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终末期肾病合并严重胆汁淤积患儿的长期体外白蛋白透析

Long-lasting extracorporeal albumin dialysis in a child with end-stage renal disease and severe cholestasis.

作者信息

Javouhey Etienne, Ranchin Bruno, Lachaux Alain, Boillot Olivier, Martin Xavier, Floret Daniel, Cochat Pierre

机构信息

Service d'urgences et de réanimation pédiatriques, Hôpital Edouard-Herriot, Hospices Civils de Lyon, 5 place d'Arsonval, Lyon, France.

出版信息

Pediatr Transplant. 2009 Mar;13(2):235-9. doi: 10.1111/j.1399-3046.2008.00946.x. Epub 2008 Apr 22.

DOI:10.1111/j.1399-3046.2008.00946.x
PMID:18433409
Abstract

We report a case of long-lasting MARS therapy as a bridge to liver-kidney transplantation. A 26-month-old girl with congenital tubulointerstitial nephritis and severe liver fibrosis was placed on MARS for an acute-on-chronic liver failure due to sepsis. She underwent two sessions with good tolerance and recovered her previous neurological status. On the basis of pruritus, sleep, and vomiting improvement, repeated MARS sessions were performed to bridge her to combined liver-kidney transplantation. During eight months, 40 sessions were performed with the MARSmini kit and the MARS monitor (Gambro, Lyon, France). The treatment significantly decreased mean pruritus score from 2.2 +/- 0.9 to 0.8 +/- 0.6 night-time awakening and vomiting episodes. Body weight, height, and HC were -3.2, -3.5 and -2.2 SDS before and -1.7, -4.2, -2.0 SDS after eight months on MARS therapy, respectively. The arm circumference/HC ratio increased from 0.28 to 0.31. Mean total bilirubin serum levels were 303 +/- 72 micromol/L before and 214 +/- 42 micromol/L after MARS cycles. Long-lasting MARS dialysis is feasible in children, decreases adverse effects of severe chronic cholestasis, and may help to preserve nutritional status prior to combined liver-kidney transplantation.

摘要

我们报告了一例将长期MARS治疗作为肝肾移植桥梁的病例。一名26个月大的女孩患有先天性肾小管间质性肾炎和严重肝纤维化,因败血症导致慢性肝功能衰竭急性发作,接受了MARS治疗。她耐受了两个疗程,恢复了之前的神经状态。基于瘙痒、睡眠和呕吐症状的改善,进行了多次MARS疗程以过渡到肝肾联合移植。在八个月的时间里,使用MARSmini套件和MARS监测仪(法国里昂甘布罗公司)进行了40次治疗。治疗显著降低了平均瘙痒评分,夜间觉醒次数从2.2±0.9降至0.8±0.6,呕吐发作次数也减少。接受MARS治疗八个月前后,体重、身高和头围的标准差评分分别为-3.2、-3.5和-2.2以及-1.7、-4.2和-2.0。上臂围/头围比值从0.28增加到0.31。MARS治疗周期前后平均血清总胆红素水平分别为303±72微摩尔/升和214±42微摩尔/升。长期MARS透析在儿童中是可行的,可减轻严重慢性胆汁淤积的不良反应,并可能有助于在肝肾联合移植前维持营养状况。

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Long-lasting extracorporeal albumin dialysis in a child with end-stage renal disease and severe cholestasis.终末期肾病合并严重胆汁淤积患儿的长期体外白蛋白透析
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