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Enlist glycogen storage disease
Enlist glycogen storage disease
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1. Would you expect glycogen to accumulate in the muscle of this patient? Why or why not? (5 points)
Yes, I would expect glycogen to accumulate in the muscle of this patient. The patient has type V glycogen storage disease which means he/she does not have muscle glycogen phosphorylase. Phosphorylase is an enzyme involved in the catalase breakdown of glycogen to glucose for use in muscle. This process is called glycogenolysis which is the breakdown of glycogen into individual glucose units in the form of glucose 1-phosphate. With-out that process glycogen will build up in the muscle because it is not getting broken down to glucose 1-phosphate that can be used for energy.
2. Would you expect lactate to accumulate in the muscle or blood of this patient during exercise? Why or why not? (5 points)
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Lactate is made from the anaerobic metabolism of glucose which travels to the liver and is converted back to glucose via gluconeogenesis; that cycle is the Cori cycle. Since the patient cannot break down glycogen to glucose, the Cori cycle could not be completed because there will not be any glucose 6-phosphate made into lactate by a pyruvate.
3. What fuels would you expect this patient to use during exercise? (10 points)
Since the patient cannot use glycose as a form of energy for the muscle I would expect this patient to use fuels such as fatty acids and proteins.
1. In normal individuals, the capacity of the liver to phosphorylate fructose (fructokinase activity) greatly exceeds the liver's capacity to split fructose 1-phosphate (aldolase B activity). Why is a deficiency of fructokinase a less serious genetic defect than a deficiency of fructose 1-phosphate aldolase? Consider what happens to fructose in each case and what effect this has on hepatic metabolism. (20
We actually saw this change occur by watching the RER values increase. At the 2:30 mark Kerbi was using 100% fats as her fuel source with an RER of .75. One minute later, at an RER of .80 she was using 50% fat and 50% carbohydrates. In between minutes 6:30 with and RER of .99 and less than the 7:00 mark with an RER of 1.04 she was using primarily 100% carbohydrates as her fuel source. Lactate began accumulating in Kerbi’s blood at the 7:00 mark with an RER of 1.04, a speed of 3.4 and a grade of 14. I know this because her RER was above the 100% carbohydrate level of 1.00, and this is also the time at which she felt uncomfortable to the point that the test
Osterberg KL, Pallardy, SE, Johnson RJ, Horswill CA. Carbohydrate exerts a mild influence on fluid retention following exercise-induced dehydration. J Appl Physiol 108:245-250, 2010.
For example, if a person had been able to consume lactose products for their life with no problems, but in an unfortunate event had to have a portion of his or her small intestine removed, there would be a change in the number of present lactase enzymes in the stomach. Because the lactase enzyme is stored in the small intestine, the person may now experience lactose intolerance due to the decrease in the presence of lactase. Knowing where the lactase enzyme is stored can aid physicians in understanding what will happen after a procedure or the introduction of a new medication. The experiment was conducted to determine the optimal ph of lactose required to produce the maximum amount of glucose. It was predicted that the optimal ph of lactose would be most efficient at lactose ph 6, and that the lower the ph, the amount of glucose produced would increase
b) Comprehensive diagnostic chemistry panel with significantly increased amylase (1626 with normal being 300-1100 U/L), total
As Fink points out, Carbohydrates, in the form of glucose, are the main source of fuel for all physical activity. Fats on the other hand are primarily used as a fuel source while resting and when performing moderately to low intensity exercises. (Fink, 2012, p.3) It’s these facts that are so significant. The event she participates in will be fairly short yet very intense with that in mind as an 800-meter track athlete the Carbohydrates are going to serve her the best. She will require immediate as well as a short sustained use of energy for her event. While she will be using both energy systems Fink states she will mainly be using anaerobic energy system which “is a major contributor to intense activities that last from 1 to 3 minutes.” (Fink, 2012, p. 50)
Lactase is an enzyme found in the digestive system. It is essential to the complete digestion of sugar in whole milk and milk products. Lactase specifically breaks down lactose, a complex sugar. Lactase cannot be absorbed by the body unless it is broken down by lactase into glucose and galactose. According to webMD, “Lacking lactase in their intestines, a person consuming dairy products may experience the symptoms of lactose intolerance…Abdominal cramping, flatulence (gas) and diarrhea can occur when a lactose intolerant person consumes milk products.” ("Lactase Enzyme oral : Uses, Side Effects, Interactions, Pictures, Warnings & Dosing - WebMD", n.d.) Lactase is not recommended for use in CHILDREN younger than 4 years of age. Safety and effectiveness in this age group have not been confirmed. (Kluwer, 2014)
LI was first recognized in the 1960s when researchers found black children responding unfavorably to milk in their diets (Harrison 812). Research led to the discovery that lactose, the major sugar in milk and related dairy products, was undigestible in some people because they were missing the enzyme lactase. Lactase breaks down lactose into its component monosaccharide sugars, glucose and galactose. In people missing lactase, lactose passes undigested through the small intestine. In some people, the undigested lactose passes through the remainder of their systems with no ill effects. In others, however, the undigested lactose becomes viscous and ferments in the colon (Englert and Guillory 903). The thickness of the liquid and the fermentation cause painful cramping, gas and sometimes diarrhea. Besides not being able to digest lactose, these people suffer from malabsorption, which causes them to receive little or none of milk's nutrients (Houts 110).1
Peppa M, Uribarri J, and Vlassara H. Glucose, Advanced Glycation End Products, and Diabetes Complications: What Is New and What Works. Clinical Diabetes October 2003 vol. 21 no. 4 186-187.
