hrp0089p3-p394 | Thyroid P3 | ESPE2018

A Case of Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) in a Girl with Newly Diagnosed Hashimoto Thyroiditis

Lim Yijuan Yvonne , Sng Anjian Andrew , Ho Cindy Wei-li , Lee Yung-Seng , Loke Kah-Yin

Background: SREAT is a rare entity in children, with about 60 cases described to date. It is characterised by acute encephalopathy, elevated anti-thyroid antibodies and exclusion of other causes of encephalopathy like infection, tumour, toxic or metabolic diseases. Typical presentations in children include behaviour changes, psychosis and seizures. The role of anti-thyroid antibodies in the pathogenesis is not clearly understood, and the titre level does not predict severity o...

hrp0086p1-p203 | Diabetes P1 | ESPE2016

Diabetes Mellitus Caused by Bone Marrow Transplantation and Total Body Irradiation – Experience from a Regional Single Centre

Candler Toby , Wei Christina , Bradley Karin , Cox Rachel , Elson Ruth , Stevens Michael , Crowne Elizabeth

Background: Diabetes is a recognised long term sequelae in childhood cancer survivors following bone marrow transplantation and total body irradiation (BMT/TBI), due to a combination of insulin deficiency and resistance.Aim: To characterise presentation, treatment and clinical course of diabetes in childhood leukaemia survivors post BMT/TBI.Method: A single centre retrospective case note review of diabetes post BMT/TBI identified f...

hrp0086p1-p251 | Diabetes P1 | ESPE2016

Metabolic Impairments among Adult Survivors of Paediatric Abdominal and Pelvic Tumours in the St Jude Lifetime Cohort Study

Wilson Carmen , Chemaitilly Wassim , Liu Wei , Srivastava Deokumar , Hudson Melissa , Robison Leslie , Ness Kirsten

Background: Adverse changes in metabolic parameters and body composition are frequently reported among childhood cancer survivors treated with cranial or total body irradiation. Data regarding the occurrence of metabolic impairments among survivors following abdominal and pelvic radiation are lacking.Objective and hypotheses: To define the prevalence of metabolic impairments among survivors of paediatric abdominal and pelvic solid tumours and to assess t...

hrp0086p1-p738 | Pituitary and Neuroendocrinology P1 | ESPE2016

Oxytocin Deficiency is Associated with Hyperphagia and Weight Gain in Hypothalamic and Common Obesity: A First-in-Humans Proof-of-Concept Study

Gan Hoong-Wei , Leeson Clare , Aitkenhead Helen , Spoudeas Helen , Martinez-Barbera Juan Pedro , Dattani Mehul

Background: Hypothalamic obesity (HyOb) is a rare form of treatment-resistant morbid obesity associated with congenital or acquired hypothalamic damage. Its pathophysiology has been attributed to hyperphagia and hyperinsulinaemia. The wider roles of oxytocin (OXT) in regulating appetite and weight have recently emerged in animal and human studies, but there is no human evidence that hypo-oxytocinaemia contributes to weight gain.Hypothesis: Hypo-oxytocina...

hrp0082fc5.6 | Neuroendocrinology | ESPE2014

The Diencephalic Syndrome of Emaciation in Infantile Hypothalamochiasmatic Low-Grade Gliomas : a Retrospective Case–Control Study of Diagnostic Parameters and Long-Term Outcomes Over 30 Years of Follow-Up

Gan Hoong-Wei , Meijer Lisethe , Gullick Georgina , Senbanjo Margaret , Walker David , Spoudeas Helen

Background: Diencephalic syndrome (DS) is a rare disorder of severe emaciation classically associated with infantile Hypothalamochiasmatic low-grade gliomas (HCLGGs) and GH excess. However, diagnostic criteria remain undefined and published literature includes non-specific tumour-related cachexia. In a large optic pathway LGG cohort (n=166), we have previously demonstrated that DS independently predicts multiple tumour progressions and severe endocrine morbidity.<...

