2012
DOI: 10.1007/s10545-012-9570-2
|View full text |Cite
|
Sign up to set email alerts
|

Treatment of acute decompensation of maple syrup urine disease in adult patients with a new parenteral amino‐acid mixture

Abstract: This new parenteral amino-acid mixture is safe and allows efficient Leu concentration decrease during acute MSUD decompensation episodes in adults. Its use avoids the need for nasogastric tube insertion.

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
3
1
1

Citation Types

3
25
0

Year Published

2014
2014
2022
2022

Publication Types

Select...
4
3

Relationship

0
7

Authors

Journals

citations
Cited by 17 publications
(28 citation statements)
references
References 9 publications
3
25
0
Order By: Relevance
“…“Oral/enteral BCAA‐free formulas” were commercially available products and the names were not recorded, thus composition may have varied. “IV BCAA‐free solution” was supplied as a hospital preparation by the French General Agency of Equipment and Health Products ( AGEPS ) and administered at a standard dose of 1‐2 g/kg of body weight per day 16 . Its composition is reported in Table S1.…”
Section: Methodsmentioning
confidence: 99%
See 2 more Smart Citations
“…“Oral/enteral BCAA‐free formulas” were commercially available products and the names were not recorded, thus composition may have varied. “IV BCAA‐free solution” was supplied as a hospital preparation by the French General Agency of Equipment and Health Products ( AGEPS ) and administered at a standard dose of 1‐2 g/kg of body weight per day 16 . Its composition is reported in Table S1.…”
Section: Methodsmentioning
confidence: 99%
“…Metabolic decompensations require urgent therapy to normalize leucine levels to avoid permanent neurological damage or death 11,12 . Metabolic decompensations in MSUD are treated by providing a BCAA‐free formula to reduce plasma BCAA levels within reference ranges and to ensure that the leucine‐isoleucine‐valine balance is maintained 15‐17 . BCAA‐free formulas given during metabolic decompensations are provided at higher concentration than standard BCAA‐free formulas due to a further reduction of intact protein intake during metabolic decompensations.…”
Section: Introductionmentioning
confidence: 99%
See 1 more Smart Citation
“… 40 Long-term treatment requires careful manipulation of calories, restriction of dietary BCAAs, and supplementation using a BCAA-free amino acid mixture ( Table 3 shows a list of available products) to provide non-BCAAs and other nutrients for protein synthesis. 41 …”
Section: Medical Nutrition Managementmentioning
confidence: 99%
“…4 MSUD is diagnosed by the presence of clinical features, elevated BCAA in plasma, and branched-chain hydroxyacids and ketoacids (BCKAs) in urine. 8,9 The world-wide incidence of MSUD is estimated to be 1 in 185,000. 10,11 To date, over 200 mutations have been detected among BCKDHA, BCKDHB and DBT (Human Gene Database, http://www.hgmd.cf.ac.uk).…”
Section: Introductionmentioning
confidence: 99%