Kayla is a 24yo, G3 P1011, who was seen for a follow-up ultrasound assessment. The patient has chronic HTN but has not been on any medication. She has had some borderline elevated BP’s over the past couple of weeks. Her BP today when she arrived was 143/81 and 5 minutes later was 137/86. Her urine evaluation is negative for protein. She did have labwork performed revealing a creatinine of .58, uric acid of 6.2, with normal liver enzymes and a platelet count of 232,000. Her 24-hr urine was also normal at 200.
The patient has been facing symptoms including increased thirst, frequent urination, unexplained weight loss, and fatigue. Having these symptoms causes his body to not be able to start cellular respiration, Electron Transport Chain, and the Krebs cycle. The patient's blood glucose level, intracellular glucose levels, and interstitial levels are out of range. The blood glucose levels normal range is between 78-108 mg/dL, but the patients is 130 mg/dL. High blood glucose levels makes your blood mucky. This slows down circulation which causes the cells to not get oxygen and the nutrients that they need. This also causes people to be fatigue. Since he is fatigue he is unable to do cellular respiration. As the cells run out of oxygen they change
The absorptive state is the time during and right after eating a meal. The absorptive state lasts for four hours, during and after each meal. During this state glucose is the most important energy fuel. Amino acids and fats are used to form degraded protein, and small amounts are used to provide ATP. Metabolites are transformed to fat if they are not used for anabolism. Glucose is formed by the conversion of fructose and galactose, which are stored in the liver from the entrance of monosaccharides. Glucose is released into the blood, or converted to glycogen and fat. Some glucose enters the liver and is used for energy, and any that is not used will be stored in skeletal muscle as glycogen or in adipose cells as fat. Liver, skeletal muscle, and adipose cells use triglycerides as their primary energy source. Amino acid are also used by the liver to synthesize plasma proteins. Essentially all of the events that occur in the absorptive state are directed by insulin.
Lactose is found naturally in all kinds of milk and milk products as the carbohydrate; it can also be found in certain forms of foods. In a lactose tolerant person, the ileum secretes enzyme lactase, which catalyzes the digestion of lactose to form monosaccharides called galactose and glucose. Under normal circumstances, the body can absorb the simpler forms of sugar into the bloodstream. A deficiency in enzyme lactase causes a state of ineffective lactose breakdown. Two main conditions can cause lactose intolerance. Individuals experiencing a deficiency in lactase and lactose malabsorption are at a higher risk of developing the intolerance (Deng, Misselwitz, Dai & Fox, 2015). Lactose deficiency causes the small intestine to produce insufficient
This may leads to the misinterpretation of the exact condition of the patient. Plasma glucose concentration is approximately 11 % higher than the whole blood glucose due to higher amount of water in the plasma (D’Orazio, 2005; D’Orazio, 2006). Neely (1991) showed that, there is higher levels of glucose in plasma as compared to the whole blood. They conducted oral glucose tolerance study with 36 pregnant and 2 non-pregnant women at risk using glucose oxidase
The concern is with her chemical test results. Darlene's nitrates, urobilinogen, hematuria, special gravity =, and bilirubin were all negative or in normal range. Darlene's ph level was high, and can be a result of either a urinary tract infection, fever or gout. She had a small amount of leukocytes, and causes no concern at this time but should be monitored, but had large amounts of keytones. Glucose, and proteins in her urine. This can be related to a number of things including liver cirrhosis plasmacytoma, cardiac insufficiency, glomerulonephritis, or pregnancy, due to her high amount of protein. Darlene's high amount of glucose could indicate renal glycosuria, diabetes mellitus, or excessive consumption of carbohydrates. Darlene's keytones is a concern because there were large amounts also found in her chemical test and can be caused by a number of issues including diabetic ketoacidosis, stress, fever, nausea and vomiting, or not enough food intake. Darlene’s keytone results tells us that she is using fats to produce energy instead of glucose. Further testing needs to be done to pinpoint the cause of these
...ncorrect is going on. The patient in the study could take supplements and vitamins to help the body get what it needs.