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Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease
- Source :
- Blood purification. 42(1)
- Publication Year :
- 2015
-
Abstract
- PubMed ID: 26998605<br />No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.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 © 2016 S. Karger AG, Basel. Copyright: All rights reserved.
- Subjects :
- Continuous renal replacement therapy
medicine.medical_specialty
medicine.medical_treatment
Encephalopathy
Maple syrup urine disease
030232 urology & nephrology
Hemodiafiltration
Leucine level
03 medical and health sciences
0302 clinical medicine
Maple Syrup Urine Disease
Metabolic Diseases
Leucine
Continuous hemodiafiltration
030225 pediatrics
medicine
Humans
Renal replacement therapy
Child
Retrospective Studies
business.industry
ComputerSystemsOrganization_COMPUTER-COMMUNICATIONNETWORKS
Infant, Newborn
Infant
Retrospective cohort study
Hematology
General Medicine
Continuous venovenous hemodiafiltration
medicine.disease
Acute metabolic decompensation
Surgery
Renal Replacement Therapy
ComputingMilieux_MANAGEMENTOFCOMPUTINGANDINFORMATIONSYSTEMS
ComputingMethodologies_PATTERNRECOGNITION
Treatment Outcome
Nephrology
Child, Preschool
InformationSystems_MISCELLANEOUS
business
Subjects
Details
- ISSN :
- 14219735
- Volume :
- 42
- Issue :
- 1
- Database :
- OpenAIRE
- Journal :
- Blood purification
- Accession number :
- edsair.doi.dedup.....42935ada386c7e9237e69f168be91c96