[FGF23]肿瘤诱导骨软化症。

Q4 Medicine
S A Gronskaia, Zh E Belaya, G A Melnichenko
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引用次数: 1

摘要

肿瘤性骨软化是一种罕见的后天性疾病。病因是间充质肿瘤分泌成纤维细胞生长因子23 (FGF23)。过量的FGF 23会破坏磷和维生素D的代谢,从而导致严重的副肿瘤综合征,表现为多发骨折、骨骼剧烈疼痛和全身性肌病。对于癌性骨软化,根治性切除肿瘤是完全治愈的可能。不幸的是,局部化、小的形成和罕见的发生频率导致这种疾病在很长一段时间内未被发现,并导致严重的致残后果。一步一步的诊断方法可以改善治疗结果。首先进行彻底的记忆,然后进行功能可视化,并通过肿瘤的解剖可视化确认诊断。之后,选择的方法是手术治疗。如果不能切除,则需要使用活性代谢产物维生素D和磷盐进行保守治疗。新的治疗方法,如FGF23抗体或FGF受体泛抑制剂,正在积极发展。这篇文章提供了一个概述现代方法的诊断和治疗这种疾病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[FGF23 tumor induced osteomalacia].

Tumor induced osteomalacia is a rare acquired disease. The cause is a mesenchymal tumor secreting fibroblast growth factor 23 (FGF23). An excessive amount of FGF 23 disrupts the metabolism of phosphorus and vitamin D, which leads to severe paraneoplastic syndrome, manifested in the form of multiple fractures, severe pain in the bones and generalized myopathy. With oncogenic osteomalacia, a complete cure is possible with radical resection of the tumor. Unfortunately, localization, small size of formations and rare frequency of occurrence lead to the fact that the disease remains unrecognized for a long time and leads to severe, disabling consequences. A step-by-step approach to diagnosis improves treatment outcomes. First, a thorough anamnesis is collected, then functional visualization is performed and the diagnosis is confirmed by anatomical visualization of the tumor. After that, the method of choice is a surgical treatment. If resection is not possible, then conservative therapy with active metabolites of vitamin D and phosphorus salts is indicated. New therapeutic approaches, such as the antibody to FGF23 or the pan-inhibitor of receptors to FGF, are actively developing. This article provides an overview of modern approaches to the diagnosis and treatment of this disease.

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来源期刊
Problemy endokrinologii
Problemy endokrinologii Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
1.40
自引率
0.00%
发文量
59
期刊介绍: Since 1955 the “Problems of Endocrinology” (or “Problemy Endocrinologii”) Journal publishes timely articles, balancing both clinical and experimental research, case reports, reviews and lectures on pressing problems of endocrinology. The Journal is aimed to the most topical issues of endocrinology: to chemical structure, biosynthesis and metabolism of hormones, the mechanism of their action at cellular and molecular level; pathogenesis and to clinic of the endocrine diseases, new methods of their diagnostics and treatment. The Journal: features original national and foreign research articles, reflecting world endocrinology development; issues thematic editions on specific areas; publishes chronicle of major international congress sessions and workshops on endocrinology, as well as state-of-the-art guidelines; is intended for scientists, endocrinologists diabetologists and specialists of allied trade, general practitioners, family physicians and pediatrics.
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