hrp0082p2-d3-440 | Growth Hormone (2) | ESPE2014

Two-Year Data from a Long-Term, Phase IV Study of Omnitrope®, a Recombinant Human GH, in Short Children Born Small for Gestational Age

Schwarz Hans-Peter , Birkholz-Walerzak Dorota , Szalecki Mieczyslaw , Walczak Mieczyslaw , Galesanu Corina , Metreveli David , Khan-Boluki Jasmin , Schuck Ellen

Background: Children born small for gestational age (SGA) are predisposed to metabolic abnormalities. While the therapeutic benefit of recombinant human growth hormone (rhGH) therapy in improving height is widely recognised, it can affect carbohydrate metabolism, potentially inducing diabetes.Objective and hypotheses: This ongoing, prospective study aims to evaluate the long-term safety and efficacy of Omnitrope® (somatropin) in children ...

hrp0082p2-d3-500 | Perinatal and Neonatal Endocrinology | ESPE2014

The Contribution of Maternal Malaria Exposure and Metabolic Markers to Change in Blood Pressure in Nigerian Children over the First 3 Years of Life

Farikullah-Mirza Jasmin , Whatmore Andrew , Ayoola Omolola , Omotade Olayemi , Butcher Imogen , Soran Handrean , Cruickshank Kennedy , Clayton Peter

Background: In Nigeria, where malaria is endemic, hypertension is common. We reported that exposure to maternal malaria resulted in smaller babies with lower BP at birth, but a greater change (Δ) in BP to 12 months of age.Objective and hypotheses: To now present BP measurements out to 3 years of age.Method: Height, weight, and blood pressure (BP) were measured on 164 babies (75 males and 89 females) at birth, 12, 24, and 36 mo...

hrp0084p1-118 | Puberty | ESPE2015

Specific Hypothalamic Activation Pattern by mGlu5 Receptor Blockade in vivo During Pubertal Development in Female Mice

Inta Ioana , de Angelis Roberto , Lankisch Katja , Dormann Christof , Pfeiffer Natascha , Boehm Ulrich , Gass Peter , Bettendorf Markus

Background: Puberty is characterised by important changes of brain networks. The glutamate system plays a main role in modulating the onset of puberty as shown for NMDA receptor agonists. However, the underlying mechanisms are poorly understood. Metabotropic mGlu5 receptors (mGluR5) are tightly linked to NMDA receptors. The effect of mGluR5 blockade on neurohormonal mechanisms in puberty initiation were not studied yet.Objective and hypotheses: To invest...

hrp0084p2-223 | Bone | ESPE2015

Refractory Hypercalcaemia of Malignancy: Responsiveness to Denosumab and Zoledronate

Giri Dinesh , Ramakrishnan Renuka , Hayden James , Brook Lynda , Das Urmi , Mughal M Zulf , Selby Peter , Dharmaraj Poonam , Senniappan Senthil

Background: Hypercalcaemia secondary to malignancy is rare in children and adolescents. Parathyroid hormone related peptide (PTH-rP) secreted by malignant cells increases bone resorption and renal calcium retention causing hypercalcaemia. We report 2 cases of hypercalcaemia of malignancy refractory to treatment with pamidronate and corticosteroids but responsive to treatment with Denosumab and Zoledronic acid.Case 1: A 17-year-old boy with epidermolysis ...

hrp0084p2-391 | Fat | ESPE2015

POMC DNA Hypermethylation Variant is Highly Associated with Obesity in Adults

Kuhnen Peter , Handke Daniela , Spranger Jochen , Fischer-Rosinsky Antje , Hinney Anke , Hebebrand Johannes , Gruters Annette , Krude Heiko

Background: POMC plays a major role in central body weight regulation. Recently we have shown that a POMC hypermethylation variant is significantly associated with obesity in children and adolescents (Kuehnen et al. PLoS Genetics 2012).Objective and hypotheses: Here we report about our extended studies to elucidate the mechanism behind the occurrence of the POMC hypermethylation variant in obese individuals.<p clas...

