hrp0084p2-450 | Growth | ESPE2015

Pharmacokinetics and Efficacy of a Long-Acting Human GH with Fc Fusion Protein

Kim Sujin , Jin Dong-Kyu , Cho Sungyoon , Huh Rimm , Kim Jinsup , Yang Aram , Kwak Hyunhee

Background: Recombinant human GH (rhGH) therapy requires daily s.c. injections, this inconvenient treatment regimen results to poor compliance of the patient. Thus, to improve patient compliance, long-acting rhGH products including various protein fusion techniques have been in development during past 15 years.Objective and hypotheses: In this study, we describe the pharmacokinetics and efficacy of a novel long-acting GH using Fc fusion protein (rhGH-Fc)...

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...

hrp0084p3-1246 | Turner | ESPE2015

Turner Syndrome with Breast Development: Case Report

Park Sung Won , Cho Sung Yoon , Kim Jinsup , Huh Rimm , Kim Su Jin , Jin Dong-kyu

Introduction: Turner syndrome (TS) is caused by monosomy or structural abnormalities of the X chromosome, with a prevalence of out 1/2500 females live birth. Most important clinical features of TS are short stature and gonadal failure. Approximately one third of girls with TS may undergo spontaneous puberty. Here we report a case of a variant TS with breast development.Case report: A 9-year-old girl was referred to our paediatric endocrinology outpatient...

hrp0097p2-62 | Diabetes and Insulin | ESPE2023

Growth patterns according to glycemic control, CGM apply, and diabetic complications in type 1 diabetes mellitus patients

Suh Junghwan , Kim Sujin , Lee Myeongseob , Song Kyungchul , Kwon Ahreum , Wook Chae Hyun , Kim Ho-Seong

Introduction: Chronic diseases such as type 1 diabetes mellitus (T1DM) may alter linear growth, but previous reports regarding growth in children with T1DM has been inconsistent. This study aims to investigate height and growth velocity of T1DM patients after diagnosis of diabetes and whether they are affected by various factors.Methods: This retrospective study, included 151 patients (male, 45.0%; mean age at diagnosis,...

hrp0086p1-p118 | Bone & Mineral Metabolism P1 | ESPE2016

Management of Tracheobronchomalacia During Asfotase Alfa Treatment in Infants with Perinatal-onset Hypophosphatasia: A Case Series

Padidela Raja , Yates Rob , Benscoter Dan , McPhail Gary , Chan Elaine , Nichani Jaya , Mughal M Zulf , Saal Howard M

Background: Hypophosphatasia (HPP) is a rare, inherited metabolic disease caused by loss-of-function mutations in the gene encoding tissue nonspecific alkaline phosphatase (TNSALP), resulting in hypomineralisation of bone. HPP presenting <6 months of age is often lethal due to respiratory insufficiency, with survival of 42% at 1 year. Asfotase alfa, a human recombinant TNSALP replacement, promotes bone mineralisation, with survival of 95% at 1 year in infants with HPP....

hrp0082lbp-d3-1002 | (1) | ESPE2014

Pseudoexon Activation in Nicotinamide Nucleotide Transhydrogenase in Two Siblings with Familial Glucocorticoid Deficiency

Chan Li , Novoselova Tatiana , Rath Shoshana , Carpenter Karen , Atkinson H , Dickinson Jan , Pachter Nick , Price G , Choong Cathy , Metherell Lou

Background: Two siblings of non-consanguineous parents presented with FGD, demonstrated by ACTH resistance with glucocorticoid but not mineralocorticoid deficiency. The proband presented at 21 months, unresponsive with hypoglycaemia (BGL 1.5 mmol/l). Endocrine evaluation subsequent to resuscitation indicated adrenal insufficiency with elevated ACTH. Hydrocortisone therapy was commenced. A sibling, 4 years younger than the proband had a short Synacthen test (SST) performed on d...

hrp0097p2-206 | Thyroid | ESPE2023

Hypothyroidism without elevation of thyroid-sttmulating hormone associated with oxcarbazepine use in children and adolescents

Kim Insung , Hwang JiHoon , Sung Juyoung , Kim Min-Sun , Park Hyunju , Heo Jung , Kim Min-ji , Lee Jee-Hun , Jin Dong-Kyu , Hyuk Kim Tae , Hoon Chung Jae , Yoon Cho Sung , Wook Kim Sung

Introduction: Hypothyroidism without elevation of thyroid-stimulating hormone level during oxcarbazepine use in children and adolescent. There have been studies on the association of oxcarbazepine, which is used as an anticonvulsant, with hypothyroidism, but studies in children and adolescents have been limited. The authors aimed to determine the effects of long-term oxcarbazepine on thyroid function in children and adolescents.M...

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...

hrp0084fc8.6 | Obesity - Basic | ESPE2015

Metabolic Effects of ADP355, Protein-Based Adiponectin Receptor Agonist, on Mice with High-Fat Diet Induced Fatty Liver Disease

Lee Eun Byoul , Kim Tae Hyuk , Lee Woo Jung , Kwon Ah Reum , Chae Hyun Wook , Kim Duk Hee , Kim Ho-Seong

Background: Adiponetin is considered a protective hormone exhibiting beneficial effects against insulin resistance, cardiovascular disease, and cancer. Non-alcoholic fatty liver disease (NAFLD) is considered the hepatic component of insulin resistance. Pharmacological activation of adiponectin signaling might be helpful for treatment of NAFLD, but it is difficult to develop the whole adiponectin protein as a drug because the C-terminal globular domain of adiponectin is extreme...

hrp0094p1-52 | Bone B | ESPE2021

Impact of Vertebral Fracture on Auxological Profile and Insulin-like Growth Factors of Children after Acute Lymphoblastic Leukemia Treatment

Bae Ahn Moon , Kim Sung Eun , Lee Na Yeong , Ki Kim Seul , Hee Kim Shin , Kyoung Cho Won , Soon Cho Kyoung , Ho Jung Min , Suh Byung-Kyu ,

Purpose: To investigate the overall prevalence of vertebral fractures (VF) following childhood acute lymphoblastic leukemia (ALL) treatment and examine the association of VF with growth trajectory and insulin-like growth factorsMethods: Children (n = 172; 59.3 % male) diagnosed with ALL at age between 2 and 18 years were assessed for VF by screening the lateral thoracolumbar spine radiographs (GenantÂ’s semi-quantitative met...