Blood Purification

Original Paper

Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease

Demirkol D.a · Şık G.c · Topal N.d · Çıtak A.c · Zeybek Ç.e · Tüten A.c · Bilge I.b

Author affiliations

aDepartment of Pediatric Intensive Care and bDepartment of Pediatric Nephrology, Koç University School of Medicine, cDepartment of Pediatric Intensive Care, Istanbul Faculty of Medicine, dDepartment of Pediatric Nephrology, Bezmialem Vakıf University, and eDepartment of Pediatric Metabolism, Cerrahpasa Faculty of Medicine, Istanbul, Turkey

Keywords: Maple syrup urine diseaseContinuous renal replacement therapyHemodiafiltrationLeucineEncephalopathy

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Blood Purif 2016;42:27-32
https://doi.org/10.1159/000443783

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: August 04, 2015
Accepted: January 06, 2016
Published online: March 22, 2016
Issue release date: June 2016

Number of Print Pages: 6
Number of Figures: 0
Number of Tables: 3

ISSN: 0253-5068 (Print)
eISSN: 1421-9735 (Online)

For additional information: https://www.karger.com/BPU

Abstract

Background: The study aims to define the efficacy of continuous renal replacement therapy in acute metabolic decompensation treatment of maple syrup urine disease (MSUD). Methods: All the neonates, infants and children who have had life threatening conditions due to MSUD and were treated with continuous venovenous hemodiafiltration (CVVHDF) were analyzed retrospectively. Results: Fourteen patients underwent 15 sessions of CVVHDF (age range 15 days to 87 months, mean 40.8 ± 31.4 months). One patient required additional CVVHDF 1 week after cessation of CVVHDF. Twenty seven percent (n = 4) of the patients were intubated and mechanically ventilated. Twelve patients responded to treatment and dramatic neurological improvement was observed within 24 h. Two of the 14 patients required 36 h of CVVHDF for neurological improvement. The mean duration of CVVHDF was 20.2 ± 8.6 (9-36) h. The mean leucine level was 1,648 ± 623.8 (714-2,768) μmol/l before and was 256.5 ± 150.6 (117-646) μmol/l at the end of treatment. No mortality was observed. Conclusion: Continuous hemodiafiltration is an effective and safe method in correcting metabolic disturbances in MSUD.

© 2016 S. Karger AG, Basel



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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Received: August 04, 2015
Accepted: January 06, 2016
Published online: March 22, 2016
Issue release date: June 2016

Number of Print Pages: 6
Number of Figures: 0
Number of Tables: 3

ISSN: 0253-5068 (Print)
eISSN: 1421-9735 (Online)

For additional information: https://www.karger.com/BPU

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2016, Vol.42, No. 1

June 2016

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