Archives of Disease in Childhood - Fetal and Neonatal Edition 2009;94:F216-F217
ORIGINAL ARTICLES
Prenatal benzoate treatment in urea cycle defects
1 Department of Paediatrics II, Hannover Medical School, Hannover, Germany
2 Department of Gynaecology and Obstetrics, Hannover Medical School, Hannover, Germany
3 Department of Paediatrics I, Hannover Medical School, Hannover, Germany
4 Medical Laboratory Bremen, Bremen, Germany
Professor Anibh M Das, Department of Paediatrics II, Hannover Medical School, Carl-Neuberg Str. 1, D-30625 Hannover, Germany; das.anibh{at}mh-hannover.de
Objective: In patients with severe urea cycle defects (UCD) metabolic decompensation with hyperammonaemia typically occurs during the first days of life resulting in severe neurological damage or death. Benzoate can eliminate nitrogen independent of the urea cycle. Usually, benzoate is started soon after birth, but prenatal administration might improve metabolic stability.
Design: Two fetuses with a prenatal diagnosis of UCD (female: citrullinaemia; male: ornithine transcarbamylase deficiency) were loaded with benzoate prenatally via the placenta by infusing their mothers with benzoate. Benzoate concentrations were measured in umbilical cord blood and the blood of the mothers and their newborns.
Results: Therapeutic concentrations of benzoate were found in umbilical cord blood and in the children’s blood. Thus, benzoate transfer across the placenta was demonstrated. Plasma ammonia and glutamine levels in the postnatal period were within the normal range.
Conclusions: Benzoate infusion of the mother shortly before birth is safe and results in therapeutic levels of benzoate in umbilical cord blood.
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
- Full Text
- Full Text (PDF)
-
All Versions of this Article:
adc.2008.144824v1
adc.2008.144824v2
94/3/F216 most recent - Submit a response
- Alert me when this article is cited
- Alert me when eLetters are posted
- Alert me if a correction is posted
Services
- Email this link to a friend
- Similar articles in ADC Online
- Similar articles in PubMed
- Add article to my folders
- Download to citation manager
- Request Permissions
Citing Articles
Google Scholar
PubMed
Topic Collections
Bookmark with
Register for free content
The full back archive is now available for all BMJ Journals. Institutional subscribers may access the entire archive as part of their subscription. Personal subscribers will also have access to all content when logged in. Non-subscribers who register have free access to all articles published before 2006 right back to volume 1 issue 1. Register here to access the free archive of all BMJ Journals.
Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.
