Tsai I-Jung, Hwu Wuh-Liang, Huang Shu-Chien, Lee Ni-Chung, Wu En-Ting, Chien Yin-Hsiu, Tsau Yong-Kwei
Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, 7 Chung-Shan South Road, Taipei, 10016, Taiwan.
Pediatr Nephrol. 2014 Jan;29(1):111-6. doi: 10.1007/s00467-013-2609-2. Epub 2013 Sep 8.
Intermittent hemodialysis (IHD) is the most efficient form of renal replacement therapy (RRT) for removing toxic substances from patients' bodies. However, the efficacy and safety of IHD in infants and young children with inborn errors of metabolism are still not clear.
This retrospective study included patients with urea cycle disorders, maple syrup urine disease, and methylmalonic acidemia who received IHD or non-IHD RRT at our hospital between 2001 and 2012 to remove ammonia, leucine, or methylmalonic acid. Both the efficacy and safety of the RRT were evaluated.
Thirty-five courses of RRT, including 25 courses of IHD and ten courses of non-IHD RRT, for 15 patients were included in the analysis. Before 2006, non-IHD RRT procedures, including peritoneal dialysis (PD) and continuous venous-venous hemofiltration (CVVH), were the most often used; from 2006 onwards IHD was used. There was one procedure-unrelated death. Catheter penetration occurred in one course of IHD. The efficacy data revealed that both the median duration of dialysis and the median 50 % toxin reduction time were shorter in IHD than in non-IHD RRT.
In infants and young children with inborn errors of metabolism, IHD is safe and more efficient than non-IHD RRT at removing toxins.
间歇性血液透析（IHD）是从患者体内清除有毒物质的最有效肾脏替代疗法（RRT）形式。然而，IHD在患有先天性代谢缺陷的婴幼儿中的疗效和安全性仍不明确。
这项回顾性研究纳入了2001年至2012年间在我院接受IHD或非IHD RRT以清除氨、亮氨酸或甲基丙二酸的尿素循环障碍、枫糖尿症和甲基丙二酸血症患者。对RRT的疗效和安全性进行了评估。
分析纳入了15例患者的35个RRT疗程，包括25个IHD疗程和10个非IHD RRT疗程。2006年之前，最常使用的是非IHD RRT程序，包括腹膜透析（PD）和持续静脉-静脉血液滤过（CVVH）；从2006年起开始使用IHD。有1例与操作无关的死亡。在1个IHD疗程中发生了导管穿透。疗效数据显示，IHD的透析中位持续时间和毒素减少50%的中位时间均短于非IHD RRT。
在患有先天性代谢缺陷的婴幼儿中，IHD在清除毒素方面比非IHD RRT更安全、更有效。
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