Massicotte-Azarniouch David, McLean Laurie, Brown Pierre Antoine
Department of Medicine.
Division of Nephrology, and.
Clin Nephrol Case Stud. 2019 Sep 9;7:54-59. doi: 10.5414/CNCS109788. eCollection 2019.
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.
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,这在当前医学时代已很少见。此类并发症可能表现为继发性甲状旁腺功能亢进(HPT),进而引发罕见的骨矿物质疾病表现,以及因营养不佳和透析过程中水溶性维生素清除导致的严重维生素C缺乏。继发性HPT会引发肾性骨营养不良,可导致面部骨骼弥漫性增大以及出现类似狮面骨病的形态学改变。我们报告了一名36岁、不坚持治疗的女性,她接受慢性透析超过10年,在多年严重HPT及新诊断出严重维生素C缺乏的情况下,出现了进行性、弥漫性面部骨骼增大。影像学检查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,无棕色瘤证据,提示为尿毒症性狮面骨病。在本报告中,我们讨论了继发性HPT的口面部表现以及维生素C缺乏通过改变维生素D代谢可能对肾性骨营养不良发展产生的促进作用。对于接受慢性透析的不坚持治疗患者,应评估其是否存在维生素C缺乏,当此类患者在严重继发性HPT的情况下出现面部特征变形时,应考虑尿毒症性狮面骨病的发生。