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Hypophosphatemia

内分泌

关键词内分泌 疾病 电解质紊乱

词汇介绍

拓展阅读

解析

Hypophosphatemia   英 /haipəufɔsfə'ti:miə/

释    义   n. 血磷酸盐过少;低磷酸盐血

例    句   Objective To study the relation between postoperative total parenteral nutrition(TPN) and hypophosphatemia and relation between hypophosphatemia and infection following gastrointestinal damage. 目的探讨胃肠损伤术后全静脉营养和低磷血症的关系以及低磷血症和胃肠损伤术后并发感染的关系。

概述

低磷血症是一种电解质紊乱,血液中存在的低水平磷酸盐。症状可能包括虚弱,呼吸困难和食欲不振。并发症可能包括癫痫发作,昏迷,横纹肌溶解或骨骼软化。原因包括酒精中毒,营养不良,糖尿病酮症酸中毒,烧伤,换气过度,和服用某些药物,它也可能发生在甲状旁腺功能亢进,甲状腺功能低下和库欣综合征的患者中。症状和体征肌肉功能障碍和无力-这种情况发生在主要的肌肉中,但也可能表现为:复视,低心排量,吞咽困难和由于呼吸肌无力而导致的呼吸抑制。精神状态变化-可能从烦躁不安到严重的困惑,瞻望和昏迷。白细胞功能障碍,导致感

FGF23 and its role in X-linked hypophosphatemia-related morbidity复制标题

FGF23及其在X连锁低磷血症相关发病率中的作用

发表时间:2019-02-26

影响因子:3.7

作者: Signe Sparre Beck-Nielsen

期刊:Orphanet J Rare Dis

Downregulation of PHEX in XLH increases skeletal OPN deposition which contributes to local inhibition of mineralisation. Meanwhile, elevated levels of serum FGF23 increase urinary phosphate excretion by downregulating renal sodium-phosphate transporters, and limit intestinal phosphate absorption by restricting active vitamin D synthesis to levels that are abnormally low or normal despite hypophosphatemia. Since phosphate insufficiency and inappropriately low levels of calcitriol [also known as 1,25(OH)2D or active vitamin D] contribute to many symptoms of XLH, conventional therapy involves supplementation with oral phosphate and calcitriol or calcitriol analogues (commonly alfacalcidol). This can correct lower limb deformities, promote growth, and improve oral health, with earlier treatment leading to better results. However, conventional therapy insufficiently corrects the biochemistry and symptoms of XLH, and can further increase serum FGF23 levels. Conventional therapy has also been associated with adverse effects including secondary hyperparathyroidism, nephrocalcinosis, nephrolithiasis, and cardiovascular abnormalities. Although hypophosphatemia is the primary link between elevated FGF23 and the pathophysiology of XLH, FGF23 has recently been proposed to also contribute to XLH via other molecular mechanisms. This review describes the central role of FGF23 in XLH pathophysiology, outlining evidence that links upregulation of FGF23 to manifestations of XLH through various molecular pathways (outlined in Fig. 2). FGF23 is introduced along with its direct regulators and receptors, followed by a brief discussion of the dysregulation of serum FGF23 in various diseases of hypophosphatemia; animal models of these diseases are also described since they are essential for understanding molecular mechanisms involved in the pathology of XLH. Finally, the manifestations of XLH are grouped by molecular mechanism and discussed, with any potential involvement of FGF23 highlighted.

译文

XLH中PHEX的下调会增加骨骼肌OPN的沉积,从而有助于局部抑制矿化。同时,升高的血清FGF23含量通过下调肾脏磷酸钠转运蛋白而增加了尿磷酸盐的排泄,并通过将活性维生素D的合成限制在异常低磷或低磷的水平,从而限制了肠道磷酸盐的吸收。由于磷酸盐不足和钙三醇[也称为1,25(OH)2D或活性维生素D]含量过低会导致XLH的许多症状,因此传统疗法包括补充口服磷酸盐和钙三醇或钙三醇类似物(通常为阿法骨化醇)。这可以纠正下肢畸形,促进生长,并改善口腔健康,早期治疗可带来更好的效果。但是,常规疗法不足以纠正XLH的生化和症状,并可能进一步增加血清FGF23水平。常规疗法还与不良反应有关,包括继发性甲状旁腺功能亢进,肾钙化,肾结石和心血管异常。尽管低磷血症是FGF23升高与XLH病理生理之间的主要联系,但最近有人提出FGF23也可通过其他分子机制对XLH做出贡献。这篇综述描述了FGF23在XLH病理生理中的核心作用,概述了通过多种分子途径将FGF23上调与XLH表现联系起来的证据(图2概述)。引入FGF23及其直接调节剂和受体,然后简要讨论各种低磷血症疾病中血清FGF23的失调。还描述了这些疾病的动物模型,因为它们对于理解XLH病理学涉及的分子机制至关重要。最后,通过分子机理对XLH的表现进行了分组并进行了讨论,并着重指出了FGF23的潜在参与。