کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8648102 | 1570392 | 2018 | 17 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Skeletal involvement in type 1 Gaucher disease: Not just bone mineral density
ترجمه فارسی عنوان
گرفتگی اسکلتی در بیماری نوع 1 گوچه: نه تنها تراکم معدنی استخوان
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کلمات کلیدی
بیماری گوچه، کیفیت استخوانی، نمره تراککال استخوان، عوارض اسکلتی،
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شناسی مولکولی
چکیده انگلیسی
Gaucher disease is characterized by multi-organ infiltration of phospholipid-laden macrophages. Bone involvement is characterized by typical deformities, osteopenia/osteoporosis, pathological fractures, and bone marrow infiltration (avascular osteonecrosis, infarction). Estimation of skeletal disease includes bone quality that contributes substantially to bone strength. We studied 23 type 1 Gaucher patients (median age 22 years, range 3-73) on Enzyme Replacement Therapy from 2 months to 26 years (median 7 years); 4 patients had pathological fractures, 10 bone infarctions, 6 avascular osteonecrosis. We noninvasively assessed bone quality by trabecular microarchitecture and macroscopic geometry, using two innovative dual-energy X-ray absorptiometry tools: Trabecular Bone Score (TBS) and Hip Structural Analysis (HSA). Bone quality parameters distinguished the patients with skeletal complications. TBS was significantly lower in patients with avascular osteonecrosis (p = 0.049) and pathological fractures (p = 0.024), while it could not identify those with bone infarctions. Among HSA parameters, the Cross Sectional Area of the intertrochanteric region and the Buckling Ratio of the narrow neck allowed the distinction of patients with avascular osteonecrosis. BMD was low in 11 patients (50%); neither BMD nor HSA were associated with pathological fractures. The combined evaluation of bone quality and bone quantity is useful to identify GD patients with more severe skeletal involvement.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Blood Cells, Molecules, and Diseases - Volume 68, February 2018, Pages 148-152
Journal: Blood Cells, Molecules, and Diseases - Volume 68, February 2018, Pages 148-152
نویسندگان
M. Baldini, G. Casirati, F.M. Ulivieri, E. Cassinerio, K. Khouri Chalouhi, E. Poggiali, L. Borin, V. Burghignoli, B.M. Cesana, M.D. Cappellini,