Aygun Fatih, Varol Fatih, Aktuglu-Zeybek Cigdem, Kiykim Ertugrul, Cam Halit
Department of Pediatric Intensive Care Unit, Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Fatih, Istanbul 34098, Turkey.
Division of Nutrition and Metabolism, Department of Pediatrics, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul 34098, Turkey.
Children (Basel). 2019 Apr 4;6(4):53. doi: 10.3390/children6040053.
: Peritoneal dialysis and continuous renal replacement therapy (CRRT) are the most frequently used treatment modalities for acute kidney injury. CRRT is currently being used for the treatment of several non-renal indications, such as congenital metabolic diseases. CRRT can efficiently remove toxic metabolites and reverse the neurological symptoms quickly. However, there is not enough data for CRRT in children with metabolic diseases. Therefore, we aimed a retrospective study to describe the use of CRRT in metabolic diseases and its associated efficacy, complications, and outcomes. : We performed a retrospective analysis of the records of all patients admitted in the pediatric intensive care unit (PICU) for CRRT treatment. : Between December 2014 and November 2018, 97 patients were eligible for the present study. The age distribution was between 2 days and 17 years, with a mean of 3.77 ± 4.71 years. There were 13 (36.1%) newborn with metabolic diseases. The patients were divided into two groups: CRRT for metabolic diseases and others. There was a significant relationship between the groups, including age ( ≤ 0.001), weight ( = 0.028), blood flow rate ( ≤ 0.001); dialysate rate ( ≤ 0.001), and replacement rate ( ≤ 0.001). The leucine reduction rate was 3.88 ± 3.65 (% per hour). The ammonia reduction rate was 4.94 ± 5.05 in the urea cycle disorder group and 5.02 ± 4.54 in the organic acidemia group. The overall survival rate was 88.9% in metabolic diseases with CRRT. : In particularly hemodynamically unstable patients, CRRT can effectively and quickly reduce plasma ammonia and leucine.
腹膜透析和连续性肾脏替代疗法（CRRT）是急性肾损伤最常用的治疗方式。目前CRRT正用于治疗多种非肾脏适应症，如先天性代谢疾病。CRRT能有效清除有毒代谢产物并迅速逆转神经症状。然而，关于代谢疾病患儿使用CRRT的数据并不充足。因此，我们旨在进行一项回顾性研究，以描述CRRT在代谢疾病中的应用及其相关疗效、并发症和结局。我们对儿科重症监护病房（PICU）中接受CRRT治疗的所有患者的记录进行了回顾性分析。在2014年12月至2018年11月期间，97例患者符合本研究条件。年龄分布在2天至17岁之间，平均年龄为3.77±4.71岁。有13例（36.1%）患有代谢疾病的新生儿。患者分为两组：代谢疾病组和其他组。两组之间存在显著关系，包括年龄（≤0.001）、体重（=0.028）、血流速度（≤0.001）；透析液流速（≤0.001）和置换液流速（≤0.001）。亮氨酸清除率为3.88±3.65（%/小时）。尿素循环障碍组的氨清除率为4.94±5.05，有机酸血症组为5.02±4.54。接受CRRT治疗的代谢疾病患者的总体生存率为88.9%。对于血流动力学特别不稳定的患者，CRRT能有效且迅速地降低血浆氨和亮氨酸水平。
