Pompe disease results from acid α-glucosidase (GAA) deficiency, and enzyme replacement therapy (ERT) with recombinant human (rh) GAA has clinical benefits, although its limitations include the short half-life of GAA and the formation of antibody responses. The present study compared the efficacy of ERT against gene transfer with an adeno-associated viral (AAV) vector containing a liver-specific...

We aimed to establish the profile of Irish patients with Hunter Syndrome (Mucopolysaccharidosis type II, MPS II) receiving weekly intravenous Enzyme Replacement Therapy (ERT) with recombinant iduronate-2-sulfatase and to assess the social impact and parental opinion of ERT through the use of a parental questionnaire. Nine patients aged 3.5- 14 years have received a mean of 2 (range 0.5-3.5) yea...

Two important mechanisms affect our ability to estimate solute concentrations quantitatively from the inversion of field-scale electrical resistivity tomography ERT data: 1 the spatially variable physical processes that govern the flow of current as well as the variation of physical properties in space and 2 the overparameterization of inverse models, which requires the imposition of a smoothin...

BACKGROUND Fabry disease is an X linked lysosomal storage disease caused by deficiency of the lysosomal enzyme alpha-galactosidase A. This leads to accumulation of globotriaosylceramide in nearly all tissues, including the blood vessels, kidney, myocardium, and nervous system. Symptoms often begin in childhood and include acroparaesthesia, with burning or tingling pain that spreads from the ext...

سابقه و هدف: آب هوای یک منطقه در دوره بلندمدت را اقلیم گویند. شناسایی طبقه‌بندی اقلیمی از دیرباز مورد توجه متخصصان هواشناسی بوده است. محققان با به کارگیری روش ­های مختلف استفاده متغیرهای همچون بارش دما، زمین مناطق همگن طبقه ­بندی کرده‌اند نتایج پهنه‌بندی ارزیابی کمبود منابع مقیاس­ های کوچک بزرگ منظور پیش‌بینی اقدامات عملی برای کنترل خشکی آسیب‌پذیر به‌کار برده‌اند. هدف این تحقیق به‌‌روزرسانی تحل...

Regardless of age, race, and ethnicity, cardiovascular disease is the number 1 cause of death for women, and its prevalence rises rapidly after menopause.1–4 This postmenopausal increase is believed to result from the loss of endogenous estrogen. Although estrogen replacement therapy (ERT) has long been thought to protect against menopauseassociated cardiovascular anomalies, osteoporosis, hot f...

We propose in this work a fast numerical algorithm for solving the equation of radiative transfer (ERT) in isotropic media. The algorithm has two steps. In the first step, we derive an integral equation for the angularly averaged ERT solution by taking advantage of the isotropy of the scattering kernel, and solve the integral equation with a fast multipole method (FMM). In the second step, we s...