hrp0086p1-p724 | Pituitary and Neuroendocrinology P1 | ESPE2016

Estimating the Psychosocial Impact of Idiopathic Central Precocious Puberty (ICPP) in Girls Aged 6 to 8 Years: A Qualitative Study

Vasse Camille , Teixeira Maria , Paulsen Anne , Malivoir Sabine , Samara-Boustani Dinane , Carel Jean-Claude , Simon Dominique

Background: Emotional and behavioural problems are often used in support of GnRH agonists therapy in girls with early pubertal timing. However, there is little evidence to show that CPP leads to psychological distress and whether treatment is associated with improved psychological outcome.Objective and hypotheses: The objective of this qualitative study was to explore the psychosocial impact of ICPP in recently diagnosed girls.Meth...

hrp0086p1-p745 | Pituitary and Neuroendocrinology P1 | ESPE2016

Constitutional Delay of Puberty or Hypogonadotropic Hypogonadism: Diagnostic Value of Inhibin B and AMH Measurements

Istanbullu Sibel , Lahlou Najiba , Chevenne Didier , Leger Juliane , Carel Jean-Claude , Martinerie Laetitia

Background: Boys with delayed puberty represent one of the main cause for pediatric endocrine referrals. Although the majority of them have constitutional delay of growth and puberty (CDGP), it is important to identify isolated hypogonadotropic hypogonadism (IHH) for optimal management.Objective and hypotheses: The aim of the study was to evaluate the usefulness of inhibin B and AMH as biological markers for distinguishing between CDGP and IHH.<p cla...

hrp0086p1-p749 | Pituitary and Neuroendocrinology P1 | ESPE2016

Age of Onset of Puberty in Yaounde, Which Normative Reference Data?

Sap Suzanne , Komba Darelle , Sobngwi Eugene , Obama Marie Therese , Koki Paul Olivier , Mbanya Jean Claude

Background: The age of onset of puberty varies from country to another and, within every country, from one socioeconomic group to another. In Sub-Saharan Africa, particularly in Cameroon, there is paucity of data on this topic.Objective and hypotheses: To determine clinically meaningful normative reference data that describe the timing of sexual maturity indicators among Cameroonian children and to determine factors that influence the onset of this matur...

hrp0086p2-p772 | Pituitary and Neuroendocrinology P2 | ESPE2016

Brain MRI in Evaluation of Endocrine Diseases of Childhood: Causal and Incidental Lesions

Selbonne Emilie , Martinerie Laetitia , Leger Juliane , Elmaleh Monique , Sebag Guy , Carel Jean-Claude

Background: Brain MRI is an essential tool in the diagnosis of neuroendocrine disorders and aims at detecting anatomical abnormalities and tumors. However, it may lead to the identification of unrelated or questionably related abnormalities in the hypothalamic-pituitary region and/or the rest of the brain parenchyma.Objective and hypotheses: To establish the prevalence of causal lesions on brain MRI of children evaluated for hypopituitarism, assess the f...

hrp0086p2-p841 | Syndromes: Mechanisms and Management P2 | ESPE2016

Safety and Efficacy of Growth Hormone (GH) in Combination with the Gonadotrophin Releasing Hormone Agonist Leuprorelin in Pubertal Children with Idiopathic Short Stature

Benabbad Imane , Tauber Maite , Rosilio Myriam , Paris Emmanuel , Berggren Lovisa , Patel Hiren , Carel Jean-Claude

Background: Due to delays in diagnosis of Idiopathic Short Stature (ISS), puberty has often started when Growth Hormone (GH) is initiated.Objective and hypotheses: To compare GH+leuprorelin (GH+L) with GH alone in patients with ISS and at Tanner puberty stage 2 or 3. We hypothesised that co-administration of GH+L for 2–3 years would delay puberty, prolong GH efficacy and increase adult height.Method: A phase 3, randomised, ope...

hrp0086lbp1 | (1) | ESPE2016

46,XY DSD due to Isolated AMH Deficiency Resulting in Persistent Müllerian Duct Syndrome (PMDS) as a Consequence of a Single-Base Deletion in a SF1-Response Element of the AMH promoter

Valeri Clara , di Clemente Nathalie , Marshall Ian , Schteingart Helena , Josso Nathalie , Rey Rodolfo , Picard Jean-Yves

Background: Isolated persistence of Müllerian ducts in an otherwise normally virilised 46,XY newborn, a condition known as PMDS, is a disorder of sex development (DSD) due to a defect limited to AMH-dependent Müllerian duct regression.Objective and hypothesis: We report the case of a patient with PMDS and extremely low serum AMH in whom no mutations were detected in the AMH gene coding sequences. A single base deletion identified in th...

hrp0082p1-d1-58 | Diabetes | ESPE2014

A leu402pro Mutation of the Non-hla Gene il18rap in Aggressive Neonatal Type 1 Diabetes Mellitus

Dirlewanger Mirjam , Blouin Jean-Louis , Bevillard Jeremy , Santoni Federico , Schwitzgebel Valerie

Background: Neonatal diabetes mellitus is defined by severe hyperglycemia appearing before 6 months of age. It occurs in about one in 200 000 live births and most cases are known to be of monogenic origin. Classical autoimmune type 1 diabetes mellitus (DM) is exceptional in this age group.Objective and hypotheses: Recently non-HLA type 1 DM susceptibility genes, such as IL18RAP, influencing the rate of progression to diabetes among children with...

hrp0082p1-d3-132 | Fat Metabolism &amp; Obesity (2) | ESPE2014

Impact of Maternal and Fetal Inflammatory Markers on Neonatal and Infant Adiposity

Donnelly Jean M , Walsh Jennifer M , Horan Mary , Molloy Eleanor J , Auliffe Fionnuala Mc

Background: The effect of maternal obesity and the associated maternal inflammation on neonatal and paediatric health and wellbeing over the early childhood years is not fully understood.Objective and hypotheses: This study aimed to determine the impact of maternal and fetal inflammatory factors on infant anthropometric measurements.Method: 265 mother–infant pairs from an RCT assessing the effect of a low glycaemic index diet ...

hrp0082p2-d3-435 | Growth Hormone (2) | ESPE2014

First-year Growth Response to GH in Relation to Final Height Outcome in Prepubertal Children with Idiopathic GH Deficiency

Straetemans Saartje , Thomas Muriel , Tenoutasse Sylvie , De Schepper Jean , Rooman Raoul

Background: Several definitions of poor growth response to first-year GH treatment, have been proposed based on the observed response of a large group of patients.Objective and hypotheses: Since a complete compensation of the height deficit is expected in children with GH deficiency (GHD) treated with GH, we have studied the different parameters for the first-year growth response in relation to the adult height gain in prepubertal children with iGHD....

hrp0082p2-d3-485 | Hypoglycaemia | ESPE2014

Nocturnal Hypoglycaemia in Diabetic Children: Continuous Glucose Monitoring Reveals More of the Iceberg

Bachmann Sara , Martin Eva , Ritschard Vreni , Hess Melanie , Jockers Katharina , Vuissoz Jean-Marc , Zumsteg Urs

Background: Hypoglycaemia is the most common and threatening complication in diabetic children. Nocturnal hypoglycaemia is mostly unrecognised and asymptomatic, but recurrent mild hypoglycaemia at night can lead to hypoglycaemia unawareness and reduced performance at daytime.Aims: To evaluate frequency and duration of nocturnal hypoglycaemia in type 1 diabetic children and to identify risk factors for such events.Patients/methods: ...