Uremic leontiasis ossea due to secondary hyperparathyroidism complicated by vitamin C deficiency in a non-adherent chronic hemodialysis patient: A case report

D. Massicotte-Azarniouch, L. McLean, P. Brown
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引用次数: 5

Abstract

Non-adherence to medical therapy in patients with end-stage kidney disease (ESKD) can lead to severe metabolic derangements rarely seen in the current medical era. Such complications may take the form of secondary hyperparathyroidism (HPT) leading to rare manifestations of bone mineral disease, and profound vitamin C deficiency from poor nutrition combined with removal of water-soluble vitamins during dialysis. Secondary HPT causes renal osteodystrophy which can lead to diffuse enlargement of the facial skeleton and morphological changes suggestive of leontiasis ossea. We report a 36-year-old, non-adherent woman on chronic dialysis for over 10 years who developed progressive, diffuse facial bone enlargement in the context of years of extreme HPT and newly diagnosed severe vitamin C deficiency. Imaging revealed diffuse hypertrophy of the maxillary and mandibular bones. Histopathology showed extensive fibro-osseous proliferation without evidence of Brown tumor, suggestive of uremic leontiasis ossea. In this report, we discuss the orofacial manifestations of secondary HPT and the possible potentiating role of vitamin C deficiency on the development of renal osteodystrophy through altered vitamin D metabolism. Non-adherent patients on chronic dialysis should be evaluated for vitamin C deficiency, and the development of uremic leontiasis ossea should be considered when such patients present with distortion of facial features in the context of severe secondary HPT.
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慢性血液透析患者继发性甲状旁腺功能亢进合并维生素C缺乏致骨性尿毒症性leontiasis 1例报告
终末期肾病(ESKD)患者不坚持药物治疗可导致严重的代谢紊乱,在当前医学时代很少见。这些并发症可能以继发性甲状旁腺功能亢进(HPT)的形式出现,导致罕见的骨矿物质疾病的表现,以及由于营养不良导致的严重维生素C缺乏,再加上透析期间水溶性维生素的去除。继发性HPT引起肾性骨营养不良,可导致面部骨骼弥漫性扩大和提示骨质疏松的形态学改变。我们报告了一名36岁的非依从性慢性透析妇女,她在多年的极端HPT和新诊断的严重维生素C缺乏症的背景下发展为进行性,弥漫性面部骨肿大。影像学显示上颌骨和下颌骨弥漫性肥大。组织病理学显示广泛的纤维性骨增生,未见棕色肿瘤,提示骨性尿毒症。在本报告中,我们讨论继发性HPT的口腔面部表现,以及维生素C缺乏通过改变维生素D代谢在肾性骨营养不良发展中的可能增强作用。慢性透析的非依从性患者应评估维生素C缺乏症,当这些患者在严重继发性HPT的情况下出现面部特征扭曲时,应考虑是否发展为骨质疏松性尿毒症。
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