کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8723007 | 1589613 | 2018 | 7 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Bone Density in Patients With Berardinelli-Seip Congenital Lipodystrophy Is Higher in Trabecular Sites and in Type 2 Patients
ترجمه فارسی عنوان
تراکم استخوان در بیماران با لیپیودیستروفی مادرزادی برادینلی-سیف در محل های تراککولار و در بیماران نوع 2 بیشتر است
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کلمات کلیدی
موضوعات مرتبط
علوم پزشکی و سلامت
پزشکی و دندانپزشکی
غدد درون ریز، دیابت و متابولیسم
چکیده انگلیسی
Berardinelli-Seip congenital lipodystrophy (BSCL) is a rare autosomal recessive syndrome characterized by a difficulty storing lipid in adipocytes, low body fat, hypoleptinemia, and hyperinsulinemia. We report here laboratory, bone mineral density (BMD), and bone mineral content findings of 21 patients (24.1â±â8.4âyr old, 14 females, 18 diabetics, 5.3% total body fat) with BSCL. The mean leptin was very low (0.91â±â0.42 ng/mL), and the mean values of the Z-scores for all studied sites were positive, except for the 33% radius (Z-score â0.5âstandard deviation [SD]). Twelve patients (57.1%) had a BMD Z-score higher than +2.5âSD in at least 1 site. There was no significant difference in the Z-scores between males and females. None of type 1 (AGPAT2) patients had Z-scores higher than +2.5âSD, and these patients had a smaller Z-score of BMD total body (0.26âSD vs 1.90âSD, pâ=â0.022) and of bone mineral content (1.59âSD vs 3.3âSD, pâ=â0.032) than type 2 (seipin) patients. Insulin, as well as HOMAIR (homeostasis model assessment), correlated positively with the BMD of all sites, except for the 33% radius. Z-Scores on this site (33% radius) were the smallest of all. More than half of our patients with BSCL have BMD Z-scores higher than +2.5âSD on at least 1 site, and this increase is more pronounced in the trabecular sites and in type 2 patients.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of Clinical Densitometry - Volume 21, Issue 1, JanuaryâMarch 2018, Pages 61-67
Journal: Journal of Clinical Densitometry - Volume 21, Issue 1, JanuaryâMarch 2018, Pages 61-67
نویسندگان
Josivan G. Lima, Lucia Helena C. Nobrega, Natalia N. Lima, Marcel C.F. dos Santos, Maria de Fatima P. Baracho, Francisco Bandeira, Leonardo Capistrano, Francisco Paulo Freire Neto, Selma Maria B. Jeronimo,