hrp0095fc3.1 | Early Life and Multisystem Endocrinology | ESPE2022

HYPO-CHEAT: Personalised Technology Reduces Real-World Hypoglycaemia by 25%

Worth Chris , Nutter Paul , Salomon-Estebanez Maria , Auckburally Sameera , Banerjee Indraneel , Harper Simon

Background and Aims: Hypoglycaemia is a life-threatening risk for many patients and prevention is individualised and complex. Continuous Glucose Monitoring (CGM) shows promise but current accuracy is insufficient for acute hypoglycaemia detection and data review services are complex and generic. Machine Learning is increasingly used but ignores weekly hypoglycaemia patterns and behaviour change and thus has demonstrated no real-world reduction in hypoglycaemia...

hrp0095rfc6.5 | Sex Development and Gonads | ESPE2022

The effect of GnRHa and testosterone treatment on growth in trans boys

Willemsen Lieve , Boogers Lidewij , Wiepjes Chantal , van Trotsenburg Paul , Klink Daniel , den Heijer Martin , Hannema Sabine

Background: Pubertal growth and adult height are important to many transgender adolescents undergoing medical transition. However, few studies are available on the impact of puberty suppression (PS) with GnRH analogues (GnRHa) and hormonal therapy (HT) with testosterone on growth. In this longitudinal cohort study, we investigated the effect of PS and HT on growth and adult height in transgender boys.Methods: A total of ...

hrp0095p1-493 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2022

Families' Experiences of Continuous Glucose Monitoring in The Management of Congenital Hyperinsulinism: A Thematic Analysis

Auckburally Sameera , Worth Chris , Salomon-Estebanez Maria , Nicholson Jacqueline , Harper Simon , W Nutter Paul , Banerjee Indraneel

Background and Aims: In patients with congenital hyperinsulinism (CHI), recurrent hypoglycaemia can lead to longstanding neurological impairments. At present, glycaemic monitoring is with infrequent fingerprick tests; a practice which can miss hypoglycaemic episodes between tests. Continuous glucose monitoring (CGM) is a promising alternative method which has the utility to identify risk and patterns of hypoglycaemia. Although CGM is well established in type 1...

hrp0095p1-519 | Growth and Syndromes | ESPE2022

Learning outcomes of a MOOC supporting healthcare professionals in treating patients with growth disorders

Dimitri Paul , Fernandez-Luque Luis , Koledova Ekaterina , Malwade Shwetambara , Abdul Shabbir Syed

Background: There is a need to increase digital health literacy in paediatric endocrinology due to the rapid emergence of digital technologies. Massive open online courses (MOOC) provide an opportunity to rapidly increase digital health capabilities at scale, as previously demonstrated in diabetes.1 To our knowledge, there are no comparable examples in the field of growth hormone deficiency.Aim: This study evaluates the ...

hrp0095p1-108 | Growth and Syndromes | ESPE2022

Efficacy, Observer-Reported Outcomes, and Safety of Once-Weekly Somapacitan in Children with Growth Hormone Deficiency (GHD): 4-Year Results from the REAL 3 Trial

Sävendahl Lars , Battelino Tadej , Højby Rasmussen Michael , Brod Meryl , Wai Lee Kai , Saenger Paul , Horikawa Reiko

Children with GHD are currently treated with daily subcutaneous growth hormone (GH) injections, which can be burdensome. Somapacitan is a long-acting GH derivative in development for once-weekly use in children with GHD. REAL 3 (NCT02616562) is a phase 2, multinational, randomised, open label, controlled trial assessing efficacy and safety of somapacitan vs daily GH (Norditropin®). Prepubertal, GH-naïve children with GHD received 0.04 (n=16), 0.08 (n</e...

hrp0092fc7.4 | Diabetes and Insulin Session 2 | ESPE2019

Metabolic Syndrome Features in Pre-Pubertal Children Born After Maternal Pre-Eclampsia

Goffin Sarah , Derraik José , Chiavaroli Valentina , Biggs Janene , Hofman Paul , Groom Katie , Cutfield Wayne

Background: Pre-eclampsia is associated with important complications for both mother and baby in the short term, but there are limited data about its long-term effects on offspring metabolism. Thus, we aimed to assess whether maternal pre-eclampsia was associated with adverse effects on metabolism and body composition in the offspring in childhood.Methods: We studied healthy pre-pubertal children (aged 4–10 years) b...

hrp0092rfc10.4 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology | ESPE2019

qPCR Screening for Xp21.2 Copy Number Variations in Patients with Elusive Aetiology of 46,XY DSD

Meinel Jakob , Dwivedi Gaurav , Holterhus Paul-Martin , Hiort Olaf , Werner Ralf

Duplications of the dosage sensitive sex locus Xp21.2 have been associated with 46,XY gonadal dysgenesis (GD) for nearly 25 years. In the past, duplications have always included the NR0B1 (nuclear receptor subfamily 0 group B, member 1, also known as DAX1) gene, a known antagonist of SF1 (Steroidogenic Factor 1) dependent SOX9 (SRY Box 9) activation and the GD was attributed to its "double gene dose". However, recent findings have questioned the neces...

hrp0092p1-12 | Adrenals and HPA Axis | ESPE2019

Trientine Treatment Mimicking Severe Hyperandrogenism

Binder Gerhard , Weber Karin , Wudy Stefan A. , Holterhus Paul-Martin , Hartleif Stefan

Drugs can interfere with immunoassays causing false measurements. Trientine (triethylene tetramine dihydrochloride) is a chelator of copper and is used in the treatment of patients with Wilson's disease as alternative for penicillamine. Trientine mainly increases urinary copper excretion leading to a negative copper balance. Serum concentrations of trientine reached under treatment are not known. This is the first report of an interference of trientine with two chemilumine...

hrp0092p1-169 | Bone, Growth Plate and Mineral Metabolism (1) | ESPE2019

Hypercalcemia as a Post Stem Cell Transplantation Complication in Children with Osteopetrosis - A Single Centre Experience

Paul Praveen George , N.A Fouzia , Korula Sophy , Mathai Sarah , George Biju , Simon Anna

Introduction: Osteopetrosis (OP) is a rare genetic disorder that is characterized by abnormal osteoclast function resulting in dense bones and marrow failure. The only definitive cure for OP is stem cell transplantation (SCT). Hypercalcemia is a well described complication in children with OP undergoing SCT. This study describes the calcium profile and treatment modalities used to maintain normocalcemia in children with OP undergoing SCT.<p class="abstext"...

hrp0092lb-3 | Late Breaking Posters | ESPE2019

Insights into the Regulation of Androgen Biosynthesis from Males with Congenital Hypogonadotropic Hypogonadism: Quantification of Bioactive Steroid Hormones Reveals Differences Between Gonadotropin Replacement and Testosterone Replacement

Rohayem Julia , Holterhus Paul-Martin , Kliesch Sabine , Nieschlag Eberhard , Zitzmann Michael , Kulle Alexandra

Background: In males with congenital hypogonadotropic hypogonadism (CHH), LH/FSH stimulation of gonads is deficient. In clinical practice, two hormone replacement strategies are employed to induce and maintain virilisation: Treatment with testosterone and gonadotropin replacement with hCG/rFSH.Objective: To delineate the role of gonadotropins in pathways of male androgen biosynthesis and to thereby better define the gona...