Two‐step transplantation for primary hyperoxaluria: A winning strategy to prevent progression of systemic oxalosis in early onset renal insufficiency cases
Two‐step transplantation for primary hyperoxaluria: A winning strategy to prevent progression of systemic oxalosis in early onset renal insufficiency cases
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原发性高草酸尿症的两步移植：预防早期肾功能不全病例中系统性草酸中毒进展的成功策略
DOI：
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发表时间：
2015
影响因子：
1.3
通讯作者：
M. Kasahara
中科院分区：
文献类型：
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作者：
K. Sasaki;S. Sakamoto;H. Uchida;T. Shigeta;M. Matsunami;H. Kanazawa;A. Fukuda;A. Nakazawa;Mai Sato;S. Ito;R. Horikawa;T. Yokoi;N. Azuma;M. Kasahara
已经提出了几种PH1的移植策略，并进行LT以纠正代谢缺陷。PH1患者通常患有ESRD，需要同时进行LKT，由于缺乏合适的器官供体，导致等待时间长。5例PH1患者在我们研究所接受了LDLT。5例患者中有3例在LDLT前接受透析，而另外2例患者被归类为CKD 3期。一个孤立的LDLT成功地进行了所有，但我们的第一个案件，谁有复杂的术后过程，并因此死于败血症后再次移植。LDLT后CKD 3期患者的肾功能得到保留。另一方面，我们的第二例ESRD病例在LDLT后6个月成功进行了LDKT，我们的婴儿病例正在等待随后的KT，在早期确定PD后没有任何LDLT后并发症。总之，两步移植策略可能是PH 1患者的救命选择，即使是全身性草酸盐中毒的小婴儿也可能是可能的。在等待后续KT时，应从RRT的长期需求角度考虑尽早恢复PD。
Several transplant strategies for PH1 have been proposed, and LT is performed to correct the metabolic defects. The patients with PH1 often suffer from ESRD and require simultaneous LKT, which leads to a long wait due to the shortage of suitable organ donors. Five patients with PH1 underwent LDLT at our institute. Three of the five patients were under dialysis before LDLT, while the other two patients were categorized as CKD stage 3. An isolated LDLT was successfully performed in all but our first case, who had complicated postoperative courses and consequently died due to sepsis after retransplantation. The renal function of the patients with CKD stage 3 was preserved after LDLT. On the other hand, our second case with ESRD underwent successful LDKT six months after LDLT, and our infant case is waiting for the subsequent KT without any post‐LDLT complications after the early establishment of PD. In conclusion, a two‐step transplant strategy may be needed as a life‐saving option for patients with PH1 and may be possible even in small infants with systemic oxalosis. While waiting for a subsequent KT, an early resumption of PD should be considered from the perspective of the long‐term requirement of RRT.
影响因子：
19.6
作者：
Hoppe B;Beck BB;Milliner DS
通讯作者：
Milliner DS