ESPE Abstracts

Introduction: Pediatric adrenocortical tumors (pACT), comprising highly malignant pediatric adrenocortical carcinomas (pACCs) and less aggressive adenomas (pACAs), present a rare yet clinically significant challenge. pACTs often pose a diagnostic and therapeutic dilemma due to their elusive differentiation and resistance to therapy. Originating from the adrenal cortex, pACTs are functional and, thus, characterized by a unique urinary steroid metabolome. This s...

Background: A third of children in the UK are obese or overweight. The majority are not in contact with medical services. Presentation to general paediatricians for unrelated condition may pose an opportunity to identify children with obesity. The UK obesity services for Children & Adolescents (OSCA) group have produced guidelines for management of obese children in secondary care.Objective and hypotheses: Determine the prevalence of obesity in gener...

A 15 year old girl presented with severe symptomatic hypoglycaemia. Diagnostic work up revealed only a diminished GH secretion at and during severe hypoglycaemia and after arginine stimulation. Injection of GH twice daily abolished the hypoglycaemia for several days. For practicability a treatment with long acting GH (Ngenla) 46 mg/week was started. Symptoms were not entirely abolished, so that the dose was increased to 54 mg /week which was successful. However 2 weeks after t...

Background: Septo-optic dysplasia (SOD) is a rare condition diagnosed in children with two or more of the following: hypopituitarism, midline brain abnormalities, and optic nerve hypoplasia (ONH). Children with SOD experience varied visual impairment and endocrine dysfunction. Autistic-like behaviours have been reported, however the nature and prevalence of these neurobehavioural impairments remain to be fully understood. The present systematic review aimed to...

Background: iMAD is a very rare cause of ACTH-independent Cushing´s syndrome in young children. Bilateral adrenalectomy is a definitive treatment, but poses young children at a high risk due to life-long adrenal insufficiency. Therefore, pharmacological treatment with metyrapone seems an alternative bridging option for a limited period of time.Case report: A 3 6/12 year old boy presented with a history of rapid wei...

Encouraged by unimolecular dual incretin co-agonists (GLP1/GIP) to enhance glycemic efficacy (Finan et al. 2013, Sci Transl Med) and GLP1/glucagon co-agonists to enhance weight loss efficacy (Day et al. 2009, Nat Chem Biol) and to restore diet-induced leptin sensitivity (Clemmensen et al. 2014, Diabetes), we recently developed the first tri-agonist (GLP1/GIP/glucagon) for the treatment of metabolic disorders (Finan et al. 2014, Nat Med). Importantly, this concerted triple agon...

A 15y old girl presented with severe hypoglycaemic symptoms in combination with low blood glucose levels (<60 mg/dl). She had symptoms for over 1 year in combination with reduced activity, development of oligomenorrhoea after 2 years of normal cycles, increased fluid intake. Continuous sc. glucose measurement revealed constantly low or reduced glucose levels apart from a few increases after meals. Metabolic laboratory testing was normal apart from a single diminished GH lev...

Background: Craniopharyngioma is a benign tumour that develops in the sellar and surrounding parasellar regions, including the hypothalamus and the pituitary gland responsible for the production and regulation of neuropeptides. Oxytocin is a hypothalamic neuropeptide that has been identified as a key modulator of appetite drive and social cognition. Here, we present a case of parent-observed improvements in neurobehavioural dysfunction following administration...

Background: Noonan syndrome (NS) is a congenital polymalformative disorder caused by aberrant up-regulated signalling through RAS GTPase. Although NS is associated with hematologic malignancies, no predisposition for neuronal tumors was reported so far.Objective and hypotheses: We describe two cases of young patients with NS and dysembryoplastic neuroepithelial tumor (DNET). This is the first case series reporting a rare type of intracranial tumor in NS....

Background: Several studies show impaired glucose tolerance at a certain number of years after start of growth hormone in SGA-children.Objective and hypotheses: To perform a longitudinal and cross-sectional evaluation of the oGTTs (glucose-insulin-pairs) in prepubertal GH-treated SGA-children and to evaluate if ISI and HOMA are effective surrogates for glucose tolerance and of prognostic value (0–1 year data).Method: In 81 pre...