hrp0092p1-182 | Diabetes and Insulin (1) | ESPE2019

The Influence of Excess Iron on Pancreatic Beta Cells

Zhang Lina , Hou Lele , Liu Zulin , Li Pinggan , Huang Siqi , Meng Zhe , Ou Hui , Jiang Zhuannan , Liang Liyang

Objective: To establish INS-1 cell iron overload model, and study the effect on iron overload, proliferation, insulin secretion, mitochondria defect and oxidative stress change.Methods: INS-1 cells were cultured with different concentrations (0 as control and 5, 10, 20, 40, 80,160,320µmol/L respectively) of ferric ammonium citrate (FAC). Labile iron pool (LIP) were calculated by detecting calcein-AM fluorescence ...

hrp0092p1-316 | Diabetes and Insulin (2) | ESPE2019

An Evaluation of the Accuracy of a Flash Glucose Monitoring System in Children with Diabetes in Comparison with Venous Blood Glucose

Cao Bingyan , Wang Rui , Gong Chunxiu , Wu Di , Su Chang , Chen Jiajia , Yi Yajun , Liu Min , Liang Xuejun , Li Wenjing

Aims: To evaluate the performance of a factory-calibrated flash glucose monitoring system in children with diabetes compared to venous blood glucose (BG).Methods: A total of 13 hospitalized participants newly diagnosed with type 1 diabetes, aged 1~14 years old, were involved in the study. Sensor glucose measurements on days 2, 3, 6, 7, 12 and 13 of wear were compared with venous BG. During these days, the venous BG ...

hrp0089p3-p272 | Multisystem Endocrine Disorders P3 | ESPE2018

Two Cases of Costello Syndrome and Literatures Review

Zhuan-Nan Jiang , Le-le Hou , Zu-lin Liu , Hui Ou , Zhe Meng , Li-na Zhang , Li-yang Liang

Objective: To investigate the clinical features and genetic characteristics of HRAS-associated Costello Syndrome.Method: Characteristics of clinical data and gene mutation of two cases Costello Syndrome in XX hospital were retrospectively analyzed. The related literature was searched by using search terms ‘HRAS’ or ‘Costello Syndrome’.Result: Both patients were presented with mental retardation, growth retardati...

hrp0089lb-p9 | Late Breaking P1 | ESPE2018

Two Siblings with Autosomal Recessive Syndromic Hypopituitarism Caused By Mutations in TBC1D32

Hietamaki Johanna , Iivonen Anna-Pauliina , Kansakoski Johanna , Miettinen Paivi J. , Liu Xiaonan , Vaaralahti Kirsi , Hero Matti , Varjosalo Markku , Raivio Taneli

Patients who suffer from congenital hypopituitarism display a wide spectrum of phenotypes including pituitary hormone deficiencies and, in some cases, additional extrapituitary manifestations depending on the causative gene. A group of genes underlying hypopituitarism has been identified, yet several of them remain unknown. Here, we identified compound heterozygous variants in the TBC1D32 gene, c.1165_1166dupGT, p.(Gln390Phefs*32) and c.2151delA, p.(Lys717Asnfs*29) in...

hrp0082p3-d1-878 | Perinatal and Neonatal Endocrinology | ESPE2014

Clinical and Genetic Analysis of 95 Cases of Congenital Hyperinsulinism

Huang Shuyue , Gong Chunxiu , Su Chang , Qi Zhan , Wu Di , Cao Bingyan , Gu Yi , Li Wenjing , Liu Min , Liang Xuejun

Background: We want to know the clinical presentation and genetic mutation of congenital hyperinsulinism (CHI) patients in our country.Objective and hypotheses: To investigate the clinical outcomes and gene mutations related to CHI in our patients.Method: We studied the therapeutic outcomes of 95 cases of CHI and analyzed the associations between gene mutations and clinical features in 55 cases.Results: Among...

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...

hrp0089lb-p14 | Late Breaking P1 | ESPE2018

Beta-cell Function in Chinese Youngsters with Type 1 Diabetes and Assessment of Surrogate Markers of Severe Insulin Deficiency

Yuan Jinna , Derraik Jose G B , Fu Junfen , Dong Guanping , Cutfield Wayne S , Wu Wei , Huang Ke , Jiang Youjun , Chen Xiaochun

Objective: We assessed whether beta-cell function progressively decreases over time with greater type 1 diabetes mellitus (T1DM) duration using a mixed-meal tolerance test (MMTT). We also assessed simpler and more practical surrogate parameters for clinical use.Methods: We studied 57 children and adolescents with T1DM in Hangzhou (China), mean age at diagnosis was 8.3 years (range 2.3 to 15.3 years), with an average diabetes duration of 2.5 years (range ...

hrp0086rfc3.5 | Pituitary | ESPE2016

Prospective Dynamic Evaluation of Hypothalamo-Pituitary Function in 30 Cases of Paediatric Craniopharyngioma, by Hypothalamic Injury and Treatment; A Single Centre Series

Guzzetti Chiara , Losa Laura , Improda Nicola , Pang Gloria , Phatarakijnirund Voraluck , Gan Hoong-Wei , Hayward Richard , Aquilina Kristian , Ederies Ash , Spoudeas Helen A.

Background: Craniopharyngiomas are the commonest pituitary tumours of childhood. Though benign histologically, their localisation and invasive tendency can cause significant neuroendocrine morbidity and late mortality.Objective and hypotheses: To prospectively determine risk factors for neuroendocrine morbidity by longitudinal survival analysis.Method: All children with craniopharyngioma newly presenting to our quaternary centre be...

hrp0082p1-d2-38 | Bone | ESPE2014

Increased Rates of Infantile Hypercalcaemia Following Guidelines for Antenatal Vitamin D3 Supplementation

Amato Lisa A , Neville Kristen A , Hameed Shihab , Quek Wei Shern , Verge Charles F , Woodhead Helen J , White Chris P , Horvath Andrea Rita , Walker Jan L

Background: Consultations for infantile hypercalcaemia have increased at Sydney Children’s Hospital since guidelines for vitamin D3 supplementation during pregnancy were introduced in 2006. Recent nationwide shortages of low-calcium formula (LCF) suggest this problem may be widespread.Aim: To determine if infantile hypercalcaemia is occurring more commonly, identify potential aetiologies and clinical significance.Me...

hrp0082p1-d3-90 | Diabetes (2) | ESPE2014

The Relationship of Serum 25-Hydroxyvitamin D with Glucose Homeostasis in Obese Children and Adolescents in Zhejiang, China

Jiang You-Jun , Huang Ke , Fu Jun-Fen , Liang Jian-Feng , Zhu Hong , Zhu Zhi-Wei , Hu Li-Fei , Dong Guan-Pin , Chen Xue-Feng

Background: Evidence of the association between vitamin D, insulin resistance and oral disposition index (oDI) in obese children and adolescents is limited.Objective and hypotheses: We investigated serum 25(OH)D levels in obese children and adolescents in Zhejiang, China, and determined the relationship between serum 25(OH)D and glucose metabolism.Method: A cross-sectional design was used. All together 348 obese and 445 non-obese c...