ORIGINAL ARTICLE Efficacy and safety of intermittent hemodialysis in infants and young children with inborn errors of metabolism I-Jung Tsai & Wuh-Liang Hwu & Shu-Chien Huang & Ni-Chung Lee & En-Ting Wu & Yin-Hsiu Chien & Yong-Kwei Tsau Received: 1 April 2013 /Revised: 12 August 2013 /Accepted: 14 August 2013 /Published online: 8 September 2013 # IPNA 2013 Abstract Background Intermittent hemodialysis (IHD) is the most ef- ficient 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. Methods 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. Results 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 proce- dures, 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 reduc- tion time were shorter in IHD than in non-IHD RRT. Conclusions In infants and young children with inborn errors of metabolism, IHD is safe and more efficient than non-IHD RRT at removing toxins. Keywords Renal replacement therapy . Intermittent hemodialysis . Inborn errors of metabolism . Urea cycle disorders . Maple syrup urine disease . Methylmalonic acidemia Introduction Acute metabolic decompensation in patients with inborn errors of metabolism (IEM) is a medical emergency that requires immediate renal replacement therapy (RRT) to remove the toxic substances from the body to prevent death or permanent brain damage [1–3]. The common forms of RRT include peritoneal dialysis (PD), continuous venous-venous hemofiltration (CVVH), continuous arteriovenous hemofiltration (CAVH), and intermittent hemodialysis (IHD). Although IHD is the most efficient form of RRT to remove toxic substances from the body [4, 5], and infants and young children are more susceptible than adult patients to damage due to IEM, due to the difficulties in vascular access and hemodynamic instability in infants and young children, PD and CVVH have been the most commonly used forms of RRT in these patients [6–8]. IHD performed in infants and young children has been described in a number of studies, but in most of these, the case numbers were small, and the safety and efficacy data were not complete [4, 9–11]. Experiences in Asian countries are espe- cially lacking. To improve the treatment of acute metabolic decompensation in IEM, we initiated IHD for infants and young children in 2006. In the study reported here, we com- pared IHD and non-IHD RRT with regard to the safety and efficacy for these patients. Materials and methods Data from RRT performed for acute metabolic decompensation in patients with urea cycle disorders (UCDs), maple syrup urine I.<J. Tsai : W.<L. Hwu : N.<C. Lee : E.<T. Wu : Y.<H. Chien : Y.<K. Tsau Department of Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, 7 Chung-Shan South Road, Taipei 10016, Taiwan W.<L. Hwu : N.<C. Lee : Y.<H. Chien (*) Department of Medical Genetics, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan e-mail: chienyh@ntu.edu.tw S.<C. Huang Department of Surgery, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan Pediatr Nephrol (2014) 29:111–116 DOI 10.1007/s00467-013-2609-2