Liver Transpl:Ⅰ型原发性高草酸盐尿症:肾移植or肝肾联合移植?

2014-12-15 MedSci MedSci原创

研究背景: Ⅰ型原发性高草酸盐尿症 (Primary hyperoxaluria type 1, PH1) 是一种罕见的常染色体隐性遗传病,法国PH1 的人群患病率为 1.05/百万,其发病机制是肝脏特异的丙氨酸乙醛酸转氨酶( AGT) 缺陷导致乙醛酸转氨生成甘氨酸减少, 而氧化生成草酸增加,由于单纯肾移植(kidney-alone transplantation ,KAT)的疗效很不理想,而肝肾

Figure 1. Renal graft survival according to the initial transplantation strategy.

研究背景:

Ⅰ型原发性高草酸盐尿症 (Primary hyperoxaluria type 1, PH1) 是一种罕见的常染色体隐性遗传病,法国PH1 的人群患病率为 1.05/百万,其发病机制是肝脏特异的丙氨酸乙醛酸转氨酶( AGT) 缺陷导致乙醛酸转氨生成甘氨酸减少, 而氧化生成草酸增加,由于单纯肾移植(kidney-alone transplantation ,KAT)的疗效很不理想,而肝肾联合移植(liver-kidney transplantation ,LKT)风险较大。

研究方法:

非选择性连续收集了54例PH1患者的临床资料(1979年-2010年,10个法国医学中心),根据移植类型将患者分为KAT组和LKT组,分析两组患者的生存率、移植物功能、手术并发症的发生率及死亡率。

结果:

54例PH1患者中行KAT手术21人,LKT手术33人,肝穿确诊33例PH1(55.6%),分子生物学确诊13例PH1(24%),血、尿高草酸盐确诊9例PH1(16.7%)。KAT和LKT的手术并发症的发生率及死亡率无差别,患者10年生存率亦无差别(KAT,21人,生存率70%;LKT,33人,生存率78%),LKT组的肾移植物的10年的存活率较高(LKT 87% vs KAT 13%, P <.001),LKT组4例(12.1%)和KAT组19例(90%)患者失去了初次移植的肾(P <.001),LKT组4例(12%)和KAT组15例(71%)患者再次行肾移植手术(P <.0001)。LKT组和KAT组由于排斥反应导致的终末期肾衰竭的发生率分别为9%和19%(P <.0001),移植肾草酸盐沉积症的复发率分别为LKT组(0例)和KAT组(11例,52%)。

结论:

LKT降低PH1患者的排斥反应率,提高患者移植肾的存活率,可以作为PH1患者终末期肾病的一线治疗方案。

原始出处

Compagnon P1, Metzler P, Samuel D, Camus C, Niaudet P, Durrbach A, Lang P, Azoulay D, Duvoux C, Bayle F, Rivalan J, Merville P, Pascal G, Thervet E, Bensman A, Rostaing L, Deschenes G, Morcet J, Feray C, Boudjema K.Long-Term Results of Combined Liver-Kidney Transplantation for Primary Hyperoxaluria Type 1: The French Experience.Liver Transpl. 2014 Dec;

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    2015-12-01 bsmagic9140
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