Document Detail


White matter integrity and executive abilities following treatment with tetrahydrobiopterin (BH4) in individuals with phenylketonuria.
MedLine Citation:
PMID:  23928118     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Tetrahydrobiopterin (BH(4)) lowers blood phenylalanine (Phe) in individuals with PKU who are responders, but its effects on the brain and cognition have not been explored thoroughly. We examined blood Phe, microstructural white matter integrity, and executive abilities in 12 BH(4) responders before (i.e., baseline) and after (i.e., follow-up) six months of treatment with BH(4). Compared with baseline, Phe in these responders decreased by 51% during a 4 week screening period after initiation of treatment and remained lowered by 37% over the 6 month follow-up period. Significant improvements in white matter integrity, evaluated by mean diffusivity from diffusion tensor imaging, were also found following six months of treatment. Improvements in executive abilities were not identified, although six months may have been a period too brief for changes in cognition to follow changes in the brain. To our knowledge, our study is the first to explore relationships among Phe, white matter integrity, executive abilities, and BH(4) treatment within a single study.
Authors:
Desirée A White; Jo Ann V Antenor-Dorsey; Dorothy K Grange; Tamara Hershey; Jerrel Rutlin; Joshua S Shimony; Robert C McKinstry; Shawn E Christ
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2013-07-19
Journal Detail:
Title:  Molecular genetics and metabolism     Volume:  110     ISSN:  1096-7206     ISO Abbreviation:  Mol. Genet. Metab.     Publication Date:  2013 Nov 
Date Detail:
Created Date:  2013-10-16     Completed Date:  2014-05-08     Revised Date:  2014-11-04    
Medline Journal Info:
Nlm Unique ID:  9805456     Medline TA:  Mol Genet Metab     Country:  United States    
Other Details:
Languages:  eng     Pagination:  213-7     Citation Subset:  IM    
Copyright Information:
© 2013.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Biopterin / analogs & derivatives,  therapeutic use
Brain / pathology*,  physiopathology*
Case-Control Studies
Child
Diffusion Tensor Imaging
Female
Follow-Up Studies
Humans
Male
Phenylalanine / blood
Phenylketonurias / blood,  drug therapy,  pathology*,  physiopathology*
Treatment Outcome
Young Adult
Grant Support
ID/Acronym/Agency:
P30 HD062171/HD/NICHD NIH HHS; P30HD062171/HD/NICHD NIH HHS; T32 DA007261/DA/NIDA NIH HHS; T32DA007261/DA/NIDA NIH HHS
Chemical
Reg. No./Substance:
17528-72-2/5,6,7,8-tetrahydrobiopterin; 22150-76-1/Biopterin; 47E5O17Y3R/Phenylalanine
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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