From the Department of Transplant Nephrology, Einstein Medical Center, Philadelphia, Pennsylvania, USA.
Exp Clin Transplant. 2021 Apr;19(4):382-385. doi: 10.6002/ect.2017.0123. Epub 2018 Apr 9.
Calciphylaxis is a rare disease characterized by calcification of small- to medium-sized blood vessels in the dermis and subcutaneous fat, resulting in cutaneous necrosis. Although most commonly shown in patients with end-stage kidney disease, it has also been reported in patients with other diseases, including alcoholic cirrhosis and malignancies. Here, we report an unusual case of calciphylaxis in an orthotopic liver transplant recipient with acute kidney injury. The patient, a 43-year-old white female with a history of type 2 diabetes mellitus, alcoholic cirrhosis, and normal kidney function, presented with decompensated liver disease and hepatorenal syndrome; she no longer responded to medical treatment and required treatment with dialysis. Ten days after admission, she underwent liver transplant, resulting in improved liver function tests. She had acute tubular necrosis (creatinine peak: 325 μmol/L) from sustained hypotension during and after surgery, which required 4 sessions of dialysis over 2weeks. Six weeks after her transplant, she developed painful, nonulcerating, erythematous plaques over her shins and thighs. Skin biopsy of the lesions showed calciphylaxis, calcium deposits, and thrombotic vasculopathy. She also developed severe hypercalcemia (calcium level of 2.75 mmol/L) from immobility, which required treatment with a bisphosphonate and hemodialysis. The lesions improved 6 weeks later, and her renal function returned to normal. Calciphylaxis diagnosed in an orthotopic liver transplant recipient with acute kidney injury has not been previously reported. We hypothesize that her chronic inflammatory state caused down-regulation and low levels of fetuin A and protein C. She also had other risk factors, including hypoalbuminemia, obesity, systemic glucocorticoids, and alcoholic liver disease. Calciphylaxis can occur in patients with alcoholic cirrhosis and acute renal failure even after liver transplant. Further studies into the pathogenesis of this disease may help us understand why it develops in these patients and not others with the same risk factors.
钙化防御是一种罕见的疾病，其特征是真皮和皮下脂肪中小至中等大小的血管钙化，导致皮肤坏死。虽然最常见于终末期肾病患者，但也有报道见于其他疾病患者，包括酒精性肝硬化和恶性肿瘤。在这里，我们报告了一例在原位肝移植受者中发生的不常见的钙化防御病例，该患者患有急性肾损伤。该患者为 43 岁白人女性，患有 2 型糖尿病、酒精性肝硬化和正常肾功能，因肝功能失代偿和肝肾综合征就诊；她对药物治疗不再有反应，需要接受透析治疗。入院 10 天后，她接受了肝移植，肝功能检查得到改善。她在手术中和手术后因持续低血压导致急性肾小管坏死（肌酐峰值：325μmol/L），需要在 2 周内进行 4 次透析。肝移植后 6 周，她的小腿和大腿出现疼痛、非溃疡性、红斑性斑块。病变皮肤活检显示钙化防御、钙沉积和血栓性血管病变。她还因长期卧床不动而出现严重高钙血症（血钙水平 2.75mmol/L），需要使用双膦酸盐和血液透析治疗。6 周后，病变得到改善，肾功能恢复正常。以前没有报道过在急性肾损伤的原位肝移植受者中诊断出的钙化防御。我们假设她的慢性炎症状态导致胎球蛋白 A 和蛋白 C 的下调和水平降低。她还存在其他危险因素，包括低白蛋白血症、肥胖、全身糖皮质激素和酒精性肝病。即使在肝移植后，钙化防御也可发生于酒精性肝硬化和急性肾衰竭患者。对这种疾病发病机制的进一步研究可能有助于我们了解为什么它会在这些患者中发生，而不是在其他具有相同危险因素的患者中发生。
