hrp0092fc5.3 | Thyroid | ESPE2019

Predominant DICER1 Mutations in Pediatric Follicular Thyroid Carcinomas

Ah Lee Young , Im Sun-Wha , Cheon Jung Kyeong , Jae Chung Eun , Ho Shin Choong , Kim Jong-II , Park Young Joo

Background: Pediatric thyroid cancer has characteristics that are distinct from adulthood thyroid cancer. Due to its very low prevalence, little is known about the genetic characteristics of pediatric follicular thyroid cancer (FTC).Methods: We investigated genetic alterations in tumor tissues from 15 patients aged < 20 years (median: 14.3 years; range: 2.4-19.0 years) using multifaceted approaches. Whole-exome seque...

hrp0092t10 | Top 20 Poster | ESPE2019

Changes in Adrenal Androgens and Steroidogenic Enzyme Activities in Children Aged 2, 4, and 6 Years: Steroid Hormone Profiling From the Prospective Cohort Study

Hyun Kim Jae , Ah Lee Young , Ho Shin Choong , Yang Sei Won , Song Junghan , Lim Youn-Hee , Kim Bung-Nyun , Inhyang Kim Johanna , Hong Yun-Chul

Introduction: Adrenarche refers to the increase in adrenal androgen synthesis. However, process of adrenal androgen production in early childhood remains to be elucidated. The aim of this study was to evaluate changes in adrenal androgen levels and steroidogenic enzyme activities associated with adrenarche using a prospective cohort.Methods: A total of 229 children (124 boys, 52.4%), who had participated in the Envir...

hrp0092p1-44 | Fat, Metabolism and Obesity | ESPE2019

Metabolic Complications After Paediatric Liver Transplantation: A 10-year Longitudinal Study in a South-East Asian Population

Beng Hui Ng Nicholas , Yijuan Lim Yvonne , Wei Li Ho Cindy , Anjian Sng Andrew , Aw Marion , Seng Lee Yung , Yin Loke Kah

Background: Improved patient and graft survival post-liver transplantation has led to a parallel increase in metabolic syndrome (MS) reported in multiple centres. We aimed to study the prevalence and risk factors of metabolic complications in our paediatric liver transplant (LT) cohort.Methods: This was a retrospective review of the LT database from 1995-2018. We studied the incidence of overweight, obesity (WHO BMI crit...

hrp0092p1-65 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Congenital Hyperinsulinism Due to Pancreatic Mosaicism for Paternal Uniparental Disomy of all Chromosome 11, with the Additional Finding of Pancreatic Mosaicism for Trisomy 12

Conwell Louise , Harraway James , Williams Mark , Joy Christopher , Scurry Bonnie , Lee Kevin , McBride Craig , Choo Kelvin , Huynh Tony , Ng Carolyn , Flanagan Sarah

We report a term male with diazoxide-unresponsive congenital hyperinsulinism (CHI) (spontaneous conception, non-consanguineous, no family history). The patient did not have macroglossia, exomphalos or lateralised overgrowth (cardinal Beckwith-Wiedemann spectrum (BWSp) features) (1). There was no polyhydramnios, macrosomia, facial naevus simplex, ear creases/pits, diastasis recti or nephromegaly/hepatomegaly (suggestive BWSp features) (1).A targeted massi...

hrp0092p1-431 | Thyroid (2) | ESPE2019

The Relationship Between Perfluoroalkyl Compounds Concentrations at Ages 2, 4, and 6 Years and Thyroid Function in Early Childhood: A Prospective Cohort Study

Young Kim Hwa , Kim Kyoung-Nam , Ah Lee Young , Lim Youn-Hee , Inhyang Kim Johanna , Kim Bung-Nyun , Oh Se-Young , Hong Yun-Chul , Ho Shin Choong

Backgrounds: Perfluoroalkyl compounds (PFCs) have been suggested as potential thyroid disrupting chemicals. However, previous studies about the associations between PFCs and childhood thyroid function are scarce, and inconclusive. We evaluated the PFC exposure in Korean preschool children, and investigated the temporal relationship with thyroid hormone concentration.Methods: From a prospective the Environment and Develop...

