hrp0084p3-995 | Gonads | ESPE2015

Puberty and Gonadal Function in Adolescents Girls after Renal Transplantation

Hecquet Blandine , Charbit Marina , Polak Michel , Bidet Maud

Background: Renal transplantation (RTx) is the most common solid organ transplant procedure. Several studies have reported on puberty and gonadal function in female RTx recipients with controversial results.Objective: We sought to describe puberty and gonadal function in adolescents after RTx before 16 years.Methods: We reported retrospectively the clinical signs of puberty, growth, medication and graft function of 20 girls aged 19...

hrp0082p1-d3-196 | Pituitary | ESPE2014

Rathke Cleft Cysts and Endocrine Dysfunction in Children

Dupre Marie , Brioude Frederic , Berard Laurence , Esteva Blandine , Houang Muriel

Background: Rathke’s cleft cysts (RCC) are benign lesions of the pituitary gland, remnants of Rathke’s pouch. RCC have rarely been reported in children and adolescents and are usually known as asymptomatic but some cases may be associated with hormonal disturbances.Objective and hypotheses: The aim of the study was to assess the characteristics and frequency of endocrine disturbances in children with RCC.Method: We retros...

hrp0095p2-264 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

StAR gene mutation : description of puberty development and genital life from 6 patients 46,XX with classic Congenital Lipoid Adrenal Hyperplasia.

Jardin-Millet Isabelle , Amouroux Cyril , Dufourg Marie-Noelle , Lambert Anne-Sophie , Bouvattier Claire , Parada-Bonte Fabienne , Reynaud Rachel , Demeinex Estelle , Courbiere Blandine , Albarel FrÉDÉRique , Netchine IrÈNe , Houang Muriel

Introduction: Congenital Lipoid Adrenal Hyperplasia (CLAH) is a severe deficiency of adrenal and gonadal steroidogenesis, caused by mutations in the StAR gene and the accumulation of cholesterol in the outer mitochondrial membrane due to steroidogenic acute regulatory protein (StAR) deficiency. Affected patients present peripheral adrenal insufficiency with early onset, and variable phenotypes at the age of puberty.Objective of o...

hrp0095p1-4 | Adrenals and HPA Axis | ESPE2022

Glucocorticoid-induced adrenal insufficiency: morning plasma cortisol values safely assess recovery of the HPA axis in pediatrics

Laulhé Margaux , Dumaine Cécile , Chevenne Didier , Leye Fallou , Faye Albert , Dozières Blandine , Strullu Marion , Viala Jérôme , Hogan Julien , Houdouin Véronique , Léger Juliane , Simon Dominique , Claude Carel Jean , Storey Caroline , Guilmin-Crépon Sophie , Martinerie Laetitia

Background and Objectives: Chronic administration of synthetic glucocorticoids affects 1% of the world population and is responsible for secondary adrenal insufficiency for 40% of the patients. In pediatrics, glucocorticoid-induced adrenal insufficiency (GI-AI) diagnosis is based on the Low Dose Synacthen Test (LD-SST). Screening for glucocorticoid-induced adrenal insufficiency implies medical and economical costs due to the necessity of an intravenous line an...

hrp0084p1-106 | Perinatal | ESPE2015

Influence of Nocturnal Glycaemia on Ventricular Repolarisation and Heart Rate Variability in Prepubertal Children with Type 1 Diabetes

Saade Marie-Beatrice , Pecquet Aurelien , Pladys Patrick , Beuchee Alain , Pele Fabienne , Nivot Sylvie , de Kerdanet Marc

Background: ‘Dead in Bed syndrome’ mechanism in childhood diabetes remains unknown. The hypothesis is that a nocturnal hypoglycaemia could lead to arrhythmias related to abnormal ventricular repolarisation.Objective and hypotheses: To look for a relationship between spontaneous fluctuations of nocturnal glycaemia, ventricular repolarization and heart rate variability (HRV) in prepubertal children with type 1 diabetes.Meth...