hrp0082p1-d2-9 | Adrenals & HP Axis | ESPE2014

Mast Cells in Human Adrenal Gland During Fetal Development

Naccache Alexandre , Duparc Celine , Louiset Estelle , Lefebvre Herve , Castanet Mireille

Background: We previously found that mast cells are present in the human adult adrenal gland with a possible role in the regulation of aldosterone secretion in both physiological conditions and aldosterone-producing adrenocortical adenomas responsible for primary hyperaldosteronism.Objective and Hypotheses: The aim of the present study was to investigate the presence of mast cells in the human fetal adrenal gland, and to provide arguments in favor of the...

hrp0084p3-1044 | Growth | ESPE2015

Value of Alkaline Phosphatase Assay in Short Stature Exploration

Naccache Alexandre , Gayet Claire , Wieliczko Marie Claire , Castanet Mireille

Background: Short stature is a common reason for pediatric endocrinologist consultation, but in many cases, no cause can be identified. Childhood hypophosphatasia has widely variable clinical features from short stature to low bone mineral density with skeletal deformities, and the place of serum alkaline phosphatase (ALP) activity assay could be raised as etiological exploration is not consensualObjective and hypotheses: The aim of our study was to eval...

hrp0089p1-p013 | Adrenals and HPA Axis P1 | ESPE2018

Role of Mast Cells in the Establishment of the Mineralocorticoid Pathway in the Developing Mouse

Naccache Alexandre , Louiset Estelle , Lamaziere Antonin , Thomas Michael , Arabo Arnaud , Lefebvre Herve , Castanet Mireille

Mast cells are known to control mineralocorticoid synthesis and secretion in human normal adrenal gland and aldosterone-producing adenomas through release of serotonin. We recently detected these immune cells in human fetal adrenal from 18 weeks of gestation in the subcapsular layer, in correlation with the expression of steroidogenic enzymes required for aldosterone biosynthesis. This observation suggests the implication of mast cells in the mineralocorticoid synthesizing pat...

hrp0097p1-153 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

Precocious puberty: a new score to assist therapeutic management

Clemenceau Claire , Cuny Ariane , Feray Dagmara , Gayet Claire , Naccache Alexandre , Fraissinet François , Castanet Mireille

Context: Premature breast enlargement before the age of 8 in girls is caused by a broad spectrum from premature thelarche to central precocious puberty. Treatment with GnRH analogues aim to delay either early onset of menstruation with its psychological consequences and/or early epiphyseal fusion. The Indication of treatment is a challenge classically based on the result of the luteinizing hormone (LH)-releasing hormone (LHRH) stimulation test.<p class="ab...

hrp0094fc6.3 | Bone and Mineral Metabolism | ESPE2021

Off-label use of cinacalcet in pediatric primary hyperparathyroidism: a French multi-centre experience

Bernardor Julie , Flammier Sacha , Salles Jean-Pierre , Castanet Mireille , Martinerie Laetitia , Lienhardt-Roussie Anne , Amouroux Cyril , Linglart Agnes , Bacchetta Justine ,

Background: For adult patients who do not meet indications to surgery, the calcimimetic cinacalcet is approved for primary hyperparathyroidism (HPT), decreasing calcium and parathyroid hormone (PTH) levels. This drug is largely used in adults, but may be a challenge in daily practice in children, because of the risk of hypocalcemia, increased QT interval and drug interactions. A few pediatric cases have highlighted its potential interest in children with HPT; ...

hrp0092lb-4 | Late Breaking Posters | ESPE2019

Resistance to Thyroid Hormone Alpha Associated with Early-Onset Severe NASH

Pautasso Valentina , Dumant-Forrest Clémentine , Guerrot Anne-Marie , Fraissinet François , Savagner Frédérique , Castanet Mireille

Introduction: Resistance to thyroid hormone alpha (RTHα) is characterised by tissue-specific hypothyroidism associated with barely normal thyroid function tests. Clinical features include dysmorphic facies, skeletal dysplasia, growth retardation, constipation, dyspraxia and intellectual deficit. Hormonal assessment often shows decreased/low-normal free thyroxine (fT4) and increased/high-normal free triiodothyronine (fT3) concentrations, resulting in a low...

hrp0086p1-p2 | Adrenal P1 | ESPE2016

Mast Cells and Steroidogenesis in the Human Fetal Adrenal

Naccache Alexandre , Louiset Estelle , Duparc Celine , Laquerriere Annie , Patrier Sophie , Renouf Sylvie , Gomez-Sanchez Celso E. , Mukai Kuniaki , Lefebvre Herve , Castanet Mireille

Background: We recently demonstrated the presence of mast cells in human fetal adrenal gland from 18–20 weeks of gestation (WG) within the subcapsular layer. In the adult adrenal, mast cells have been implicated in mineralocorticoid synthesis and secretion especially in aldosterone-producing adenomas. Because similarities exist between tumors and normal fetal development and as cell-to-cell interactions involving immune cells are implicated in organogenesis, we hypothesiz...