Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease
BLOOD PURIFICATION, cilt.42, sa.1, ss.27-32, 2016 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 42 Sayı: 1
- Basım Tarihi: 2016
- Doi Numarası: 10.1159/000443783
- Dergi Adı: BLOOD PURIFICATION
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Sayfa Sayıları: ss.27-32
- Anahtar Kelimeler: Maple syrup urine disease, Continuous renal replacement therapy, Hemodiafiltration, Leucine, Encephalopathy, BRANCHED-CHAIN AMINO, EXTRACORPOREAL REMOVAL THERAPY, RENAL REPLACEMENT THERAPY, INBORN-ERRORS, PERITONEAL-DIALYSIS, ACUTE-PHASE, HEMODIALYSIS, ACIDS, HEMOFILTRATION, CHILDREN
- Acıbadem Mehmet Ali Aydınlar Üniversitesi Adresli: Evet
Özet
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) mu mol/l before and was 256.5 +/- 150.6 (117-646) mu mol/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. (C) 2016 S. Karger AG, Basel