ESPE Abstracts

Background: The Fibroblast Growth Factors (FGFs) are a large family of proteins including paracrine, intracrine, and endocrine FGFs. Paracrine and endocrine FGFs interact with specific cell surface receptors (FGFRs) that, via intracellular tyrosine kinase activity, initiate a cascade of downstream intracellular events. Specificity of paracrine/autocrine FGF activity is provided locally by the local production of these FGFs and their cognate receptors. In contrast, endocrine FG...

Background: SWEET (www.sweet-project.eu) is a non-profit entity endorsed by ISPAD aiming to create an extensive network of certified CoRs for childhood diabetes in order to ensure high quality care.Objective and hypotheses: SWEET aims at an improved and more uniform care for people with diabetes through comparing processes and outcomes among participating members. The results of data analysis are conveyed to memb...

Obesity in childhood is associated to several cardiovascular risk factors summarized in the definition of metabolic syndrome such as hypertension, dyslipidemia and impaired glucose tolerance. Besides others, the pathogenetic background is insulin resistance, which deteriorates in mid puberty and normalizes at end of puberty. Accordingly, blood pressure, lipids, fasting glucose and 2 h glucose in oGTT increased from prepubertal stage to pubertal stage and decreased from puberta...

Type 2 diabetes mellitus (T2DM) is emerging as a new clinical problem within pediatric practice. Recent reports indicate an increasing prevalence of T2DM in children and adolescents around the world in all ethnicities, even if the prevalence of obesity is not increasing any more. There are great differences in T2DM prevalence worldwide. The majority of young people diagnosed with T2DM was found in specific ethnic subgroups such as African-American, Hispanic, Asian/Pacific Isla...

Adolescent obesity has an immense burden of disease and results in premature death due to several mechanisms: The classic cardiovascular risk factors associated with obesity, such as hypertension, dyslipidemia and type 2 diabetes mellitus, lead to cardiovascular diseases and consequently to premature death. Furthermore, adolescent obesity is associated with many life- limiting cancer types occurring 10 to 20 years later replacing smoking as the leading modifiable cause for can...

Background: The diagnosis of turner syndrome (TS) must be included in the differential diagnosis of all girls with short stature. Despite overall earlier diagnosis and treatment there still remain patients with TS who present late with delayed puberty. Although growth hormone (GH) is known to increase final height (FH) in girls with TS, little evidence exists on treatment in late-presenting girls.Objective and hypotheses: To assess the effect of late GH ...

Background: Osteosclerotic metaphyseal dysplasia (OSMD, OMIM 615198) is an extremely rare autosomal recessive disorder, within the family of sclerosing bone dysplasias. It is a distinctive type of osteopetrosis characterised by a unique pattern of osteosclerosis predominantly involving the metaphyseal margins of the long tubular bones. OSMD is characterised by skeletal deformity and multiple fractures and associated clinically with developmental delay, hypoton...

Background: Recurrence of craniopharyngiomas influences mortality. Apart from the extent of surgical resection, few clinical parameters have been consistently shown to be associated with recurrence. Radical resection is difficult due to infiltration of surrounding tissue and unacceptable morbidity. Predictors of recurrence are therefore needed.Aim: To establish a multinational cohort of patients with craniopharyngioma an...

Introduction: The BSPED Peer review programme was initiated in 2011 to provide a regular cycle of independent impartial professional assessment, against quality standards for Specialised Paediatric Endocrine Services (SPES) in the UK.1 We present here an evaluation of the outcomes from the first review cycle completed in 2017.Methods: We examined pre-review self-assessments (4–6 weeks before a site visit by the Peer Review team) and post-...

Introduction: Prescribing of recombinant human GH for growth failure in UK children is based on guidance from the National Institute of Clinical Excellence. In 2013, the BSPED initiated this national audit of children/adolescents newly-prescribed GH to monitor trends in NHS prescribing practice. Here we have examined these trends from 2013 to 2016.Patient population: All patients aged ≤16.0 years newly starting GH therapy in the UK.<p class="ab...