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[低磷血症性肿瘤性骨软化症]

[Hypophosphatemic oncogenic osteomalacia].

作者信息

Mátyus J, Szebenyi B, Rédl P, Mikita J, Gáspár L, Haris A, Radó J, Kakuk G

机构信息

Debreceni Egyetem, Orvos-, Egészségtudományi Centrum, Altalános Orvostudományi Kar, I. Belklinika.

出版信息

Orv Hetil. 2000 Dec 17;141(51):2785-8.

Abstract

The first case of oncogen osteomalacia in Hungary is reported, to draw the attention of the medical profession to it and to present the new data about its pathomechanism. Pathological hip fracture caused by hypophosphataemic osteomalacia due to isolated renal phosphate wasting was found in a previously healthy 19 years old sportsman. In spite of daily 1.5 micrograms calcitriol treatment and phosphate supplementation, hypophosphataemia persisted for 13 years and he needed regular indometacin medication for his bone pain. During that time an 1.5 cm gingival tumour was found and radically removed. The serum phosphate level returned to normal in a few hours after the operation (preoperative 0.51, after 2, 4 and 8 hours 0.61, 0.68 and 0.79 mmol/l respectively), and remained normal without calcitriol. The histological examination showed epulis with fibroblast and vascular cell proliferation, which has never been previously reported in connection with oncogenic osteomalacia. The pain resolved after 3 months and the bone density became normal in one year. Oncogenic osteomalacia must be considered in every case presenting with atypical hypophosphataemic osteomalacia. Careful dental examination is needed also in the course of search for the underlying tumour. Every tumour-like growth, even the common epulis, has to be operated radically and serum phosphate monitored in the postoperative period in all such cases.

摘要

本文报告了匈牙利首例致癌性骨软化症病例,旨在引起医学界对该病的关注,并介绍其发病机制的新数据。一名此前健康的19岁运动员因孤立性肾磷酸盐消耗导致低磷性骨软化症,进而引发病理性髋部骨折。尽管每天服用1.5微克骨化三醇并补充磷酸盐,但低磷血症仍持续了13年,他需要定期服用吲哚美辛来缓解骨痛。在此期间,发现了一个1.5厘米的牙龈肿瘤并进行了根治性切除。术后几小时内血清磷酸盐水平恢复正常(术前0.51,术后2、4和8小时分别为0.61、0.68和0.79毫摩尔/升),且未服用骨化三醇时仍保持正常。组织学检查显示牙龈瘤伴有成纤维细胞和血管细胞增生,这在以前从未与致癌性骨软化症相关联的病例中报道过。疼痛在3个月后缓解,骨密度在1年内恢复正常。对于每一例表现为非典型低磷性骨软化症病例,都必须考虑致癌性骨软化症。在寻找潜在肿瘤的过程中,也需要仔细进行牙科检查。在所有此类病例中,每一个肿瘤样生长物,即使是常见的牙龈瘤,都必须进行根治性手术,并在术后监测血清磷酸盐水平。

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