Exocrine pancreatic insuffiency is loss of ability to properly digest food due to inadequate pancreatic enzyme activity. The etiology of exocrine pancreatic insuffiency, in humans, is mostly chronic pancreatitis or diseases affecting pancreas such as cytsic fibrosis, Shwachman-Diamond syndrome. It may also occur as a complication of gastric or pancreatic surgical procedures due to decrease of e...

The paper by De Fost et al. in the current issue of Blood contains important data with regard to dose-sensitivity in the treatment of Gaucher disease, but the title is misleading. It implies that high-dose enzyme replacement therapy may be superior to low-dose enzyme replacement therapy. However, it is apparent that “superiority” in response is limited to two surrogate markers, serum chitotrios...

We aimed to evaluate the outcome of enzyme replacement therapy (ERT) in Turkish Gaucher patients since it first became available in our country. Eleven patients with type I and one patient with type III Gaucher disease (GD) received therapy as 30-60 U/kg and 120 U/kg every two weeks, respectively, for at least six months, starting a mean period of 4.2 years after the diagnosis. Assessment of re...

Mucopolysaccharidoses (MPS) are severe inherited metabolic disorders caused by storage of glycosaminoglycans (GAGs). The level of accumulated GAGs is an important parameter in assessment of the severity of the disease and the efficacy of treatment; unfortunately, biochemical methods are often unreliable and only semi-quantitative. Therefore, finding other methods for estimation of GAG levels an...

This is a retrospective analysis of Fabry Outcome Survey data from children/adults (n = 677) receiving agalsidase alfa enzyme replacement therapy for a median of 3 years, examining cerebrovascular, cardiac, and renal morbidity endpoints separately. Cardiac events occurred at younger ages than cerebrovascular or renal events, cerebrovascular events were more frequent in females than males, and m...

Equilibrium binding studies demonstrate that purified Escherichia coli isocitrate dehydrogenase binds isocitrate, alpha-ketoglutarate, NADP, and NADPH at 1:1 ratios of substrate to enzyme monomer. The phosphorylated enzyme, which is completely inactive, is unable to bind isocitrate but retains the ability to bind NADP and NADPH. Replacement of serine 113, which is the site of phosphorylation, b...

today power consumption is considered to be one of the important issues. therefore, its reduction plays a considerable role in developing systems. previous studies have shown that approximately 50% of total power consumption is used in cache memories. there is a direct relationship between power consumption and replacement quantity made in cache. the less the number of replacements is, the less...