title: High-volume continuous venovenous hemofiltration as an effective therapy for acute management of inborn errors of metabolism in young children.
abstract: OBJECTIVE
  Renal replacement therapies (RRTs) have been used for the acute management of inborn errors of metabolism. Hemodialysis is the most effective modality. The aim of this article is to demonstrate that high-volume hemofiltration can offer an alternative way to effectively remove small molecules.
  
  
  METHODS
  Eight patients presented with acute neurological deterioration due to ammonia or organic acid accumulation. Different RRTs were applied, including continuous venovenous hemofiltration (CVVH, n = 7), continuous arteriovenous hemofiltration (CAVH, n = 2), continuous venovenous hemodialysis (CVVHD, n = 1), intermittent hemodialysis (HD, n = 1), and peritoneal dialysis (PD, n = 2).
  
  
  RESULTS
  Ammonia 50% reduction time in HD was 1.7 h while in CVVH it was 2-14.5 h. The greater the ultrafiltration flow was, the sooner patients regained consciousness. CAVH, CVVHD or PD was not sufficient enough.
  
  
  CONCLUSIONS
  CVVH also has a good clearance for organic acid and ammonia if applying high-volume hemofiltration (>35 ml/kg/h). It can be therefore be considered as an alternative therapy if infant HD is not available.
authors.affiliation: Division of Pediatric Nephrology, Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Yi-Chun
authors.initials: YC
authors.lastname: Lai
authors.forename: Hsin-Ping
authors.initials: HP
authors.lastname: Huang
authors.forename: I-Jung
authors.initials: IJ
authors.lastname: Tsai
authors.forename: Yong-Kwei
authors.initials: YK
authors.lastname: Tsau
mesh_terms: D020165:Carbamoyl-Phosphate Synthase I Deficiency Disease / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006440:Hemofiltration / Q000379:methods*; D006801:Humans; D022124:Hyperammonemia / Q000209:etiology / Q000628:therapy*; D007223:Infant; D007231:Infant, Newborn; D007239:Infections / Q000097:blood / Q000150:complications; D008297:Male; D008375:Maple Syrup Urine Disease / Q000628:therapy; D008661:Metabolism, Inborn Errors / Q000097:blood / Q000150:complications / Q000628:therapy*; D008764:Methylmalonic Acid / Q000097:blood; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000628:therapy; D017582:Renal Replacement Therapy / Q000379:methods; D016896:Treatment Outcome
chemical_list: D008764:Methylmalonic Acid