内分泌
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
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，导致感染恶化。低三磷酸腺苷（ATP）水平引起的细胞膜不稳定-这可能导致横纹肌溶解，血清肌酸磷酸激酶水平升高，以及溶血性贫血。减少了2,3-双磷酸甘油的产生，增加了对血液中氧气的亲和力。大牙髓腔的牙齿。
诊断方法
低磷血症是通过测量血液中磷酸盐的浓度来诊断的。磷酸盐浓度低于0.81 mmol/L（2.5 mg/dL）被认为可诊断为低磷血症，尽管可能还需要进行其他测试才能确定该疾病的根本原因。
治疗
有标准的磷酸钾静脉注射制剂，通常用于营养不良的人和酗酒者。在无静脉治疗的情况下，口服补充剂也很有用。静脉注射磷酸盐校正期间的监测参数：每次给药后2至4小时应监测磷水平，还应监测血清钾，钙和镁水平。还建议进行心脏监护。
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
影响指数：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
