کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2827874 1162469 2011 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Gaucher disease type I: Assessment of basal metabolic rate in patients from southern Brazil
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شناسی مولکولی
پیش نمایش صفحه اول مقاله
Gaucher disease type I: Assessment of basal metabolic rate in patients from southern Brazil
چکیده انگلیسی

IntroductionGaucher disease (GD) is characterized by clinical heterogeneity and is associated with metabolic abnormalities such as increased resting energy expenditure.ObjectivesTo assess the basal metabolic rate (BMR) of patients with GD type I followed at the Gaucher Disease Reference Center of Rio Grande do Sul, Brazil.Patients and MethodsFourteen patients (male = 6) and 14 healthy controls matched by gender, age and body mass index (BMI) were included in the study. The nutritional status of patients was assessed by BMI. The BMR was measured by indirect calorimetry. In two patients, it was possible to perform BMR in the pre- and the post-treatment periods.ResultsMean age and BMI of patients and controls were, respectively, 32.8 ± 17.6 and 32.1 ± 16.6 years and 23.3 ± 3.1 and 22.4 ± 3.1 kg/m2. Twelve patients were receiving enzyme replacement therapy (ERT) with imiglucerase (mean duration of treatment = 5.2 ± 4.3 years; mean dosage of imiglucerase = 24.2 ± 7.3 UI/kg/inf). Five patients (36%) were overweight, and nine (64%) were normal weight. Mean BMR of patients on ERT was 27.1% higher than that of controls (p = 0.007). There was no difference between the BMR of patients on ERT and not on ERT (n = 4) (p = 0.92). Comparing the BMR of patients on ERT and their controls with the BMR estimated by the Harris-Benedict equation, the BMR of patients was 6.3% higher than the estimated (p = 0.1), while the BMR of their controls was 17.0% lower than the estimated (p = 0.001).ConclusionMost treated GD type I patients were normal weight. The patients including those on ERT showed higher BMR when compared to controls. Imiglucerase is probably unable to normalize the hypermetabolism presented by GD type I patients. Additional studies should be performed to confirm our findings.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Blood Cells, Molecules, and Diseases - Volume 46, Issue 1, 15 January 2011, Pages 42–46
نویسندگان
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