hrp0084p2-401 | GH &amp; IGF | ESPE2015

GRB10 Knockdown in Zebrafish is Associated with Decreased Weight-to-length Ratio without Alterations in AKT and ERK Activity: A Model to Study Human Growth Regulation

De Leonibus Chiara , Broadbent Jack , Murray Philip , Whitehead Joseph , Hurlstone Adam , Shiels Holly , Clayton Peter , Stevens Adam

Background: In humans GRB10 negatively regulates GH and IGF1 signaling predominantly via altering phosphorylation of PI3K/mTOR/AKT and MEK/ERK pathways which relate to both growth and metabolic function. We have previously shown that Grb10 knockdown in Zebrafish results in overgrowth with an increase in length and head size. However the impact on weight in relation to length has not been assessed.Objective: To develop a model t...

hrp0094fc1.4 | Adrenal | ESPE2021

Acute Illness and Death in Children with Adrenal Insufficiency

Worth Chris , Vyas Avni , Banerjee Indraneel , Lin John , Jones Julie , Stokes Helen , Komlosy Nicci , Ball Steve , Clayton Peter ,

Background: Adrenal Insufficiency (AI) is a heterogeneous diagnosis comprising multiple central and peripheral causes. The unifying feature is the requirement for glucocorticoid replacement and subsequent risk of life-threatening Adrenal Crisis (AC) and Adrenal Death (AD). Very few studies have investigated rates of AD in children. Multiple studies have reported symptoms of children with AC as interpreted by medical staff but no studies have looked at parental...

hrp0094p1-101 | Adrenal B | ESPE2021

The management of adrenal cell carcinoma in a single tertiary centre: 25 year experience

Goff Nicole , Hughes Claire , Katugampola Harshini , Mushtaq Imran , Hindmarsh Peter , Peters Catherine , Brain Caroline , Jorgensen Mette , Dattani Mehul ,

Background: Adrenal cortical carcinoma (ACC) in children is rare and aggressive, with the mainstay of treatment being surgical resection, although there have been recent improvements in outcomes with chemotherapy. Further characterisation of the presenting features and biochemical markers are needed to support earlier diagnosis. Refractory hypertension related to high cortisol concentrations prior to surgery, and post-operative decrease in cortisol can be chal...

hrp0094p1-179 | Growth Hormone and IGFs B | ESPE2021

Novel dominant negative GH receptor variants provide important insights into GH receptor physiology

Andrews Afiya , Cottrell Emily , Maharaj Avinaash , Ladha Tasneem , Williams Jack , Metherell Louise A , McCormick Peter J , Storr Helen L ,

Background: Growth hormone insensitivity (GHI) encompasses normal/elevated growth hormone (GH), low IGF-I levels and growth restriction. Non-classical/mild-moderate GHI is an emerging entity which is poorly characterised, and, in many subjects, the underlying cause is unclear. Heterozygous dominant negative (DN) variants located in the intracellular/transmembrane domain of the GH receptor (GHR) cause a ‘non-classical’ GHI phenotype.<p class="abst...

hrp0097p1-4 | Adrenals and HPA Axis | ESPE2023

Major importance of genetic background in cortisol metabolism: Salivary diurnal glucocorticoid profiles in monozygotic twins with intra-twin birthweight-differences

Schulte Sandra , Eberhardt Nora , Roedig Thea , Schreiner Felix , Plamper Michaela , Bartmann Peter , Holterhus Paul-Martin , Kulle Alexandra , Gohlke Bettina

Objective: Although low birthweight (bw) and unfavourable intrauterine conditions have been associated with metabolic sequelae in later life, little is known about their impact on glucocorticoid metabolism. We studied monozygotic twins with intra-twin bw-differences from birth to adolescence to analyse the long-term impact of bw and catch-up growth on glucocorticoid metabolism.Methods: 46 monozygotic prepubertal twin-pai...