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Successful ABO-Incompatible Living-Donor Renal Transplant Without Splenectomy For Renal Coloboma Syndrome: A Case Report

机译:不进行脾切除术的成功ABO不相容活体供体肾移植治疗肾小球瘤综合征:病例报告

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To our knowledge, this is the first report of an ABO-incompatible living-donor renal transplant without a splenectomy performed in a patient with renal coloboma syndrome, a rare disorder caused by PAX2 gene mutations, and that presents with renal and optic nerve hypodysplasia and disorders of the central nervous system. Many patients with renal coloboma syndrome develop end-stage renal disease requiring renal replacement therapy. Few reports of a well-defined course of renal transplant for coloboma syndrome have been published. We treated a 22-year-old man who had end-stage renal disease from renal coloboma syndrome. We performed an ABO-incompatible living-donor renal transplant with a kidney donated by his father. Two years after the transplant, the patient has good preserved renal function, and his compliance with the immunosuppressive regimen was good.
机译:据我们所知,这是首次报道患有肾小球瘤综合征(一种由PAX2基因突变引起的罕见疾病)的患者,未进行脾切除术而进行的ABO不相容的活体供体肾移植,并伴有肾和视神经发育不良和中枢神经系统疾病。许多患有肾小球瘤综合征的患者会发展为终末期肾脏疾病,需要进行肾脏替代治疗。关于肾小球瘤综合征的明确定义的肾脏移植过程的报道很少。我们治疗了一名患有肾小球瘤综合征终末期肾脏疾病的22岁男性。我们用他父亲捐赠的肾脏进行了ABO不相容的活体供肾移植。移植后两年,患者的肾脏功能得以良好保存,其对免疫抑制方案的依从性良好。

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