Pompe’s Disease and the Effects of Alpha-Glucosidase Deficiency

The following is an excerpt from the introduction of this article: The energy that the body needs in order to function is obtained from carbohydrates that we get through our diet. These carbohydrates are monosacharides, disaccharides and polysacharides. The polysaccharides and disaccharides are hydrolyzed to monosaccharide’s such glucose (which comprises roughly 80%) fructose and galactose. Most cells convert the fructose and galactose to glucose. The body can use the glucose or store it. If energy is needed, glucose can be oxidized through the many reactions of glycolysis which gives a net production of 2 ATP and 2 NADH from one molecule of glucose. In the presence of oxygen the product of glycolysis pyruvic acid will be decarboxilated to Acetyl Coenzyme A (Acetyl COA) in the mitochondrial matrix forming carbon dioxide and 2 NADH in the process. The acetyl COA subsequently enters the citric acid cycle (the Krebs cycle) where after a series of reactions, 2 molecules of ATP, 4 molecules of carbon dioxide, 6 NADH and 2 FADH2 are produced. The NADH’s enter the electron transport chain and over a series of reactions with the proteins in the mitochondrial matrix form 3 ATP per molecule of NADH and 2 ATP per molecule of FADH2. (Murray) In accordance with homeostasis, the presence of high energy will cause the body to balance energy levels by forming storage molecules of energy for use in times of need. This storage form of glucose is called glycogen; the synthesis is called glycogenesis. This glycogen can later be degraded into is monomer units for the use in oxidative phosphorylation. (Champe)

Median Nerve and Ulnar Nerve Entrapment with Cubital Tunnel Syndrome in a Hemodialysis Patient Following Creation of an Arteriovenous Fistula.

Neurological and vascular complications associated with creation of arteriovenous access need to be recognized promptly to deliver appropriate interventions for relief of symptoms and avoid loss of function of the involved extremity. We present here a 55-year-old female with end-stage renal disease on hemodialysis secondary to diabetic nephropathy who had a surgical creation of first stage of the brachial artery-basilic vein fistula in the left arm. She subsequently developed pain and weakness of the left arm which was diagnosed as median and ulnar nerve entrapment. She was treated with surgical nerve release and neurolysis and her symptoms improved