当前位置： SCI文献检索 > JOURNAL OF HEPATOLOGY期刊下所有文献 > Multiple voxel 1H MR spectroscopy of phosphorylase-b kinase deficient patients (GSD IXa) showing an accumulation of fat in the liver that resolves with aging.

Multiple voxel 1H MR spectroscopy of phosphorylase-b kinase deficient patients (GSD IXa) showing an accumulation of fat in the liver that resolves with aging.

Abstract：

BACKGROUND/AIMS:Phosphorylase-b deficient patients suffer from glycogen storage disease (GSD IXa) leading to liver enlargement which usually resolves during puberty and adolescence. This pathology has not yet been documented by (1)H MR spectroscopy (MRS) investigation. METHODS:MRS of eight GSD IXa patients was performed in this study to assess whether or not liver fat content is elevated in GSD IXa and decreases with aging. An improvement in our MRS method compared with previous liver fat MRS studies is that we measured a plane of liver voxels at once rather than a single MRS voxel, yielding a reliable determination of liver fat content. RESULTS:Fat contents of 3.4-10% were observed in young GSD IXa patients, as compared with 0.5-0.9% in controls, these dropped to control levels in patients past age 40 (r = -0.82; P < 0.01). CONCLUSIONS:Liver fat content is increased in glycogen storage disease (GSD IXa) and normalizes with ageing. Assessing liver fat levels in this population is a novel and interesting concept. This could potentially enhance the understanding of liver function in that 20% of the population who has increased liver fat.

journal_name

J Hepatol

journal_title

Journal of hepatology

authors

Sijens PE,Smit GP,Borgdorff MA,Kappert P,Oudkerk M

doi

10.1016/j.jhep.2006.06.021

subject

Has Abstract

pub_date

2006-12-01 00:00:00

pages

851-5

issue

6

eissn

0168-8278

issn

1600-0641

pii

S0168-8278(06)00406-5

journal_volume

45

pub_type

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