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肿瘤性骨软化症。42例世界文献综述及2例新病例报告。

Oncogenous osteomalacia. Review of the world literature of 42 cases and report of two new cases.

作者信息

Ryan E A, Reiss E

出版信息

Am J Med. 1984 Sep;77(3):501-12. doi: 10.1016/0002-9343(84)90112-8.

Abstract

The clinical and biochemical data in 42 reported cases of oncogenous osteomalacia are reviewed, and data in two previously unreported cases are recorded. It is likely that the syndrome is more common than suggested by the paucity of reports and may account for a substantial fraction of nonfamilial, adult-onset "idiopathic" osteomalacia. Tumors associated with the syndrome are characteristically benign, of mesenchymal origin, highly vascular, and composed principally of giant and spindle cells. Complete excision of tumors results in cure of the osteomalacia in the majority of patients. The syndrome presumably represents an instance of humor-induced phosphaturia but supporting experimental data are scanty. Plasma levels of 1,25-dihydroxycholecalciferol are uniformly low, and treatment with this metabolite is generally very effective; however, abnormal vitamin D metabolism cannot by itself account for the syndrome.

摘要

回顾了42例报道的肿瘤性骨软化症病例的临床和生化数据,并记录了2例此前未报道病例的数据。该综合征可能比报道的罕见情况更为常见,可能是成人非家族性“特发性”骨软化症的重要组成部分。与该综合征相关的肿瘤通常为良性,起源于间充质,血管丰富,主要由巨细胞和梭形细胞组成。大多数患者肿瘤完全切除后骨软化症得以治愈。该综合征可能是体液性磷尿症的一个实例,但支持性实验数据较少。1,25 - 二羟胆钙化醇的血浆水平普遍较低,用这种代谢产物治疗通常非常有效;然而,维生素D代谢异常本身并不能解释该综合征。

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