کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2936065 1576369 2007 4 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Increased aortic stiffness in glycogenosis type 2 (Pompe's disease)
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی کاردیولوژی و پزشکی قلب و عروق
پیش نمایش صفحه اول مقاله
Increased aortic stiffness in glycogenosis type 2 (Pompe's disease)
چکیده انگلیسی

BackgroundPompe's disease, also known as acid maltase deficiency or glycogen storage disease type II, is an autosomal recessive disorder in which deficient activity of the enzyme acid α-glucosidase causes intra-lysosomal accumulation of glycogen in muscle and other tissues. The current study was designed to assess aortic stiffness index (β), as a characteristic of aortic elasticity during transthoracic echocardiography in patients with Pompe's disease.MethodsA total of 17 patients (age 44 ± 8 years, 5 males) with Pompe's disease were studied. Their results were compared to 17 age- and gender-matched controls. In all patients, the ascending aorta was recorded with M-mode echocardiography. β was calculated as ln(SBP/DBP)/[(SD−DD)/DD], where SBP and DBP are the systolic and diastolic blood pressures, SD and DD are the systolic and diastolic aortic diameters, and ‘ln’ is the natural logarithm.ResultsDiastolic aortic diameter was 27.4 ± 2.4 mm in Pompe patients and 25.6 ± 2.7 mm in controls (P < 0.05). Systolic aortic diameters did not differ between the groups (29.4 ± 2.5 mm vs 28.3 ± 2.4 mm, P = ns). Aortic stiffness index (β) was increased in Pompe patients compared to controls (14.6 ± 10.1 vs 5.1 ± 2.6, P < 0.001).ConclusionsThe results of this study indicate that aortic stiffness is increased in patients with Pompe's disease. This may be due to glycogen storage in the vessel wall causing reduced vascular elasticity.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: International Journal of Cardiology - Volume 120, Issue 1, 9 August 2007, Pages 138–141
نویسندگان
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