hrp0082p1-d2-29 | Autoimmune Endocrine Disease | ESPE2014

Standard Population Screening for Diabetes Mellitus has Low Sensitivity in Identifying Diabetes in Adult Survivors of Childhood Bone Marrow Transplantation with Total Body Irradiation

Wei Christina , Unsworth Rebecca , Davis Nikki , Elson Ruth , Bradley Karin , Stevens Michael , Crowne Elizabeth

Background: Adult survivors of childhood leukaemia treated Bone marrow transplantation with total body irradiation (BMT/TBI) have an increased risk of diabetes mellitus (DM) disproportionate to their level of adiposity or other recognised risk factors. Post prandial hyperglycaemia due to reduced β-cell reserve after irradiation will be missed by fasting glucose (FG) levels. However, the UK National Institute of Clinical Excellence (NICE) screening guidelines recommend the...

hrp0082p3-d2-747 | Diabetes (3) | ESPE2014

Comparison the Clinical Efficacy of Autologous Hematopoietie Stem Cell Transplantation and Traditional Insulin Therapy in Newly Diagnosed Primary Childhood Type 1 Diabetes

Yi Gu , Chunxiu Gong , Xiaoxia Peng , Liya Wei , Chang Su , Fengting Li , Yi Wang

Objective: Evaluation the clinical efficacy of autologous hematopoietie stem cell transplantation and traditional insulin therapy.Methods: This is a case–control study. The subject investigated were diagnosed primary childhood type 1 diabetes in Beijing Children’s Hospital Endocrinology Centre, and there are 14 cases did the immune intervention combined with autologous hematopoietie stem cell transplantation in other hospital during the first 3...

hrp0084p1-24 | Diabetes | ESPE2015

The Influence of miR-125b in Pancreatic β-Cell Apoptosis

Han Bei , Zhu Ziyang , Gu Wei , Ni Shining , Yang Ruixue , Shi Xing , Yuan Xuewen , Gao Wentao

Background: Type 1 diabetes is characterised by destruction of islet β cell by autoimmune insulitis and islet cell apoptosis. This study analysed the microRNA 125b how to regulate pancreatic β-cells dysfunction, aiming to elucidate the association between miRNA125b and type 1 diabetes.Objective and hypotheses: Recent study indicate miRNA may have role in the development of type 1 diabetes, so this study analyse the miRNA expression profile in t...

hrp0084p1-157 | Miscelleaneous | ESPE2015

How do Adolescent Minors Banking Sperm Before Cancer Therapy Subsequently use the Fertility Service? A Post Banking Re-Evaluation

Pigot James , Michaelidou Maria , Williamson Elizabeth , Webb Alison , Gan Hoong-Wei , Spoudeas Helen

Background: Gonadotoxic cancer therapy may cause adult male infertility. We previously reported that, of 2/3rds of underage males agreeing to pre-treatment sperm cryopreservation before cancer treatment, 2/3rds succeeded.Objective and hypotheses: i) To evaluate how many patients banking sperm successfully (GP1) returned for post treatment re-evaluation compared with those who attempted but failed (GP2). ii) To compare intergroup survival and fertility ra...

hrp0084p2-240 | Bone | ESPE2015

Size-Corrected Bone Mineral Density is not Affected by Haematopoietic Stem Cell Transplantation and Total Body Irradiation in Leukaemia Survivors

Wei Christina , Elson Ruth , Cox Rachel , Bradley Karin , Barton John , Stevens Michael , Crowne Elizabeth

Background: Childhood haematopoietic stem cell transplantation and total body irradiation (HSCT/TBI) survivors have multiple risk factors for reduced bone mineral density (BMD) and poor growth. Reduced z-scores from dual energy x-Ray absorptiometry (DEXA) have been reported, but are unreliable in patients with short stature/abnormal body composition.Objective: To investigate the influence of HSCT/TBI on size-corrected BMD in childhood leukaemia ...