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58th Annual ESPE

Vienna, Austria
19 Sep 2019 - 21 Sep 2019

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The theme of this year’s meeting is Variety and Variation in Paediatric Endocrinology. Join us in Vienna to explore the diversity we encounter in our discipline and the care that we have to exercise when using the term “normality”. Whilst gaining an update on the latest treatments, clinical best practice and cutting edge research in the field of paediatric endocrinology.

Poster Category 2

Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia)

hrp0092p2-143 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Two Siblings with Tyrosinaemia Type 1 and Transient Hyperinsulinaemic Hypoglycaemia

Sotiridou Ellada , Aftab Sommayya , Dastamani Antonia , Doodson Louise , Batzios Spyros , Shah Pratik

Introduction: Tyrosinaemia type 1 (TT1) is a rare autosomal recessively inherited disorder of tyrosine metabolism leading to accumulation of tyrosine and its metabolites in liver, kidney and central nervous system. TT1 is a heterogeneous disorder with a broad spectrum of clinical manifestations. Hypoglycaemia is common, especially in the acute phase of the disease due to liver failure and reduced hepatic clearance of insulin. However, confirmed cases of hyperi...

hrp0092p2-144 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Glucagon Therapy In Preterm Infants With Hyperinsulinemic Hypoglycaemia

Salimi Dafsari Roschan , Roper Marcia , Groß Maximilian , Wiechers Cornelia , Sabir Hemmen , Mayatepek Ertan , Meissner Thomas

Background: The treatment of preterms with hyperinsulinemic hypoglycaemia is a well-known challenge. One of the difficulties of the therapy is the excessive application of intravenous fluids to compensate high carbohydrate needs. There are various drug alternatives such as glucagon, diazoxide or somatostatin analogues apart from intravenous glucose application.Hypothesis: Intravenous or continuous subcutaneous glucagon t...

hrp0092p2-145 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Neonatal Hypo-ketotic Hypoglycemia Secondary to Transient Hyperinsulinism. Diazoxide Responsiveness and Experience with Fasting Test after Treatment Withdrawal

Salamanca Luis , Itza Nerea , Mora Cristina , Dominguez Jesús , de Pipaón Miguel Sáez , Campos Angel , González Isabel

Introduction: Transient hyperinsulinism is described in neonates with stress factors (intrauterine growth restriction (IUGR), large for gestational age (LGA), perinatal asphyxia, infants of diabetic mother etc.). Recognition and early treatment is prioritary to avoid neurological morbidity related with recurrent hypoglycemia.Objective: Describe the incidence of transient hyperinsulinism. Clinical charac...

hrp0092p2-146 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Severe Neonatal Hyperparathyroidism Due to a Novel Homozygous Mutation of the Calcium-Sensing Receptor (CaSR)

hacohen solovitz amir , Tenenbaum-Rakover Yardena , Spiegel Ronen , Weinberger Jeffrey , Gillis David , Goor Zamir Gershon , Levine Michael A. , Almagor Tal

Homozygous loss-of-function mutations of the calcium-sensing receptor gene (CaSR) are associated with neonatal severe hyperparathyroidism (NSHPT), a life-threatening condition with a challenging treatment approach.We report a 7-day-old-female infant who was admitted to our Pediatric Department due to poor sucking. On examination she was lethargic and hypotonic. Laboratory evaluation revealed extreme hypercalcemia of 23.54 mg/dL (N: 7.6–10.4...

hrp0092p2-147 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

A Rare Cause of Pediatric Hypoglycemia in a Boy: A Malignat Insulinoma

Valenzise Mariella , Zirilli Giuseppina , Cannavo' Laura , Passanisi Stefano , Li Pomi Alessandra , Wasniewska Malgorzata

Insulinoma is a rare neuroendocrine tumor, usually benign, but can be life-threatening in causing hypoglycemic accidents. It presents in individuals aged between 8 and 82 years and can occur sporadically or constitute a part of multiple endocrine neoplasia type 1 (MEN-1).The nonspecific-symptoms and small size of these tumors led to difficulties of diagnosis and localization.Here we describe the case of a 11-year-old boy, who came to our attention for a ...

