hrp0086fc3.6 | Pituitary | ESPE2016

Pegvisomant is More Effective in Stunting Growth than Somatostatin Analogs in Childhood Acromegaly/Gigantism

Thomas-Teinturier Cecile , Simonin Gilbert , Vaczlavik Anna , Ajaltouni Zaina , Gaillard Stephan , Bougneres Pierre , Chanson Philippe

Background: We describe our experience in medical therapy for invasive somatotroph pituitary macroadenomas in 8 children or adolescents presenting with acromegaly/gigantism, in terms of growth and IGF-I levels control.Patients: Eight children, aged 5 to 17 years (median 12.4 years), presented with growth hormone (GH) hypersecretion related to somatotroph pituitary macroadenomas with cavernous sinus invasion in 6/8. Genetic testing revealed AIP mutation i...

hrp0086fc5.2 | Management of Disorders of Insulin Secretion | ESPE2016

Diabetes and Insulin Injection Modalities: Effects on Hepatic Expression and Activity of 11β-Hydroxysteroid Dehydrogenase Type 1 in Juvenile Diabetic Rats

Rougeon Veronica , Moisan Marie-Pierre , Barthe Nicole , Beauvieux Marie-Christine , Marissal-Arvy Nathalie , Barat Pascal

Background and hypotheses: Recent results showing elevated tetrahydrocortisol/tetrahydrocorticosterone ratio (THFs/THE) in morning urines of diabetic children compared to controls suggest an increased nocturnal activity of 11 β-hydroxysteroid dehydrogenase type 1 (11β-HSD1). We made the hypothesis that a reduced inhibition of hepatic 11β-HSD1 activity by exogenous insulin, due to its subcutaneous (SC) administration and absence of first hepatic passage, could ex...

hrp0086fc9.2 | Pathophysiology of Disorders of Insulin Secretion | ESPE2016

NBAS Mutations, a New Monogenic Cause of DISOPHAL, a New Syndrome with Type 1 Diabetes (T1D)

Rothenbuhler Anya , Maluenda Jerome , Aumont Cedric , Picard Veronique , Bougneres Pierre , Melki Judith

Background: While non-autoimmune T1D is rare in late childhood, few monogenic causes have yet been identified.Objective: 1) to identify the genetic basis of the yet unreported disease phenotype associating late childhood antibody-negative T1D, short stature, optic atrophy (OA), Pelger-Huët anomaly (PHA) of leukocytes and recurrent liver cytolysis: the “DISOPHAL” syndrome; 2) to attract comparable cases for further genetic investigation.</p...

hrp0086fc13.1 | Management of Obesity | ESPE2016

Quantitative Proteomic of Rat Livers Shows a Major Reprogramming of Mitochondrial Enzymes in Food-Restriction and Increased Stress Hallmarks in Ad Libitum Feeding

Gat-Yablonski Galia , Finka Andrija , Pinto Galit , Quadroni Manfredo , Shtaif Biana , Goloubinoff Pierre

Background: Studies in young mammals on the qualitative and quantitative molecular effects of food restriction (RES) and re-feeding, leading to catch up growth (CU) are scarce. Whereas RES may lead to growth and developmental deficits in children, it is a proven treatment to prolong life in all animals checked.Objective and hypotheses: We used MS analysis to understand how RES might lead to growth attenuation and prolonged life span and identify the majo...

hrp0086p1-p191 | Diabetes P1 | ESPE2016

Insulin Pump Does Not Allow a Better Control than Injections in Childhood Type 1 Diabetes (T1d) in the ISIS-Diab Cohort

Fur Sophie Le , Castell Anne Laure , Lucchini Philippe , Bougneres Pierre , Network ISIS-Diab

Background: The use of insulin pumps is rapidly spreading within the paediatric T1D community. A few small studies have promoted pump usage, while large registries have shown almost no advantage of this treatment.Objectives: Compare the results of treatment with insulin pump (‘Pump’) with insulin injections (‘Inj’) in a large cohort of children with T1D in field conditions.Patients: We studied 3137 children of t...

hrp0086p1-p224 | Diabetes P1 | ESPE2016

Space-time Environmental Associations in Childhood Type 1 Diabetes (T1D). A Case-control Geographical Approach in the ISIS-Diab Cohort

Bougneres Pierre , Le Fur Sophie , Valtat Sophie , Valleron Alain-Jacques , ISIS-Diab Network Network

Background: T1D concordance in MZ twins being ~40%, non-heritable factors play a major causal role in this autoimmune disease. T1D has recently increased in young European children. Collecting prospective environmental data in a cohort of millions children-years starting soon after birth seems unpracticable. Retrospective case-control studies are an alternative, provided biased controls and recall bias can both be avoided.Objectives: To develop a ‘v...

hrp0086p1-p238 | Diabetes P1 | ESPE2016

Correction of Carnitine Deficiency in Children with Recent Onset Type 1 Diabetes

Castell Anne Laure , Bibal Cecile , Gaignard Pauline , De Filippo Gianpaolo , Bougneres Pierre

Background: Carnitine deficiency (CD) has been reported in children at time of type 1 diabetes (T1D) diagnosis. By impairing free fatty acid ß-oxidation in liver, muscle mass and pancreatic ß cells, CD might impair glucose homeostasis and residual insulin secretion. We postulate that reversed of these FAO defects may help regenerate a healthier ß cell mass and increased the diabetes honeymoon duration.Objective: Evaluate the effects of car...

hrp0086p1-p250 | Diabetes P1 | ESPE2016

Type 1 Diabetes (T1D) Management with Few Blood Glucose (BG) Measurements but Frequent Free Adjustment of Treatment with Cell Phones or E-mails

Bibal Cecile , Laure Castell Anne , Aboumrad Brigitte , Lucchini Philippe , Bougneres Pierre

Background: Many children with type 1 diabetes (T1D) are asked to measure blood glucose (BG) 4–6 times a day routinely to adjust insulin dosages. There is no evidence though that such high frequency is beneficial to HbA1C or glycemic profile, although this is often claimed.Objectives: We challenged ‘the more BG measurements, the better control’ dogma for alleviation of T1D burden in child life and evaluation of HbA1C.<p class="abstext"...

hrp0086p1-p254 | Diabetes P1 | ESPE2016

A Case-Control Search of Environmental (E) Factors for Childhood Type 1 Diabetes (T1D) Using Lifeline Questionnaires in the ISIS-Diab Cohort

Balazard Felix , Le Fur Sophie , Biau Gerard , Valleron Alain-Jacques , Bougneres Pierre

Background: The rapidly increasing T1D incidence in European children suggests the recent emergence or extension of predisposing E factors, or the decrease of protective E factors acting during pregnancy, infancy or early childhood. E research has preferentially focused on specific candidate factors, such as hygiene hypothesis, enteroviruses, alimentation, in cohort or case-control studies. The risk of bias is a major concern in E studies.Objectives: To ...

hrp0086p2-p266 | Diabetes P2 | ESPE2016

Gene-Environment (GxE) Interactions in Childhood Type 1 Diabetes (T1D): A Case-only Geographical Approach in the ISIS-Diab Cohort

Bougneres Pierre , Fur Sophie Le , Valtat Sophie , Lathrop Mark , Valleron Alain-Jacques , ISIS-Diab Network France

Background: T1D concordance in MZ twins being ~40%, non-heritable factors play a major causal role in this autoimmune disease. T1D has recently increased in young European children. Collecting prospective environmental data in a cohort of millions children-years starting soon after birth seems unpracticable. Retrospective case–control studies are an alternative, provided biased controls and recall bias can both be avoided.Objectives: To develop a &#...