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11.15. Genotype, mortality, morbidity, and outcomes of 3[beta]-hydroxysteroid dehydrogenase deficiency in Algeria

A Ladjouze , M Donaldson , I Plotton , N Djenane , K Mohammedi , V Tardy-Guidollet , D Mallet , K Boulesnane , Z Bouzerar , Y Morel , F Roucher-Boulez

Brief summary: This study describes the genetic and clinical characteristics of 3βHSD2 deficiency in children seen at a single center in Algeria. It describes clinical outcomes, including the frequency of adrenal rest tumors in this population.3β-hydroxysteroid dehydrogenase 2 deficiency (3βHSD2) is a rare form of congenital adrenal hyperplasia. This mixed longitudinal and cross-sectional study was performed in a single Algerian center bet...

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ey0015.8-18 | New Paradigms | ESPEYB15

8.18 PKA signaling drives reticularis differentiation and sexually dimorphic adrenal cortex renewal

T Dumontet , I Sahut-Barnola , A Septier , N Montanier , I Plotton , F Roucher-Boulez , V Ducros , AM Lefrançois-Martinez , JC Pointud , M Zubair , KI Morohashi , DT Breault , P Val , A Martinez

To read the full abstract: JCI Insight. 2018;3(2). pii: 98394The (human) adrenal cortex undergoes massive changes in structure and function from fetal to postnatal life, with the first consisting of a small outer definitive zone and a larger inner fetal zone, and the latter finally consisting of three distinct layers, namely the zona glomerulosa (ZG), fasciculata (ZF) and reticularis (ZR). Ho...

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ESPE Yearbook of Paediatric Endocrinology

11.15. Genotype, mortality, morbidity, and outcomes of 3[beta]-hydroxysteroid dehydrogenase deficiency in Algeria

8.18 PKA signaling drives reticularis differentiation and sexually dimorphic adrenal cortex renewal

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