Karmegam Sathish, Shetty Anupkumar
Methodist Dallas Medical Center and Dallas Nephrology Associates, Dallas, Texas, USA.
Hemodial Int. 2017 Oct;21 Suppl 2:S62-S66. doi: 10.1111/hdi.12599.
A 60-year-old African American man with end stage renal disease on hemodialysis (HD) for the past 2.5 years developed severe hyperparathyroidism. Other past medical history included atrial fibrillation, type II diabetes mellitus, hypertension, gout, pericardial effusion needing pericardial window, deep vein thrombosis, mitral insufficiency, and cardiomyopathy with implantable cardioversion device placement. His parathyroid hormone (PTH) level peaked at 4,191 pg/mL despite being on cinacalcet, sevelamer, and paricalcitol. He underwent a subtotal parathyroidectomy in January 2015, after which his PTH levels dropped to 184 pg/mL. Approximately 4 weeks later he developed extensive, painful necrotic skin lesions in both his lower extremities and buttocks, suggestive of calciphylaxis which was confirmed by tissue biopsy. The patient was treated with elaborate wound care, wound debridements, increased dialysis dose, and IV sodium thiosulfate (STS) during hemodialysis. Besides STS, he was treated with narcotics, gabapentin, topical lidocaine on intact skin, and oral steroids for pain control. Even though his lesions improved initially, he deteriorated due to recurrent sepsis, respiratory failure, and prolonged hospitalization which culminated in stopping dialysis before he passed away. Calciphylaxis, or calcific uremic arteriolopathy, is a life-threatening complication of end stage renal disease. Treatment of this condition is multidisciplinary which includes elaborate wound care, increasing dialysis dose, and discontinuing vitamin D supplements and calcium containing phosphate binders. Even though STS has been recommended off-label, several studies have shown promising results with resolution of lesions. Thus, sodium thiosulfate has become the mainstay of treatment. Parathyroidectomy is a recommended modality of treatment in those with high PTH levels. Our case was unique in that calciphylaxis developed after subtotal parathyroidectomy. We believe that this is due to a decreased PTH level and decreasing bone turnover which resulted in more circulating calcium facilitating vascular and soft tissue calcification. The exact mechanism of developing calciphylaxis after parathyroidectomy is unknown. Even though parathyroidectomy is an effective treatment for calciphylaxis, clinicians should be aware that it can rarely present after parathyroidectomy.
一名60岁的非裔美国男性，过去2.5年一直接受终末期肾病的血液透析治疗，并发严重甲状旁腺功能亢进。其他既往病史包括心房颤动、II型糖尿病、高血压、痛风、因心包积液需行心包开窗术、深静脉血栓形成、二尖瓣关闭不全以及植入心脏复律除颤器的心肌病。尽管服用了西那卡塞、司维拉姆和帕立骨化醇，他的甲状旁腺激素（PTH）水平仍高达4191 pg/mL。2015年1月，他接受了甲状旁腺次全切除术，术后PTH水平降至184 pg/mL。大约4周后，他双下肢和臀部出现广泛、疼痛的坏死性皮肤病变，提示钙化防御，经组织活检确诊。患者接受了精心的伤口护理、伤口清创、增加透析剂量以及血液透析期间静脉注射硫代硫酸钠（STS）治疗。除了STS，还给他使用了麻醉药、加巴喷丁、完整皮肤上涂抹利多卡因以及口服类固醇来控制疼痛。尽管他的病变最初有所改善，但由于反复发生败血症、呼吸衰竭以及长期住院，病情恶化，最终在去世前停止了透析。钙化防御，即钙化性尿毒症小动脉病，是终末期肾病的一种危及生命的并发症。这种疾病的治疗需要多学科协作，包括精心的伤口护理、增加透析剂量以及停用维生素D补充剂和含钙的磷结合剂。尽管STS已被推荐用于非适应证治疗，但多项研究表明其对病变的消退有良好效果。因此，硫代硫酸钠已成为主要治疗方法。甲状旁腺切除术是PTH水平高的患者推荐的治疗方式。我们的病例独特之处在于钙化防御发生在甲状旁腺次全切除术后。我们认为这是由于PTH水平降低和骨转换减少，导致更多循环钙促进血管和软组织钙化。甲状旁腺切除术后发生钙化防御的确切机制尚不清楚。尽管甲状旁腺切除术是治疗钙化防御的有效方法，但临床医生应意识到它在甲状旁腺切除术后很少出现。
