Continuous Venovenous Hemodiafiltration in the Treatment of Maple Syrup Urine Disease.
Blood Purif
; 42(1): 27-32, 2016.
Article
en En
| MEDLINE
| ID: mdl-26998605
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.
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Enfermedad de la Orina de Jarabe de Arce
Tipo de estudio:
Etiology_studies
/
Observational_studies
/
Risk_factors_studies
Límite:
Child
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Child, preschool
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Humans
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Infant
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Newborn
Idioma:
En
Revista:
Blood Purif
Año:
2016
Tipo del documento:
Article
País de afiliación:
Turquía