| BH(4) therapy impacts the nutrition status and intake in children with phenylketonuria: 2-year follow-up. | |
| | |
MedLine Citation:
|
PMID: 20941642 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
|
The impact of tetrahydrobiopterin (BH(4)) treatment on phenylalanine tolerance, medical-food consumption, and nutrition status in patients with phenylketonuria (PKU) was investigated. Six children (5-12 years) with well-controlled PKU, responding to a BH(4) dose of 20 mg/kg per day, were assessed for 24 months. Mean dietary phenylalanine tolerance increased from 421 ± 128 to 1470 ± 455 mg/day. Height Z scores significantly improved from 0.25 ± 0.99 at baseline to 0.53 ± 1.16 at 24 months (p for trend < 0.001). Patients tolerated more phenylalanine and more intact protein and required less medical food (protein supplement). Improved linear growth and nutrition status was seen over the course of the 24-month follow-up. Due to the variation in phenylalanine tolerance, dietary recommendations should be tailored to the patient's individual requirements. |
| | |
Authors:
|
Rani H Singh; Meghan E Quirk; Teresa D Douglas; Mary C Brauchla |
Publication Detail:
|
Type: Journal Article Date: 2010-10-13 |
Journal Detail:
|
Title: Journal of inherited metabolic disease Volume: 33 ISSN: 1573-2665 ISO Abbreviation: J. Inherit. Metab. Dis. Publication Date: 2010 Dec |
Date Detail:
|
Created Date: 2010-11-29 Completed Date: - Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 7910918 Medline TA: J Inherit Metab Dis Country: Netherlands |
Other Details:
|
Languages: eng Pagination: 689-95 Citation Subset: IM |
Affiliation:
|
Department of Human Genetics, Emory University School of Medicine, Atlanta, GA 30030, USA. rsingh@emory.edu |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Neural markers expression in rat bone marrow mesenchymal stem cell cultures treated with neurosteroi...
Next Document: Biomarkers for mitochondrial respiratory chain disorders.