hrp0094p1-77 | Fetal Endocrinology and Multisystem Disorders A | ESPE2021

Real-Time Continuous Glucose Monitoring in the Management of Neonates with Persistent Hypoglycemia

Win Myat , Beardsall Kathy , Thankamony Ajay , Beckett Rowan ,

Background: Persistent hypoglycaemia is common in the newborn, due to prematurity or congenital hyperinsulinism (CHI) and is associated with the risk of poor neurodevelopmental outcome. Adequate monitoring is critical in prevention but is dependent on frequent blood sampling. Continuous glucose monitoring (CGM) is increasingly being used in children with type 1 diabetes mellitus who also are at risk from hypoglycemia, but use in neonatology remains limited. Pr...

hrp0089p2-p116 | Diabetes & Insulin P2 | ESPE2018

Effect of a Reduced Fluid Replacement Regimen on the Resolution of Diabetic Ketoacidosis (DKA) in Children

Hapuarachchi Danica Shanee , Ahmed Jaberuzzaman , Gevers Evelien , Moodambail Abdul , Thankamony Ajay

Background: A substantially reduced fluid replacement regimen was introduced in the ‘New’ British Society of Paediatric Endocrinology Diabetes (2015) compared to ‘Old’(2009) guideline for DKA management. However, data on varying fluid replacement regimens is limited and we explored this by comparing outcomes of the 2 guidelines on the resolution of DKA.Methods: In a retrospective audit of consecutively admitted patients (age <18 y...

hrp0086rfc4.3 | Pathophysiology of Obesity | ESPE2016

Early Growth Patterns are Associated with Alterations in Adipocytokine Levels and Fat Distribution Measured by DXA in 982 Children/Adolescents

Tinggaard Jeanette , Thankamony Ajay , Jensen Rikke B , Main Katharina M , Juul Anders

Background: Early growth trajectories are associated with childhood BMI and fat distribution as well as adulthood type 2 diabetes. Mechanisms by which early growth determines later adiposity remain unclear, but the effect may be mediated through adipocytokines.Objective and hypotheses: We describe the association between infant growth, adolescent fat distribution and serum adipocytokines. We hypothesize that poor or rapid early growth is associated with ...

hrp0082fc11.4 | Pituitary | ESPE2014

Management of Hyperhydration in a Child with Syndrome of Inappropriate Antidiuretic Hormone Secretion (Siadh) Using a Selective Vasopresin Receptor Agonist

Delgado-Carballar Violeta , Thankamony Ajay , Willemsen Ruben , Elleri Daniela , Dunger David

Background: Management of SIADH is challenging and no optimal therapies are available in children. We present a case of an 11 years old boy with severe SIADH resistant to 30% fluid restriction in the context of an intracranial suprasellar high grade B-cell lymphoma who requires hyperhydration for chemotherapy protocol. Tolvaptan is an oral highly selective arginine vasopressin V2 receptor antagonist, which has been approved for use in SIADH in adults.Obj...

hrp0082p1-d3-94 | Sex Development | ESPE2014

Prenatal Exposure to Phthalates and Phenols in Relation to Anogenital Distance at Birth in Male Infants

Fisher Benjamin G , Thankamony Ajay , Ong Ken K , Dunger David B , Hughes Ieuan A , Acerini Carlo L

Introduction: Increasing incidence of male reproductive disorders may be due to fetal exposure to putative endocrine disruptor chemicals (EDCs), such as phthalates and phenols. Anogenital Distance (AGD) is a biomarker of fetal androgen action in animals, and has recently been linked to testicular dysgenesis syndrome in humans.Objective: To examine the relationship between prenatal phthalate and phenol exposure and birth AGD in male infants.<p class="...

hrp0082p2-d1-572 | Sex Development | ESPE2014

Anogenital Distance, Penis Growth, and Masculine Behaviour Evidence for Independent Neurobehavioral Effects of Foetal Versus Postnatal Androgen Exposure in Boys

