Tuna Kubra, Ilkun Olesya, Dziegielewski Peter T, Sharma Anu
Division of Endocrinology, Diabetes and Metabolism, University of Florida College of Medicine, Gainesville, Florida.
Division of Nephrology, University of Florida College of Medicine, Gainesville, Florida.
AACE Clin Case Rep. 2024 Sep 12;11(1):5-9. doi: 10.1016/j.aace.2024.09.001. eCollection 2025 Jan-Feb.
BACKGROUND/OBJECTIVE: Severe progressive overgrowth of the facial bones known as uremic leontiasis ossea (ULO) is a rare complication of resistant hyperparathyroidism in end-stage renal disease (ESRD). The objective of this report is to describe the presentation and treatment of ULO.
A 48-year-old woman with a history of hypertension, coronary artery disease, and ESRD on hemodialysis presented with severe secondary hyperparathyroidism and calciphylaxis. She had significant changes to her face in the last 3 months leading to oropharyngeal dysphagia and difficulty articulating. Physical examination revealed bony overgrowth in her upper jaw and hard palate, widely spaced teeth, and calcinosis cutis. Her parathyroid hormone (PTH), calcium, and phosphorus levels were 5066 pg/mL (normal range, 12-88 pg/mL); 10.0 mg/dL (8.4-10.2 mg/dL); and 5.4 mg/dL (2.7-4.5 mg/dL); respectively. Using a multidisciplinary approach, she successfully underwent a 3.5-gland parathyroidectomy (immediate postoperative PTH level, 600 pg/mL). She was discharged without complication. Pathology showed hypercellular parathyroid glands with reactive changes.
ULO, the most severe form of renal osteodystrophy, results in hypertrophy of the craniofacial skeleton. It carries the risk of significant comorbidities due to cranial nerve compression, respiratory compromise, dysarthria, and dysphagia.
With prolonged, uncontrolled PTH stimulation in ESRD, significant facial disfiguration with disabilities can occur. It is of utmost importance to adhere to guideline-specified PTH targets in persons with ESRD to prevent patient harm from permanent physical deformities.
背景/目的：面部骨骼严重进行性过度生长，即所谓的尿毒症性狮面骨病（ULO），是终末期肾病（ESRD）中难治性甲状旁腺功能亢进的一种罕见并发症。本报告的目的是描述ULO的临床表现及治疗方法。
一名48岁女性，有高血压、冠状动脉疾病史，因ESRD接受血液透析治疗，出现严重继发性甲状旁腺功能亢进和钙化防御。在过去3个月里，她的面部发生了显著变化，导致口咽吞咽困难和言语不清。体格检查发现其上颌骨和硬腭骨质过度生长、牙齿间距增宽以及皮肤钙化。她的甲状旁腺激素（PTH）、钙和磷水平分别为5066 pg/mL（正常范围12 - 88 pg/mL）、10.0 mg/dL（8.4 - 10.2 mg/dL）和5.4 mg/dL（2.7 - 4.5 mg/dL）。采用多学科方法，她成功接受了3.5枚甲状旁腺切除术（术后即刻PTH水平为600 pg/mL）。她出院时无并发症。病理检查显示甲状旁腺细胞增多并伴有反应性改变。
ULO是肾性骨营养不良最严重的形式，可导致颅面骨骼肥大。由于颅神经受压、呼吸功能受损、构音障碍和吞咽困难，它存在显著合并症的风险。
在ESRD患者中，若PTH刺激长期未得到控制，可能会出现严重的面部畸形并导致残疾。对于ESRD患者，严格遵循指南规定的PTH目标以防止患者因永久性身体畸形而受到伤害至关重要。
