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肾上腺皮质发育和相关疾病导致肾上腺皮质功能不全。

Adrenal cortex development and related disorders leading to adrenal insufficiency.

机构信息

Pediatric Endocrinology, Diabetology and Metabolism, Department of Pediatrics, Bern and Department of BioMedical Research, University Hospital Inselspital, University of Bern, 3010, Bern, Switzerland.

出版信息

Mol Cell Endocrinol. 2021 May 1;527:111206. doi: 10.1016/j.mce.2021.111206. Epub 2021 Feb 16.

DOI:10.1016/j.mce.2021.111206
PMID:33607267
Abstract

The adult human adrenal cortex produces steroid hormones that are crucial for life, supporting immune response, glucose homeostasis, salt balance and sexual maturation. It consists of three histologically distinct and functionally specialized zones. The fetal adrenal forms from mesodermal material and produces predominantly adrenal C steroids from its fetal zone, which involutes after birth. Transition to the adult cortex occurs immediately after birth for the formation of the zona glomerulosa and fasciculata for aldosterone and cortisol production and continues through infancy until the zona reticularis for adrenal androgen production is formed with adrenarche. The development of this indispensable organ is complex and not fully understood. This article gives an overview of recent knowledge gained of adrenal biology from two perspectives: one, from basic science studying adrenal development, zonation and homeostasis; and two, from adrenal disorders identified in persons manifesting with various isolated or syndromic forms of primary adrenal insufficiency.

摘要

成人肾上腺皮质产生的类固醇激素对生命至关重要,支持免疫反应、葡萄糖稳态、盐平衡和性成熟。它由三个在组织学上明显不同且功能专门化的区域组成。胎儿肾上腺由中胚层物质形成,并从其胎儿区产生主要的肾上腺 C 类固醇,该区域在出生后退化。向成人皮质的过渡在出生后立即发生,用于形成产生醛固酮和皮质醇的球状带和束状带,并在婴儿期持续进行,直到形成产生肾上腺雄激素的网状带,这一过程伴随着肾上腺功能初现。这个不可或缺的器官的发育非常复杂,目前还不完全清楚。本文从两个角度概述了从基础科学研究肾上腺发育、分区和稳态,以及从表现出各种孤立或综合征形式原发性肾上腺功能不全的个体中确定的肾上腺疾病两个方面获得的关于肾上腺生物学的最新知识。

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