两例因 FGF23 过量而导致的低磷血症骨软化症患者在接受治疗后骨密度得到改善

JCEM case reports Pub Date : 2024-08-27 eCollection Date: 2024-09-01 DOI:10.1210/jcemcr/luae073
Lara McHan, Marilyn Augustine
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引用次数: 0

摘要

过量的成纤维细胞生长因子-23(FGF23)会导致肾磷消耗和维生素 D 的激活受损,从而导致骨软化症。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。我们介绍了两名 FGF23 介导的低磷血症患者,他们在确诊时骨矿物质密度(BMD)较低,经过治疗后 BMD 明显改善。患者 1 是一名 43 岁的男性,多年来一直有进行性疼痛、行走困难和多处骨折。患者 2 是一名 48 岁的非语言男性,患有自闭症和智力障碍,数月来活动能力逐渐下降,推测为疼痛和多处骨折。两个病例的检查结果均显示低磷血症、肾磷消耗证据和 FGF23 升高。患者 1 在影像学检查中发现左翼皮下肿块,经切除后症状迅速改善,被诊断为 TIO;术后他的腰椎 (LS) BMD 增加了 96%。患者 2 几年来接受了多次扫描,但没有发现分泌 FGF23 的肿瘤。他一直接受磷和钙三醇药物治疗,功能有所改善,腰椎 BMD 增加了 48%。通过治疗,两名患者的 BMD 都有所改善,而通过手术治疗的 TIO 患者的改善更为明显。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Two Cases of Improved Bone Mineral Density Following Treatment of Hypophosphatemic Osteomalacia Due to FGF23 Excess.

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.

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