hrp0089fc10.2 | Late Breaking | ESPE2018

Efficacy and Safety of Once-Weekly Somapacitan in Childhood Growth Hormone Deficiency: Results of a Randomised Open-Label, Controlled Phase 2 Trial

Savendal Lars , Rasmussen Michael , Horikawa Reiko , Khadilkar Vaman , Battelino Tadej , Saenger Paul

Background: Growth hormone deficiency (GHD) requires long-term daily injections with GH replacement therapy and is associated with considerable treatment burden by patients and caregivers. Somapacitan is a long-acting GH derivative that is being developed for once-weekly dosing in adults and children with GHD. A well-established protraction method, which is successfully in use to extend the half-life of insulin and glucagon-like peptide (GLP)-1, has been applied in developing ...

hrp0089p1-p009 | Adrenals and HPA Axis P1 | ESPE2018

The Relationship of Baseline, Incremental and Peak Cortisol Following a Short Synacthen Test – Single-centre Analysis of Three Years’ Data

Aji Apoorva , Colyer Sharon , Burn Sarah , Dimitri Paul , Wright Neil , Krone Nils , Elder Charlotte

Introduction: The Short Synacthen Test (SST) is the most popular diagnostic investigation of adrenal insufficiency (AI) amongst adult and paediatric endocrinologists. AI can present insidiously and symptoms may be non-specific. The number of medical indications for glucocorticoids is growing and SST usage is correspondingly increasing. There is evidence that an early morning plasma cortisol (EMC) of below ~100–150 nmol/l is highly predictive of failing the SST and the cor...

hrp0089p2-p021 | Adrenals and HPA Axis P2 | ESPE2018

Borderline Peak Plasma Cortisol Following Synacthen Stimulation – Single-centre Analysis of Three Years Data

Burn Sarah , Colyer Sharon , Dimitri Paul , Wright Neil , Krone Nils , Elder Charlotte

Introduction: The Short Synacthen Test (SST) is the most popular diagnostic investigation of adrenal insufficiency (AI) worldwide. Symptoms of AI are frequently non-specific, often delaying diagnosis, however fortunately cases of adrenal crisis remain relatively rare. Diagnostic cut-offs for plasma cortisol on SST are controversial, made more complicated by modern assays and paediatric normative values extrapolated from adult data. Some advocate a division between biochemical ...

hrp0089p2-p368 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P2 | ESPE2018

A Case of Gonadal Dysgenesis Due to a Novel Homozygous Mutation in NR5A2 Gene

Denzer Friederike , Denzer Christian , Hornig Nadine , Holterhus Paul-Martin , Hiort Olaf , Wabitsch Martin

Background: Steroidogenic factor (SF1, NR5A2) regulates multiple genes known to be involved in gonadal development, adrenal development, steroidogenesis, and gonadotroph development. Heterozygous mutations in the NR5A1 gene have been described in association with mild to severe gonadal dysgenesis with or without adrenal failure. Homozygous mutations are rare and have also been described in association with gonadal dysgenesis with or without adrenal failure.<p class="abstex...

hrp0089p3-p386 | Thyroid P3 | ESPE2018

Papillary Thyroid Carcinoma in a 7 Year Old Boy Presenting with a Goiter Without Microcalcifications and Enlarged Cervical Lymph Nodes

Becker Marianne , Vega Letizia , De Beaufort Carine , Philippe Paul , Kieffer Isabelle

Introduction: Only 1.8% of thyroid malignancies occur during childhood, explaining very limited pediatric data. Most frequent in children are papillary thyroid carcinoma (PTC), occurring after exposure to radiation, and presenting as a thyroid nodule ± cervical lymph nodes. PTC may present as diffusely infiltrating disease of the thyroid with microcalcifications. We report an uncommon presentation of a PTC in a 7 year old boy.Case report: The boy wa...

hrp0086fc2.3 | Bone &amp; Mineral Metabolism | ESPE2016

The Impact of Intragastric Balloon Placement Suppported by a Lifestyle Intervention Programme on Cortical and Trabecular Microstructure and Strength in Severely Obese Adolescents

Sachdev Pooja , Reece Lindsey , Copeland Rob , Wales Jerry , Dimitri Paul , Wright Neil

Background: The effect of profound weight loss following obesity surgery on skeletal microarchitecture and strength in adolescents has not been studied. Obese children are at an increased risk of fracture and childhood obesity leads to reconfiguration of trabecular bone without augmenting bone strength.Objective and hypotheses: To examine the impact of weight loss following 6 months treatment with an intragastric balloon supported by a lifestyle interven...

hrp0086rfc4.4 | Pathophysiology of Obesity | ESPE2016

Metabolic Syndrome Markers Correlate with Gut Microbiome Activity in Children Born Very Preterm

Chiavaroli Valentina , Jayasinghe Thilini N , Ekblad Cameron , Derraik Jose , Hofman Paul , O'Sullivan Justin , Cutfield Wayne

Background: Fifteen years ago children born very preterm (<32 weeks of gestation) were reported to be insulin resistant. Neonatal intensive care has since improved considerably, but it is unclear whether this has affected long-term outcomes in those born preterm. Abnormalities in gut microbiome, which influence host metabolism, have been found in preterm newborns.Objective and hypotheses: We aimed to assess whether children born very preterm still ha...

hrp0086rfc10.4 | Perinatal Endocrinology | ESPE2016

Pharmacokinetics of Intravenous Glucagon in Children with Hyperinsulinaemic Hypoglycaemia

Shah Pratik , Rahman Sofia , Gilbert Clare , Morgan Kate , Hinchey Louise , Bech Paul , Amin Rakesh , Hussain Khalid

Background: Hyperinsulinaemic hypoglycaemia (HH) is one of the common causes of hypoglycaemia in infants and children. It can cause severe brain injury in children if not treated promptly. Diazoxide is first-line treatment for HH. Glucagon infusion is used in the management of children with HH. However it is unclear what dose of glucagon should be used in children.Objective and hypotheses: To evaluate the efficacy, safety and pharmacokinetics of intraven...

hrp0086p1-p455 | Fat Metabolism and Obesity P1 | ESPE2016

Seven-year Follow-up of Mothers from a Randomized Controlled Trial of Exercise in Pregnancy and their Offspring

Chiavaroli Valentina , Hopkins Sarah , Derraik Jose , Seneviratne Sumudu , Biggs Janene , Rodrigues Raquel , Cutfield Wayne , Hofman Paul

Background: We have previously shown in a randomised controlled trial that moderate-intensity exercise over the last 20 weeks of gestation in healthy nulliparous women led to a birth weight reduction of approximately 250 g.Objective and hypotheses: We aimed to assess the long-term effects of exercise in pregnancy on anthropometry and body composition in mothers and offspring 7 years after the intervention. We hypothesized that women who exercised in preg...

hrp0086p1-p556 | Perinatal Endocrinology P1 | ESPE2016

Pancreatic Glucagon Secretion is Severely Impaired and Somatostatin Secretion Unchanged in Patients with Hyperinsulinaemic Hypoglycaemia

Shah Pratik , Rahman Sofia , Gilbert Clare , Morgan Kate , Hinchey Louise , Bech Paul , Amin Rakesh , Hussain Khalid

Background: Hyperinsulinaemic hypoglycaemia (HH) is a common cause of hypoglycaemia in children. Glucagon is an important counter-regulatory hormone and the role of somatostatin is not known in children with HH.Objective and hypotheses: To understand the roles of glucagon and somatostatin in children with HH.Method: Children admitted for management of HH in our hospital were included in the study. Plasma insulin, glucagon and somat...