کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3315590 1211278 2006 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
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
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی بیماری‌های گوارشی
پیش نمایش صفحه اول مقاله
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
چکیده انگلیسی

Background/AimsPhosphorylase-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 1H MR spectroscopy (MRS) investigation.MethodsMRS 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.ResultsFat 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).ConclusionsLiver 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.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of Hepatology - Volume 45, Issue 6, December 2006, Pages 851–855
نویسندگان
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