title: 1H MR chemical shift imaging detection of phenylalanine in patients suffering from phenylketonuria (PKU).
abstract: Short echo time single voxel methods were used in previous MR spectroscopy studies of phenylalanine (Phe) levels in phenylketonuria (PKU) patients. In this study, apparent T2 relaxation time of the 7.3-ppm Phe multiplet signal in the brain of PKU patients was assessed in order to establish which echo time would be optimal. 1H chemical shift imaging (CSI) examinations of a transverse plain above the ventricles of the brain were performed in 10 PKU patients and 11 persons not suffering from PKU at 1.5 T, using four echo times (TE 20, 40, 135 and 270 ms). Phe was detectable only when the signals from all CSI voxels were summarized. In patients suffering from PKU the T2 relaxation times of choline, creatine and N-acetyl aspartate (NAA) were similar to those previously reported for healthy volunteers (between 200 and 325 ms). The T2 of Phe in brain tissue was 215 +/- 120 ms (standard deviation). In the PKU patients the brain tissue Phe concentrations were 141 +/- 69 microM as opposed to 58 +/- 23 microM in the persons not suffering from PKU. In the detection of Phe, MR spectroscopy performed at TE 135 or 270 ms is not inferior to that performed at TE 20 or 40 ms (all previous studies). Best results were obtained at TE=135 ms, relating to the fact that at that particular TE, the visibility of a compound with a T2 of 215 ms still is good, while interfering signals from short-TE compounds are negligible.
authors.affiliation: Department of Radiology, University Hospital Groningen, Hanzeplein 1, P.O. Box 30001, Groningen, The Netherlands. p.e.sijens@rad.azg.nl
authors.forename: Paul E
authors.initials: PE
authors.lastname: Sijens
authors.forename: Matthijs
authors.initials: M
authors.lastname: Oudkerk
authors.forename: Dirk-Jan
authors.initials: DJ
authors.lastname: Reijngoud
authors.forename: Klaas L
authors.initials: KL
authors.lastname: Leenders
authors.forename: Harold W
authors.initials: HW
authors.lastname: de Valk
authors.forename: Francjan J
authors.initials: FJ
authors.lastname: van Spronsen
mesh_terms: D000293:Adolescent; D000328:Adult; D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000032:analysis; D001923:Brain Chemistry; D002552:Cerebral Ventricles / Q000737:chemistry; D002794:Choline / Q000032:analysis; D003401:Creatine / Q000032:analysis; D005260:Female; D006801:Humans; D006859:Hydrogen; D007091:Image Processing, Computer-Assisted / Q000379:methods; D009682:Magnetic Resonance Spectroscopy / Q000379:methods; D008297:Male; D010649:Phenylalanine / Q000032:analysis* / Q000097:blood; D010661:Phenylketonurias / Q000378:metabolism*
chemical_list: D001224:Aspartic Acid; D010649:Phenylalanine; D006859:Hydrogen; C000179:N-acetylaspartate; D003401:Creatine; D002794:Choline