblastic leukemia (ALL) frequently receive high doses dexamethasone during treatment, which may induce acute side effects. The aims of the current study were to determine the influence of a five-day dexamethasone course on changes in leptin, fat mass, body mass index (BMI), hunger, sleep and fatigue and to explore the associations between these changes.Methods: Pediatric AL...