1H MR chemical shift imaging detection of phenylalanine in patients suffering from phenylketonuria (PKU)

European Radiology
P E SijensFrancjan J van Spronsen

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, relati...Continue Reading

Citations

Jan 20, 2007·European Radiology·Alfonso Di CostanzoTommaso Scarabino
Oct 30, 2014·Journal of Cerebral Blood Flow and Metabolism : Official Journal of the International Society of Cerebral Blood Flow and Metabolism·Martijn J de GrootFrancjan J van Spronsen
Oct 21, 2015·Journal of Computer Assisted Tomography·Stephan UlmerFrank J Ahlhelm
Jun 27, 2018·Orphanet Journal of Rare Diseases·Gina A Montoya ParraAnita MacDonald

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