hrp0094p2-393 | Pituitary, neuroendocrinology and puberty | ESPE2021

First Morning Voided Urinary Gonadotropin Measurements for the Early Screening, Diagnosis and Follow-up of Central Precocious Puberty Patients

Ye Feng , Luo Xiaoping , Dong Guoqing , Chen Ruimin , Dong Zhiya , Lu Wenli , Fu Junfen , Zhan Shumin , Luo Feihong , Gong Chunxiu , Wei Liya , Yang Yu , Zhu Min , Ma Huamei , Chen Qiuli , Cheng Xinran , Gou Peng ,

Central precocious puberty (CPP) is one of the most common pediatric endocrine diseases with an ever increasing incidence. CPP is associated with the loss of final adult height, early menarche, psychological problems and an increased risk of developing diseases in later adulthood such as female reproductive system tumors. The gonadotropin-releasing hormone stimulation test (GnRHST) is the gold standard for the diagnosis of CPP. However, the test is costly and time consuming to...

hrp0097p1-361 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

First interim analysis of the value of First Morning Voided(FMV)Urinary GnRH for the Diagnosis in China CPP Patients

Ye Feng , Luo Xiaoping , Dong Guoqing , Chen Ruimin , Dong Zhiya , Lu Wenli , Luo Feihong , Gong Chunxiu , Wei Liya , Yang Yu , Zhu Min , Ma Huamei , Chen Qiuli , Cheng Xinran , Gou Peng

Background: The gold standard for CPP diagnosis is gonadotropin releasing hormone stimulation test (GnRHST). However, this test is expensive, invasive and inconvenient for screening. Some recent studies have demonstrated that the FMV urinary luteinizing hormone (U-LH) has a strong correlation with LH peak value and serum basal LH, and a high consistency with Tanner staging results, but due to the lack of large-sample, multicenter clinical research data, a form...

hrp0095p1-567 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

Two Cases with Hypergonadotropic Hypogonadism Due to Derivative X Chromosome

Elmaoğulları Selin , Sezer Abdullah , Öztoprak Ülkühan , Berna Çelik Ertaş Nur , Savaş Erdeve Şenay , Çetinkaya Semra

Introduction: A derivative chromosome (der) is a structurally rearranged chromosome generated either by a chromosome rearrangement involving two or more chromosomes or by multiple chromosome aberrations within a single chromosome. Balanced segmental anomalies such as inversion or translocation can lead to derivative chromosomes with partial duplication and deletion during meiosis. Here we present two patients with hypergonadotropic hypogonadism and various acc...

hrp0092lb-21 | Late Breaking Posters | ESPE2019

How Can the Occurrence of Delayed Elevation of Thyroid Stimulating Hormone in Preterm Infants Born Between 35 and 36 Weeks Gestation be Predicted?

Jung Heo You , Ah Lee Young , Jeong Lee Yun , Hee Lim Youn , Rim Chung Hye , Han Shin Seung , Ho Shin Choong , Won Yang Sei

Objective: We evaluated frequency and risk factors of delayed TSH elevation (dTSH) and investigated follow-up outcomes in the dTSH group with venous TSH (v-TSH) levels of 6–20 mU/L according to whether late preterm infants born at gestational age (GA) 35–36 weeks had risk factors.Methods: The medical records of 810 neonates (414 boys) born at Seoul National University Hospital who had a normal neonatal screenin...

hrp0086p1-p549 | Perinatal Endocrinology P1 | ESPE2016

Congenital Hyperinsulinism in Infancy: The Profiles of Insulin Secretory Granules are Markedly Different in Focal- and Diffuse β-Cells

Han Bing , Mohamed Zainab , Salomon-Estebanez Maria , Padidela Raja , Skae Mars , Craigie Ross , Rigby Lindsey , Cosgrove Karen , Banerjee Indi , Dunne Mark

Background: The mechanisms responsible for inappropriate insulin release from β-cells in congenital hyperinsulinism in infancy (CHI) have largely focused upon defects in KATP channels. Little is known about insulin biogenesis, the profiles of insulin in insulin-containing secretory granules or whether the impact of KATP channel defects is the same in diffuse- and focal disease.Objective and hypotheses: To define the ultrastruct...

hrp0082fc9.1 | Beta cells | ESPE2014

Inappropriately High Rates of Cell Proliferation in Diffuse Congenital Hyperinsulinism are Linked to Nuclear Expression of CDK6

Salisbury Rachel , Han Bing , Mohamed Zainaba , De Krijger Ronald , Gardner Laurienne , Gardner Julia , Cosgrove Karen , Padidela Raja , Newbould Melanie , Banerjee Indraneel , Hanley Neil , Dunne Mark

Background: Congenital hyperinsulinism of infancy (CHI) mainly arises from loss-of-function mutations in the KATP channel genes. As a consequence, insulin release is uncontrolled and causes persistent or recurrent episodes of hypoglycaemia in neonates. In patients with diffuse-CHI (CHI-D) increased rates of cell proliferation has been reported, but the causes of proliferation are unknown.Objective/Hypotheses: To assess the extent of cell proliferation an...

hrp0094fc2.2 | Fat, Metabolism and Obesity | ESPE2021

Phase 3 Trial of Setmelanotide in Participants With Bardet-Biedl Syndrome: Placebo-Controlled Results

Argente Jesus , Clement Karine , Dollfus Helene , Han Joan , Haqq Andrea , Martos-Moreno Gabriel , Mittleman Robert , Stewart Murray , Webster Matt , Yanovski Jack , Yuan Guojun , Haws Robert ,

Background: In a Phase 3 trial in participants with obesity due to Bardet-Biedl syndrome (BBS) and Alström syndrome, setmelanotide was associated with significant reduction from baseline in body weight and hunger at Week 52 (primary endpoint), with outcomes driven by responses in individuals with BBS.Methods: In the same trial (NCT03746522), individuals with BBS or Alström syndrome were randomized and received ...

hrp0095p1-498 | GH and IGFs | ESPE2022

A Long-Acting Growth Hormone Module For Paediatric Growth Hormone Deficiency In The Global Registry For Novel Therapies In Rare Bone & Endocrine Conditions – The GloBE-Reg LAGH Study

Miller Bradley , Savendahl Lars , Hickman Krystina , Smythe Christopher , Chen Ching , Choi Jin-Ho , Dou Xinyu , Gong Chunxiu , Hamza Rasha , Horikawa Reiko , Jorge Alexander , Faisal Ahmed S.

Introduction: Post-marketing surveillance registries provided extensive information about the safety and efficacy of daily growth hormone (GH) therapy during treatment. With the availability of novel long-acting GH (LAGH) therapies, it is important to determine whether the novel molecules or the different pattern of GH exposure lead to changes in the efficacy and safety profile. Therefore, new surveillance registries of LAGH are warranted.<p class="abstext...

hrp0095p1-499 | GH and IGFs | ESPE2022

Long-term safety and effectiveness of recombinant human growth Hormone inKorean pediatric patients with growth disorders: 9-year interim analysis fromLG Growth Study

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

This study aimed to evaluate the long-term safety and effectiveness of recombinant human growth hormone (rhGH; Eutropin®, Eutropin®Pen, Eutropin®AQ, and Eutropin®Plus, LG Chem, Ltd.) treatment in Korean pediatric patients. This observational study has been conducted since 2011, and the data were collected up to August 2021 for interim analysis. The incidence rates of all adverse events (AEs) were assessed for safety a...

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...