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两例因FGF23过量导致的低磷性骨软化症经治疗后骨密度改善的病例

Two Cases of Improved Bone Mineral Density Following Treatment of Hypophosphatemic Osteomalacia Due to FGF23 Excess.

作者信息

McHan Lara, Augustine Marilyn

机构信息

University of Rochester, Strong Memorial Hospital, Rochester, NY 14642, USA.

出版信息

JCEM Case Rep. 2024 Aug 27;2(9):luae073. doi: 10.1210/jcemcr/luae073. eCollection 2024 Sep.

Abstract

Excess fibroblast growth factor-23 (FGF23) causes renal phosphorous wasting and impaired activation of vitamin D leading to osteomalacia. Tumor-induced osteomalacia (TIO) is a rare cause of FGF23-mediated hypophosphatemia. We present 2 patients with FGF23-mediated hypophosphatemia who had low bone mineral density (BMD) at diagnosis and remarkable improvements in BMD with treatment. Patient 1 is a 43-year-old man who had years of progressive pain, difficulty ambulating, and multiple fractures. Patient 2 is a 48-year-old nonverbal man with autism and intellectual disability who had months of progressively declining mobility, presumed pain, and multiple fractures. Workup in both cases revealed hypophosphatemia, evidence of renal phosphorous wasting, and elevated FGF23. Patient 1 was diagnosed with TIO when imaging identified a subcutaneous left flank mass and excision resulted in rapid symptom improvement; he experienced a 96% increase in lumbar spine (LS) BMD after surgery. Patient 2 has had multiple scans over several years, but no FGF23-secreting tumor has been identified. He has been maintained on medical treatment with phosphorous and calcitriol with improvement in functioning and 48% increase in LS BMD. Both patients had improvements in BMD with treatment, with more pronounced improvement in the patient with TIO managed surgically.

摘要

成纤维细胞生长因子23(FGF23)过量会导致肾脏磷流失以及维生素D活化受损,进而引发骨软化症。肿瘤诱导的骨软化症(TIO)是FGF23介导的低磷血症的一种罕见病因。我们报告了2例FGF23介导的低磷血症患者,他们在诊断时骨矿物质密度(BMD)较低,经治疗后BMD有显著改善。患者1是一名43岁男性,多年来一直遭受进行性疼痛、行走困难和多处骨折。患者2是一名48岁患有自闭症和智力残疾的无语言能力男性,数月来行动能力逐渐下降,推测存在疼痛,并发生多处骨折。两例患者的检查均显示低磷血症、肾脏磷流失证据以及FGF23升高。当影像学检查发现患者1左侧腰部皮下肿块,切除后症状迅速改善,从而诊断为TIO;术后他的腰椎(LS)BMD增加了96%。患者2在几年内进行了多次扫描,但未发现分泌FGF23的肿瘤。他一直接受磷和骨化三醇的药物治疗,功能有所改善,LS BMD增加了48%。两名患者经治疗后BMD均有改善,手术治疗的TIO患者改善更为明显。

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