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成年输血依赖型β地中海贫血患者的肾移植:1例具有挑战性的病例报告及文献综述

Kidney transplantation in an adult with transfusion-dependent beta thalassemia: A challenging case report and literature review.

作者信息

Kalamara Tsampika-Vasileia N, Zarkada Evangelia G, Kasimatis Efstratios D, Kofinas Athanasios G, Klonizakis Philippos I, Vlachaki Efthymia C

机构信息

Adults Thalassemia Unit, Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration Hospital, Thessaloniki, Greece.

Department of Nephrology, Aristotle University of Thessaloniki, Hippokration Hospital, Thessaloniki, Greece.

出版信息

Arch Clin Cases. 2023 Jun 23;10(2):97-101. doi: 10.22551/2023.39.1002.10250. eCollection 2023.

Abstract

The markedly increased survival of transfusion-dependent beta thalassemia patients has led to the recognition of new complications, such as renal disorders. Kidney transplantation is nowadays the preferred treatment option for end-stage kidney disease (ESKD). We describe a case of a 49-year-old woman with β-Transfusion Dependent Thalassemia, who developed ESKD as a result of focal segmental glomerulosclerosis and received a deceased-donor kidney transplant following hemodialysis for over a decade. The particular challenges of this case are discussed, including the long-term survival in hemodialysis. Our patient had to overcome multiple obstacles, including hypercoagulability issues presented in the form of thromboembolism, infections, such as hepatitis C and gastroenteritis, and the acute T-cell-mediated rejection, which had to be managed postoperatively. A review of the current literature revealed only one previous report of a thalassemia patient who successfully underwent renal transplantation. More than a year after the transplantation our patient presents with a normal glomerular filtration rate (GFR=62ml/min/1.73m) and creatinine level (Cr=0.96mg/dL) and is transfused every 3 weeks. In conclusion, renal transplantation is possible in patients with TDT and should not be discouraged. Regular transfusions and optimal follow-up for the elimination of post-transplant complications are required.

摘要

依赖输血的β地中海贫血患者生存率显著提高,引发了对新并发症的认识,如肾脏疾病。如今,肾移植是终末期肾病(ESKD)的首选治疗方案。我们描述了一例49岁依赖输血的β地中海贫血女性患者,她因局灶节段性肾小球硬化症发展为ESKD,并在接受十多年血液透析后接受了已故供体肾移植。本文讨论了该病例的特殊挑战,包括血液透析的长期生存情况。我们的患者必须克服多个障碍,包括以血栓栓塞形式出现的高凝问题、感染,如丙型肝炎和肠胃炎,以及术后必须处理的急性T细胞介导的排斥反应。对当前文献的回顾显示,此前仅有一篇关于地中海贫血患者成功接受肾移植的报道。移植一年多后,我们的患者肾小球滤过率正常(GFR = 62ml/min/1.73m²),肌酐水平正常(Cr = 0.96mg/dL),每3周输血一次。总之,TDT患者进行肾移植是可行的,不应被劝阻。需要定期输血并进行最佳随访以消除移植后并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5782/10289047/f78d556ede5e/acc-10-10250-g001.jpg

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