ESPE Abstracts

Objectives: X-linked hypophosphataemia (XLH) is a rare, hereditary phosphate-wasting disorder characterised by excessive activity of fibroblast growth factor 23. The International XLH Registry (NCT03193476) (initiated in August 2017, target 1,200 children and adults with XLH, running for 10 years) will provide information on the natural history of XLH and impact of treatment on patient outcomes. This report summarises baseline data from the first interim analy...

Background: Non-classic congenital adrenal hyperplasia (NC-CAH) is caused by pathogenic variants of the CYP21A2 gene, and in most cases is not associated with cortisol deficiency as is the classic (severe) form. The majority of children with NC-CAH are asymptomatic and do not require cortisol replacement therapy with hydrocortisone unless they develop symptoms of hyperandrogenemia such as early pubarche, growth acceleration, advanced bone age, and ultimately s...

Objectives: Recently, aromatase inhibitors are increasingly prescribed as an alternative off-label therapy for short stature in boys, yet its efficiency is controversial. This study aimed to evaluate the effect of aromatase inhibitors on the treatment of short statured boys in adolescence by a comprehensive meta-analysis of published randomized controlled trials.Methods: Literature search, quality assessment and data ext...

Background: Low vitamin D levels have been linked to stunted growth and lower bone mineral density. Vitamin D insufficiency is a recognised condition in children in the UK. However, optimal levels of vitamin D are not adequately defined and guidance regarding supplementation is also limited.Aim: To identify vitamin D levels in a select cohort of Caucasian children aged 0–16 years, and describe how preterm birth, obesity, age and malabsorptive condit...

Objective: To establish INS-1 cell iron overload model, and study the effect on proliferation, insulin-secretion and oxidative stress change.Methods: INS-1 cells were cultured in either normal or high glucose medium. Both groups were treated with different concentrations of (5, 10, 20, 40, 80 μmol/l respectively) of ferric ammonium citrate (FAC). Number and state of the cells were detected by trypan blue staining. Labile iron pool (LIP) were calcula...

Background: Melatonin is known to regulate a variety of biological processes. The investigation and application of melatonin in oral development have got a lot of attention. The study was performed to investigate the effects of melatonin on development of human dentin formation.Objective and hypotheses: To investigate the role of melatonin in proliferation and differentiation of human dental pulp cells(hDPCs).Method: HDPCs were obt...

Background: Abnormalities in glucose homeostasis are fairly common complications in thalassemia major (TM) patients. Previous studies had shown associations between some endocrinopathies and iron overload of the myocardium, liver as assessed by MRI techniques. This study aimed at determining the relationship among cardiac T2*, liver T2* and abnormal glucose in TM patients.Objective and hypotheses: A total of 34 (27 male) transfusion-dependent TM patients...

Background: Adrenoleukodystrophy (ALD) is a genetic disease associated with demyelination of the CNS, adrenal insufficiency, and accumulation of very long-chain fatty acids in tissue and body fluids.Objective: To research the clinical features, laboratory tests, imaging examinations and treatment on children who suffer from X-linked ALD. Also aim at revealing the correlation between the severity of disease and level of very long chain fatty acids (VLCFAs...

Background: Because ghrelin is closely linked to insulin resistance and type 2 diabetes mellitus in adults, ghrelin might also participate in the development and progression of pathological changes in insulin resistance in SGA infants.Objective and hypotheses: This study aimed to determine insulin sensitivity index (ISI), variation in ghrelin levels, and their relationship in small for gestational age (SGA) rats.Method: The SGA ani...

Background: Notch1 – Delta-like ligand 4 (Dll4) signaling pathway has a biological effect of negative feedback regulation to VEGF in retinal vascular development process. There are few studies on the inhibition of Notch1-Dll4 signaling pathway in ROP and the regulatory pathway of VEGF.Objective and hypotheses: 67-day-old mice were divided into oxygen-induced retinopathy group and control group. We took ten mice from each group at postnatal day 7 (p7...