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Titolo:
IN-VIVO NMR-SPECTROSCOPY IN PATIENTS WITH PHENYLKETONURIA - CHANGES OF CEREBRAL PHENYLALANINE LEVELS UNDER DIETARY-TREATMENT
Autore:
MOLLER HE; VERMATHEN P; ULLRICH K; WEGLAGE J; KOCH HG; PETERS PE;
Indirizzi:
UNIV MUNSTER,INST KLIN RADIOL,ALBERT SCHWEITZER STR 33 D-48129 MUNSTER GERMANY UNIV MUNSTER,INST PHYS CHEM D-48129 MUNSTER GERMANY UNIV MUNSTER,KINDERHEILKUNDE KLIN & POLIKLIN D-48129 MUNSTER GERMANY
Titolo Testata:
Neuropediatrics
fascicolo: 4, volume: 26, anno: 1995,
pagine: 199 - 202
SICI:
0174-304X(1995)26:4<199:INIPWP>2.0.ZU;2-4
Fonte:
ISI
Lingua:
ENG
Soggetto:
BLOOD-BRAIN-BARRIER; METABOLITES; TRANSPORT; INVIVO; WATER;
Keywords:
BLOOD-BRAIN BARRIER TRANSPORT; CEREBRAL PHENYLALANINE; PROTON MAGNETIC RESONANCE SPECTROSCOPY; PHENYLKETONURIA; TRANSPORT KINETICS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
17
Recensione:
Indirizzi per estratti:
Citazione:
H.E. Moller et al., "IN-VIVO NMR-SPECTROSCOPY IN PATIENTS WITH PHENYLKETONURIA - CHANGES OF CEREBRAL PHENYLALANINE LEVELS UNDER DIETARY-TREATMENT", Neuropediatrics, 26(4), 1995, pp. 199-202

Abstract

Localized proton magnetic resonance spectroscopy at short echo times was used to measure phenylalanine (Phe) in parieto-occipital periventricular brain. Six treated adult patients with phenylketonuria were investigated repeatedly following reinstitution of a Phe-restricted diet. Difference spectroscopy clearly enabled the identification of elevated cerebral Phe levels by subtracting spectra obtained from healthy volunteers. Estimates of absolute brain concentrations always yielded values well below the serum levels with ratios [Phe](brain)/[Phe](serum) ranging from 0.27 to 0.63. A plot of [Phe](brain) versus [Phe](serum) could be fitted to a straight line (R = 0.90) if [Phe](serum) was below 1.3 mM. Measurements at higher serum levels could only be performed in one patient and yielded brain Phe concentrations of 0.63 +/- 0.10 mM suggesting a saturation of the carrier systems. The feasibility to quantify Phe transport across the blood-brain barrier in humans non-invasively employing in-vivo proton spectroscopy can effectively improve the prognostic significance of serum data.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 20/01/21 alle ore 15:33:29