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唐氏综合征患者的肾移植:北美儿科肾移植协作研究报告

Renal transplantation in Down syndrome: a report of the North American Pediatric Renal Transplant Cooperative Study.

作者信息

Baqi N, Tejani A, Sullivan E K

机构信息

The SUNY Health Science Center at Brooklyn, New York, USA.

出版信息

Pediatr Transplant. 1998 Aug;2(3):211-5.

Abstract

There are few published reports on renal transplantation in patients with Down syndrome and renal failure. By means of a questionnaire sent to participating renal centers of the North American Pediatric Renal Transplant Cooperative Study, this study identified 14 Down syndrome patients who received renal transplants between January 1987 and November 1995. There were 6 males and 8 females between the ages of 6 and 21 years. Of the group, 8 patients were Caucasian and 6 were of African American ethnicity. Four patients had a primary diagnosis of obstructive uropathy, two had hypoplastic/dysplastic renal disease, two had focal segmental glomerulosclerosis, two had a diagnosis of chronic glomerulonephritis and four patients had unspecified diagnoses. Eight patients received cadaver kidneys whilst the other six had a living related transplant from a parent or sibling. Only one patient had received a prior kidney transplant, ten patients were on dialysis at the time they were transplanted and 4 patients were preemptively transplanted. Standard immunosuppression with Cyclosporine, Imuran and Prednisone was used. In addition, nine patients received induction therapy at the time of transplantation. Follow-up data revealed that 5 grafts (35.7%) had failed, two as a result of rejection and three due to patient death. At the time of data cutoff there were 3 deaths, one due to viral infection and the other two secondary to cardio-pulmonary complications.

摘要

关于唐氏综合征合并肾衰竭患者肾移植的已发表报告很少。通过向北美儿科肾移植协作研究的参与肾中心发送问卷,本研究确定了14例在1987年1月至1995年11月期间接受肾移植的唐氏综合征患者。其中有6名男性和8名女性,年龄在6至21岁之间。该组中,8例患者为白种人,6例为非裔美国人。4例患者的初步诊断为梗阻性尿路病,2例为肾发育不全/发育异常性肾病,2例为局灶节段性肾小球硬化,2例诊断为慢性肾小球肾炎,4例患者诊断不明。8例患者接受了尸体肾移植,另外6例接受了来自父母或兄弟姐妹的活体亲属移植。只有1例患者曾接受过肾移植,10例患者在移植时正在接受透析,4例患者接受了先发制人的移植。使用了环孢素、硫唑嘌呤和泼尼松的标准免疫抑制方案。此外,9例患者在移植时接受了诱导治疗。随访数据显示,5个移植物(35.7%)失败,2个因排斥反应,3个因患者死亡。在数据截止时,有3例死亡,1例因病毒感染,另外2例继发于心肺并发症。

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