Hypophyse

J Léger (Professeur de pédiatrie), P Czernichow (Professeur de pédiatrie)
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Abstract

Ante- and post-hypophysis are the two constituents of the hypophysis. The ante-hypophysis synthesizes and secretes mainly the growth hormone, thyrotropin, prolactin, adrenocorticotropic hormones and the gonadotrophins. The vasopressin and oxytocin hormones are secreted by the post-hypophysis. The hypophysis development and differentiation are controlled by a chain of different factors that involve numerous signalisation genes from adjacent structures, in addition to specific transcription factors, to date insufficiently known. The regulation and mode of action of the various hormones are now well identified. Activating and inhibiting hormones synthesized in the hypothalamus regulate the functioning of ante-hypophysis cells. They act through specific cellular receptors. In children, hypothalamus-hypophysis hypofunction is far more frequently observed than hyperfunction. Hypopituitarisms are serious diseases which, if untreated, may have irreversible consequences such as severe nanism in case of somatotropic insufficiency, mental retardation in case of thyrotropic insufficiency, lack of puberty development and sterility in case of gonadotropic insufficiency, and even death in case of corticotropic insufficiency. The diagnosis of hypophysis insufficiency is based on clinical data, and results of functional investigations, and brain magnetic resonance imaging. Treatments are substitutive, given for life, and concerned patients necessitate lifelong medical management. Hypophysis insufficiency may be either isolated or multiple, either acquired (mainly in relation with a tumoral or infiltrative process in the hypothalamus-hypophysis area), or congenital. Various types of human congenital insufficiency (isolated or combined) due to a genetic cause have been described these last years. However, the molecular mechanisms that produce the major part of genetic hypopituitarisms remain to be elucidated.

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垂体前和垂体后是垂体的两个组成部分。垂体前主要合成和分泌生长激素、促甲状腺激素、催乳素、促肾上腺皮质激素和促性腺激素。垂体后垂体分泌抗利尿激素和催产素。垂体的发育和分化是由一系列不同的因素控制的,这些因素包括来自相邻结构的许多信号基因,以及迄今为止尚未充分了解的特定转录因子。各种激素的调节和作用方式现在已经很好地确定了。激活和抑制下丘脑合成的激素调节垂体前细胞的功能。它们通过特定的细胞受体起作用。在儿童中,下丘脑-垂体功能减退远比功能亢进更常见。垂体功能减退症是一种严重的疾病,如果不加以治疗,可能会造成不可逆转的后果,如:促性腺功能不全时导致严重的发育迟缓,促性腺功能不全时导致青春期发育迟缓和不育,促皮质功能不全时甚至导致死亡。垂体功能不全的诊断是基于临床资料、功能检查结果和脑磁共振成像。治疗是替代的,是终身的,关心的病人需要终身的医疗管理。垂体功能不全可能是孤立的,也可能是多发的,可能是后天的(主要与下丘脑-垂体区肿瘤或浸润过程有关),也可能是先天性的。由于遗传原因,各种类型的人类先天性功能不全(单独或合并)已被描述为最近几年。然而,产生大部分遗传性垂体功能低下的分子机制仍有待阐明。
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