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.<...

hrp0082fc11.4 | Pituitary | ESPE2014

Management of Hyperhydration in a Child with Syndrome of Inappropriate Antidiuretic Hormone Secretion (Siadh) Using a Selective Vasopresin Receptor Agonist

Delgado-Carballar Violeta , Thankamony Ajay , Willemsen Ruben , Elleri Daniela , Dunger David

Background: Management of SIADH is challenging and no optimal therapies are available in children. We present a case of an 11 years old boy with severe SIADH resistant to 30% fluid restriction in the context of an intracranial suprasellar high grade B-cell lymphoma who requires hyperhydration for chemotherapy protocol. Tolvaptan is an oral highly selective arginine vasopressin V2 receptor antagonist, which has been approved for use in SIADH in adults.Obj...

hrp0082fc13.4 | Thyroid | ESPE2014

The Prevalence of Congenital Malformations in Infants with TSH Elevation on Newborn Screening: the Importance of Distinguishing Between True and Transient Congenital Hypothyroidism

Ouarezki Yasmine , Mansour Chourouk , Jones Jeremy , alghanay Abubaker , Smith Sarah , Stone David , Donaldson Malcolm

Backgrounds: The prevalence of congenital malformations (CM) is higher in infants referred with capillary (c) TSH elevation on newborn screening. However, establishing the prevalence of CM ± dysmorphic syndromes in true congenital hypothyroidism (CH) requires careful distinction between true and transient CH.Objective: To determine the prevalence of CM ± dysmorphic syndromes in all infants referred with TSH elevation on newborn screening in Sco...

hrp0082p1-d3-52 | Bone (1) | ESPE2014

Opposing Effects of Childhood Obesity on Radial and Tibial Bone Microstructure

Dimitri Paul , Jacques Richard , Paggiosi Margaret , King David , Walsh Jennifer , Bishop Nick , Eastell Richard

Background: Bone mass is low in obese children when measured by conventional techniques. However, these imaging modalities cannot quantify alterations in bone microstructure and strength. High resolution peripheral quantitative computed tomography (HRpQCT – isotropic voxel size 82 mm) provides the resolution required to determine 3-dimensional in-vivo bone microstructure; microfinite element (microFE) analysis of HRpQCT images provides insight into skeletal biome...

hrp0082p1-d3-127 | Fat Metabolism &amp; Obesity (2) | ESPE2014

Infancy Lipidomic Analyses and Associations with Early Nutrition and Growth

Prentice Philippa , Koulman Albert , Matthews Lee , Acerini Carlo , Ong Ken , Dunger David

Background: Links between early life exposures and long-term health outcomes may in part be due to nutritional programming, and suggested benefits of breast feeding during infancy include reduced risk of obesity and metabolic disease. Mechanisms remain unexplained but potential differences in lipid exposures during infancy may be involved.Objective and hypotheses: To explore the effects of breast- or formula-feeding on lipidomic profiles we used recently...

hrp0082p1-d3-94 | Sex Development | ESPE2014

Prenatal Exposure to Phthalates and Phenols in Relation to Anogenital Distance at Birth in Male Infants

Fisher Benjamin G , Thankamony Ajay , Ong Ken K , Dunger David B , Hughes Ieuan A , Acerini Carlo L

Introduction: Increasing incidence of male reproductive disorders may be due to fetal exposure to putative endocrine disruptor chemicals (EDCs), such as phthalates and phenols. Anogenital Distance (AGD) is a biomarker of fetal androgen action in animals, and has recently been linked to testicular dysgenesis syndrome in humans.Objective: To examine the relationship between prenatal phthalate and phenol exposure and birth AGD in male infants.<p class="...

hrp0082p2-d1-421 | Growth Hormone | ESPE2014

The Effects of Delaying Puberty with GnRH Agonists in Patients with Idiopathic GH Deficiency

Dunger David B , Lindberg Anders , Dorr Helmut G , Camacho-Hubner Cecilia , Geffner Mitchell E

Background: Treating central precocious puberty with GnRH agonist (GnRHa) to increase height gain is well-established. Although not recommended, GnRHa have also been used in patients with IGHD at onset of puberty yet there are few data on its efficacy.Objective and hypotheses: Growth prediction models derived from KIGS (Pfizer International Growth Database) may provide an opportunity to estimate additional height gain produced by pubertal blockade.<p...

hrp0082p2-d1-460 | Growth | ESPE2014

Skeletal Disproportion and Final Adult Height in Childhood-Onset Crohn’s Disease

Mason Avril , Iljuhhina Jelena , Laird Susan , Ross David , Ahmed Syed Faisal , Gaya Dan R , Gerasamidis Konstantinos

Background: Short stature is a recognized complication of Crohn’s disease (CD) in children. Less is known on the impact of disease on final adult height and body proportions.Objective and hypotheses: To assess height and body proportions in 48 adults (F: 30; M: 18) with childhood (ACO) or adult (AAO) onset CD and 44 children (C) (F:22; M:22) with CD.Method: Standing, sitting height (SH) and leg length (SILL) were measured and ...

hrp0082p2-d1-516 | Pituitary | ESPE2014

Neuroendocrine Dysfunctions Following Traumatic Brain Injury in Children: a 12-Month Prospective Study

Rojo Pilar , Carcavilla Atilano , Ortiz Irene , Arjona David , Borrego Raul , Aragones Angel

Background: Traumatic brain injury (TBI) is a frequent cause of endocrine dysfunctions. However, studies in children are scarce.Objective and hypotheses: To determine pituitary function in children after TBI. To analyze risk factors related with endocrine dysfunctions after 12 months follow up.Method: A prospective study of endocrine function in children after head injury was performed. Data was collected for baseline Glasgow coma ...

hrp0082p2-d1-572 | Sex Development | ESPE2014

Anogenital Distance, Penis Growth, and Masculine Behaviour Evidence for Independent Neurobehavioral Effects of Foetal Versus Postnatal Androgen Exposure in Boys

Pasterski Vickie , Acerini Carlo , Dunger David , Ong Ken , Hughes Ieuan , Thankamony Ajay , Hines Melissa

Background: Associations between foetal androgen deficiency and variations in anogenital distance (AGD) suggest that AGD is a reliable indicator of foetal androgen exposure. Similarly, variation in postnatal penis growth associated with variations in testosterone show penis growth to be a potential biomarker of early postnatal androgen exposure. Though variation in early androgen exposure is also hypothesized to underlie neurobehavioral masculinisation, until now, no reports h...