hrp0084p3-1106 | Pituitary | ESPE2015

Acquired Long QT Syndrome in a 14-year-old Boy with Panhypopituitarism

Kim Yoo-Mi , Seo Jung-Ho , Cheon Chong Kun , Yim Young-Tak , Kim Su Young

Background: Acquired QT prolongation can be caused by electrolyte abnormality, myocarditis, cerebrovascular disease, drug intoxication and hormonal disorders such as hypopituitarism, hypothyroidism, and adrenal insufficiency.Case presentation: We describe a 14-year-old boy with hypopituitarism after trans-sphenoidal surgery (TSS) due to suprasellar mass who manifested bradycardia and QT prolongation on electrocardiogram. This subject complained of blurre...

hrp0084p3-1155 | Puberty | ESPE2015

Familial Precocious Puberty: Clinical Characteristics and GnRH Agonist Response

Jeong Hwalrim , Kwon Eun Byul , Lee Hae Sang , Lim Jung Sub , Hwang Jin Soon

Background: Familial precocious puberty is defined by the existence of more than one affected member either in the proband generation or in the pedigree. Recently, several gene mutation cause familial CPP is elucidated, gain of function mutations in KISS1 and KISS1R, loss of function mutations in the MKRN3, the feature of familial precocious puberty is not fully understood.Objective: To investigate the clinical characteristics of familial precocious pube...

hrp0097p1-463 | Fat, Metabolism and Obesity | ESPE2023

Association between lean mass and metabolic syndrome risk in Korean children and adolescents

Suk Shim Young , Sang Lee Hae , Rim Jeong Hwal , Sub Lim Jung , Soon Hwang Jin

Introduction: Skeletal muscle plays a crucial role in glucose disposal, and studies have shown a positive relationship between muscle mass and insulin sensitivity. However, an elevated lean mass has also been associated with metabolically adverse outcomes. This study aimed to evaluate the association between the risk of metabolic syndrome (MetS) and lean mass using dual-energy X-ray absorptiometry (DXA) in a nationally representative sample from the Korea Nati...

hrp0097p1-498 | GH and IGFs | ESPE2023

The relationship between body mass index and therapeutic effect in children with idiopathic growth hormone deficiency

Sang Lee Hae , Suk Shim Young , Soon Hwang Jin , Sub Lim Jung

Purpose: The aim of this study is to investigate the influence of body mass index (BMI) on therapeutic effect in prepubertal children with idiopathic growth hormone deficiency (IGHD).Methods: We conducted a retrospective study by chart review in a single center. A total of 138 patients (male n=90, female n=48) with idiopathic growth hormone deficiency who were was treated growth hormone for at least 2 y...

hrp0097p1-353 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

Clinical features of hyperprolactinemia in children and adolescents

Sang Lee Hae , Suk Shim Young , Sub Lim Jung , Soon Hwang Jin

Background/Purpose: Hyperprolactinemia is a rare endocrine disorder in childhood and there are limited etiological, clinical, and demographic data. The purpose of this study was to evaluate the clinical features and course of hyperprolactinemia in childhood and adolescents and to help diagnose and plan the management.Methods: In this single-center retrospective study included 21 patients with hyperprolactinemia from Ajou...

hrp0097p1-394 | Thyroid | ESPE2023

Assessment of five domains of neurodevelopment and growth in congenital hypothyroidism: Serial 6-year follow-up study of 408 patients

Kyung Jung Mo , Kyo Ha Eun , Ryung Cha Hey , Yong Han Man

Background: There is a link between congenital hypothyroidism (CH) and neurodevelopmental abnormalities, but no longitudinal studies have yet identified reliable quantifiable measures.Purpose: To evaluate associations between CH and abnormalities in neurodevelopment and growth, and identify the timing of these abnormalities and the best time for intervention.Methods: Data from the ...

hrp0095p1-88 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2022

An Ongoing Phase 2 Study of HM15136, a Novel Long-acting Glucagon Analogue, in Subjects with Congenital Hyperinsulinism (ACHIEVE, Outline Protocol)

Shin Wonjung , Kang Jiyeon , Lee Grace , Maria Arbelaez Ana , Banerjee Indi , Dastamani Antonia , Mohnike Klaus , Okawa Erin , Cho Hyungjin , D De León Diva

Congenital hyperinsulinism (CHI) is an ultra-rare disease characterized by excessive insulin secretion that results in persistent hypoglycemia. If left untreated, CHI-induced severe prolonged hypoglycemia may lead to permanent neurologic damage. Currently used pharmacologic agents fail to prevent hypoglycemia in a subset of patients with CHI. HM15136 is a novel long-acting glucagon analogue that have demonstrated good stability and extended half-life ranging from 77 to 167 hou...

hrp0086fc7.5 | Gonads & DSD | ESPE2016

Targeted Exome Sequencing for Genetic Diagnosis of Patients with Disorders of Sex Development

Kim Ja Hye , Kang Eungu , Kim Gu-Hwan , Jang Ja-Hyun , Cho Eun-Hae , Lee Beom Hee , Yoo Han-Wook , Choi Jin-Ho

Patients with Disorders of sex development (DSD) can present with a large phenotypic spectrum and caused by a number of different genetic defects. Therefore, it is difficult to reach a specific diagnosis using traditional approaches including biochemical analysis and single gene sequencing in a number of patients with DSD. Recently, next-generation sequencing technologies have revolutionized the identification of causative genes with diseases with genetic heterogeneity using m...

hrp0097p1-67 | Fat, Metabolism and Obesity | ESPE2023

Percentage of Appendicular Skeletal Muscle Mass Reference and Association with Metabolic Syndrome in Korean Adolescents

Hye Lee Da , Kang Sung-Chan , Hwang Seung-sik , Jeong Lee Yun , Young Kim Hwa , Yong Lee Seong , Ho Shin Choong , Kim Jaehyun

Purpose: The association between appendicular skeletal muscle mass (ASM) and cardiometabolic risk has been emphasized. We estimated reference values of the percentage of ASM (PASM) and investigated its association with metabolic syndrome (MS) in Korean adolescents.Methods: Data from Korea National Health and Nutrition Examination Survey performed between 2009 and 2011 was used. Tables and graphs of reference PASM were ge...

hrp0082p1-d2-245 | Thyroid (1) | ESPE2014

Thyroid Dysfunction in Children After Hematopoietic Stem Cell Transplantation: Short Term Follow-Up for 12 Months

Jeon Yeon Jin , Jung In Ah , Kim Shin Hee , Cho Won-Kyoung , Lee Jae-Wook , Cho Kyoung Soon , Park So Hyun , Chung Nak-Gyun , Jung Min-Ho , Cho Bin , Suh Byung-Kyu

Background: We evaluated 12 months follow-up of thyroid function in patients who underwent hematopoietic stem cell transplantation (HSCT) during childhood and adolescents.Methods: We studied 83 hematologic-malignancy patients (46 boys and 37 girls, acute lymphoblastic leukemia=25, acute myeloid leukemia=51, chronic myelogenous leukemia=7) who underwent HSCT between January 2006 and December 2011.The mean age at HSCT was 9.78±4...