hrp0092p2-148 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Neonatal Hyperglycemia

Bochkova Larisa , Gumeniuk Olga

Hyperglycemia in newborns is most common in premature infants. At present, in clinical practice, the attitude towards this type of metabolic disorders is not well defined and controversial.Objective: To determine the feasibility of prescribing insulin for hyperglycemia in premature newborns.Patients and Methods: We observed 68 newborns with a birth weight of 1326 ± 119.8 g and a gestational ag...

hrp0092p2-149 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Clinical and Genetic Characteristics of Patients with Hyperinsulinaemic Hypoglycaemia Diagnosed and Treated at a Tertiary Endocrine Center, a part of the ENDO-ERN

Galcheva Sonya , Iotova Violeta , Ellard Sian , Chuperkova Jivka , Bazdarska Yuliya , Bocheva Yana , Flanagan Sarah E.

Background: Hyperinsulinaemic hypoglycaemia (HH) is a clinically and genetically heterogeneous group of disorders characterized by persistent hypoglycaemia due to inappropriate insulin secretion from the pancreatic β-cell.Aim: The objective was to analyze the demographic, clinical and genetic characteristics of patients with HH, diagnosed and/or treated at a tertiary endocrine center, part of the European referen...

hrp0092p2-150 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Relations of O2 Supplementation to Blood Serum Insulin-like Growth Factor-II / Insulin-like Growth Factor-binding Protein-3 Ratios in the Not-life-threatened Human Newborn; Role of Oral-enteral Caloric Intake Beyond Axillary Temperature

Terzi Cesare , Virdis Raffaele , Magnani Cristiana , Tridenti Gabriele , Cerioli Andrea , Riani Marco , Chesi Elena , Bernasconi Sergio , De Angelis Gian Luigi , Blum Werner F. , Banchini Giacomo

Introduction: Oral/enteral caloric intake (KOE) insufficiency may accompany human newborn (NWB) respiratory derangements. We detected axillary temperature(TEMP) relations to NWB blood serum Insulin-like Growth Factor-II(IG2)-blood serum Insulin-like Growth Factor-Binding Protein-3(IB3) ratios(IG2/IB3R). Here we evaluate the TEMP-independent relevance of birth gestational age(GA) and KOE to relationships of O2 supplementation in respiratory gases(O2S) with IG2/...

hrp0092p2-151 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Refractory Hyperinsulinaemic Hypoglycaemia in Beckwith-Wiedemann Syndrome due to Imprinting Centre 1 Gain of Methylation: Severity Discordant to Genotype

Conwell Louise , McBride Craig , Choo Kelvin , Tadgell Shawn , Fuery Michelle , Davies Janene

Introduction: Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome caused by multiple epigenetic/genetic changes affecting imprinted genes in 11p15.5 region. Phenotypic expression is variable. Hyperinsulinaemic hypoglycaemia is common (30-60%). Persistent, severe, refractory cases are usually associated with 11p15 paternal uniparental disomy, particularly the rare context of a coexisting paternal inactivating KATP channel variant. Those c...

hrp0092p2-152 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Congenital Hyperinsulinism due to Compound Heterozygous Mutations in ABCC8 Fully Responsive to Diazoxide Therapy

Taylor-Miller Tashunka , Deshpande Ruma , Burren Christine P , Munyard Paul , Giri Dinesh

Background: Congenital Hyperinsulinism (CHI), a condition characterised by dysregulation of insulin secretion from the pancreatic beta cells, remains one of the most common causes of hyperinsulinemic, hypoketotic hypoglycaemia in the newborn period. Mutations in ABCC8 and KCNJ11 constitute the majority of genetic forms of CHI. Biallelic inactivating mutations (homozygous or compound heterozygous) in ABCC8 and KCNJ11 are know...