Impact of sodium phenylbutyrate treatment in acute management of maple syrup urine disease attacks: a single-center experience
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, vol.34, no.1, pp.121-126, 2021 (SCI-Expanded, Scopus)
- Publication Type: Article / Article
- Volume: 34 Issue: 1
- Publication Date: 2021
- Doi Number: 10.1515/jpem-2020-0356
- Journal Name: JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
- Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, BIOSIS, CAB Abstracts, EMBASE, MEDLINE
- Page Numbers: pp.121-126
- Istanbul University Affiliated: Yes
Abstract
Objectives: Accurate management of metabolic decompensation in maple syrup urine disease (MSUD) has a crucial role, as acute attacks can cause neurological sequels and can be life threatening. Here, we aimed to evaluate effect of sodium phenylbutyrate (NaPBA) in acute management of MSUD attacks.