ESPE Abstracts

Background: Childhood pineal and/or suprasellar intracranial germ cell tumours (IGCT) are highly curable (>90%) with neuraxial radiation alone; international (SIOP) trials have aimed to decrease late radiation-induced neuroendocrine morbidity by substituting chemotherapy. However, without longitudinal study, disease and treatment contributions to long term outcomes remain unknown.Objective: To define tumour and treatment factors implicated in neuroen...

Background: Type 1 diabetes mellitus (T1DM) continues to pose serious health risks with devastating long-term complications. UK management and control of T1DM in children and young people (CYP) remains amongst the poorest in Europe and significant variations in diabetes health outcomes are evident. In 2012–13 a Best Practice Tariff (BPT) for paediatric diabetes care was introduced and a National Peer Review Quality Assurance programme (DQuINS) developed. Both were develop...

Background: Studies have shown that PAI-1 4G polymorphism is related to increased plasma PAI-1 levels, obesity, dyslipidaemia and cardiovascular disease (CVD) in adults. Few studies have explored that relationship in overweight/obese (Ow/Ob) children/adolescents.Objective and hypotheses: We investigated the relation between plasma PAI-1 levels, PAI-1 4g polymorphisms and lipid profiles in Ow/Ob children/ adolescents compared with healthy normal ...

Background: We previously reported in a national cohort, that adherence to subcutaneous Growth Hormone (GH) treatment in children is better with jet delivery compared with needle devices (Spoudeas et al. Patient Prefer Adherence. 2014;8:1255–1263). We also separately reported that adherent children showed significantly improved height outcomes at one year.Objective and hypotheses: To examine the potential influence of adherence and demograp...

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.<...

Background: Hypogonadotrophic hypogonadism (HH) is a complex developmental disorder characterized by a reduction in gonadotrophins (LH, FSH) released from the anterior pituitary. LH and FSH stimulate the ovaries or testes to release sex hormones that cause the onset of puberty, therefore delay in onset or complete absence of puberty is seen in the phenotype, often accompanied by short stature and genital abnormalities. When anosmia accompanies HH in the phenotype it is termed ...

Background and objective: Children with Thickened pituitary stalk (TPS) and/or Idiopathic central diabetes insipidus (ICDI) present to different (endocrine, oncology, and ophthalmology) specialists. Their rarity, absence of agreed radiological criteria or consensus guidance, make their management problematic. Biopsy is too dangerous and cases may remain undiagnosed or evolve over decades. We aimed: i) to longitudinally characterize a large childhood cohort presenting with TPS ...

Background: It has been previously reported that with current management, 9/10 children with craniopharyngioma will survive 10 years. Though most suffer both chronic neuroendocrine and cognitive impairments from disease and/or treatment, few prospective cognitive assessments have been reported which might separate the contributions of each.Objective and hypotheses: i) To prospectively evaluate the prevalence of cognitive dysfunction, before and after a c...

Background: Hypopituitarism in children is a complex condition and its hierarchical evolution at different developmental windows is unpredictable. Magnetic resonance imaging (MRI) is helpful but largely a subjective assessment of anomalies of the hypothalamo-pituitary (H-P) structures. We aimed to test the utility of a quantitative measure of pituitary and stalk in predicting neuroendocrine phenotypes.Patients and Methods:</stron...

Introduction: Easypod-connect™ for childhood growth disorders is a unique connected system that enables the transmission of injection adherence information for recombinant human growth hormone (r-hGH). Although this system has the potential to facilitate greater adherence, observational studies have shown declining adherence over prolonged periods when used without additional support. Supplemental nurse practitioner support has been envisaged but not inv...