hrp0092lb-23 | Late Breaking Posters | ESPE2019

Long-Term Safety and Effectiveness of Recombinant Human Growth Hormone in Korean Pediatric Patients with Growth Disorders: 7-year Interim Analysis from LG Growth Study

Ah Lee Young , Chung Sochung , Rhie Young-Jun , Hyun Kim Jae , Chae Hyun-Wook , Yoo Jae-ho , Ho Choi Jin , Tae Hwang Il

Objectives: This study aimed to evaluate the long-term safety and effectiveness of recombinant human growth hormone (rhGH) (Eutropin® Inj.., Eutropin®Pen Inj., Eutropin®AQ Inj., and Eutropin®Plus Inj., LG Chem, Ltd.) based on the interim analysis of a 7-year accumulated data of the LG Growth Study (LGS) in Korean pediatric patients with growth disorders including growth hormone deficiency (GHD), T...

hrp0092p2-85 | Diabetes and Insulin | ESPE2019

Clinical Characteristics and Treatment Outcomes in Patients with Autoantibody-negative Ketosis-prone Diabetes

Kim Shin-Hee , Ahn Moon-Bae , Kim Seulki , Choi Yujung , Lee Seonhwa , Cho Won Kyoung , Cho Kyoung Soon , Jung Min Ho , Kyu Suh Byung

Objective: Ketosis-prone diabetes (KPD), characterized by presentation with diabetic ketoacidosis (DKA) in patients lacking the typical features of autoimmune type 1 diabetes, is a heterogeneous syndrome. The objective of this study is to ascertain the presence of A-β+ (autoantibody-negative, β-cell functional reserve) KPD in Korean children and adolescents and to study their characteristics in this group. Method: Diabetes ...

hrp0089p1-p015 | Adrenals and HPA Axis P1 | ESPE2018

New Insights into Low Dose Dexamethasone Suppression Test in Paediatric Cushing’s Syndrome

Wilkinson Ingrid C E , Martin Lee , Grossman Ashley B , Monson John P , Akker Scott , Savage Martin O , Drake William M , Storr Helen L

Background: The Low dose dexamethasone suppression test (LDDST) is an important investigation for suspected Cushing’s Syndrome (CS). The traditional definition of normal suppression of serum cortisol to ≤50 nmol/l during the LDDST (0.5 mg 6 hrly × 48 h) comes from a time when biochemical autoanalysers did not routinely detect very low values. Previous studies reported 5.1–8.3% of patients with Cushing’s Disease (CD) suppressed to <50 nmol/l at 48 ...

hrp0089p1-p063 | Diabetes &amp; Insulin P1 | ESPE2018

Glucose Intolerance in Survivors of Childhood Hematologic Disorders

Lee Seonhwa , Choi Yujung , Kim Seul ki , Ahn Moonbae , Chun Min-Kyo , Kim Shinhee , Cho Wonkyoung , Cho Kyoungsoon , Jung Minho , Suh Byungkyu

Purpose: To investigate overall characteristics of glucose intolerance in childhood survivors of hematologic diseases and suggest risk factors which increase A1c (glycated hemoglobin) level.Methods: Based on a retrospective review of 394 children who were diagnosed with acute leukemia or aplastic anemia between 2015 and 2016 under the age of 15, glucose intolerance was observed in 14 patients. A definition of glucose intolerance was A1c above 5.7%. Auxol...

hrp0089p3-p201 | GH &amp; IGFs P3 | ESPE2018

Effects on Near-adult Height and Safety of Recombinant Human Growth Hormone in Growth Hormone Deficiency and Turner Syndrome Patients: Results from the LG Growth Study

Choi Jin-Ho , Chung Sochung , Rhee Young-Jun , Hyun Kim Jae , Chae Hyun-Wook , Yoo Jae-ho , Lee Young Ah , Hwang Il Tae

Objectives: This study was performed to evaluate effectiveness on near-adult height (NAH) and safety of recombinant human growth hormone (rhGH) (Eutropin® Inj., Eutropin®Plus Inj., Eutropin®AQ Inj., LG Chem, Ltd.) treatment in children with growth hormone deficiency (GHD) and Turner syndrome (TS).Methods: The LG Growth Study (LGS) is a multicenter, long-term, observational study designed to evaluate the lon...