Pasterski Vickie , Acerini Carlo , Dunger David , Ong Ken , Hughes Ieuan , Thankamony Ajay , Hines Melissa

Background: Associations between foetal androgen deficiency and variations in anogenital distance (AGD) suggest that AGD is a reliable indicator of foetal androgen exposure. Similarly, variation in postnatal penis growth associated with variations in testosterone show penis growth to be a potential biomarker of early postnatal androgen exposure. Though variation in early androgen exposure is also hypothesized to underlie neurobehavioral masculinisation, until now, no reports h...

hrp0089p2-p342 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P2 | ESPE2018

In-silico Gene-protein Analysis and Clinical Phenotype Characterisation of Three Novel NR5A1/SF1 Gene Mutations Presenting with 46,XY DSD

Cuccaro Rieko Tadokoro , Thankamony Ajay , Hendriks A Emile J , Alvi Sabah , Armstrong Ruth , Bruty Jonathan , A Hughes Ieuan , L Acerini Carlo

Background: Disorders of sex development (DSD) due to mutations in the NR5A1 (SF1) gene result in a highly variable phenotype.Objective: To report the clinical phenotype and the molecular/structural characteristics of the gene-protein product arising from three novel mutations of the NR5A1 (SF1) gene found in patients presenting with 46,XY DSD.Method: Phenotype determined from interrogation of clinical case notes. Interpre...

hrp0086p1-p347 | Gonads &amp; DSD P1 | ESPE2016

The International AGD Consortium: A Multi-center Study of 3939 Infants and Children with Anogenital Distance Measurements

Lindhardt Johansen Marie , Thankamony Ajay , Acerini Carlo , Kold Jensen Tina , Main Katharina M. , Holm Petersen Jorgen , Swan Shanna , Juul Anders

Background: The anogenital distance (AGD) is considered a valid marker of altered androgen action in utero. Recently, reduced AGD has been shown in males with hypospadias, cryptorchidism, reduced semen quality and infertility. It has also been proposed as a new marker to be used by clinicians when managing patients with Disorders of Sex Development. However, little normative data exist and measurement methods vary.Objective and hypotheses: We established...

hrp0092rfc14.5 | Adrenals and HP Axis | ESPE2019

Bioactive IGF-I Concentration Compared to Total IGF-I Concentration Before and After 1 Year of High-Dose Growth Hormone in Short Children Born Small for Gestational Age - North European SGA Study (NESGAS)

Beck Jensen Rikke , Gersel Wegmann Mathilde , Thankamony Ajay , Roche Edna , Hoey Hilary , Kirk Jeremy , Ivarsson Sten-A. , Söder Olle , Frystyk Jan , Dunger David B. , Juul Anders

Background: Children born small for gestational age (SGA) exhibit wide variations in the activity of growth hormone (GH)/insulin–like growth factor–I (IGF-I) axis and this heterogeneity may result in supra physiological concentrations of IGF-I during GH treatment. The long-term effects of elevated IGF-I levels has been a matter of concern. We explored the variations in total IGF-I and bioactive IGF-I and the associations with growth and glucose metab...

hrp0086p1-p604 | Growth P1 | ESPE2016

The Exon3-Deleted Growth Hormone Receptor Gene Polymorphism (d3-GHR) is Associated with Increased Spontaneous Growth and Impaired Insulin Sensitivity in Prepubertal Short SGA Children (NESGAS)

Wegmann Mathilde Gersel , Jensen Rikke Beck , Thankamony Ajay , Kirk Jeremy , Donaldson Malcolm , Ivarsson Sten-A , Soder Olle , Roche Edna , Hoey Hillary , Dunger David B , Juul Anders

Background: A common polymorphism in the growth hormone receptor gene (d3-GHR) was found to be associated with pre- and postnatal growth and GH-induced growth. D3-GHR was associated with glucose metabolism in adults with GHD and acromegaly, but this has not previously been explored in children.Objective and hypotheses: We examined the impact of the GHR-exon-3 deletion on glucose metabolism and anthropometrics in short SGA children before and following 1 ...