Carbohydrates abnormalities

Carbohydrate abnormalities, such as renal glycosuria (a transport defect), pentosuria (enzyme deficiency, xylitol dehydrogenase I. lactase deficiencies, fructose intolerance, galactosemia, galacloki-nase deficiency, oxalosis, and several glycogenoses (von Gierke s, Forbes . Andersen s, Hers s. and Tarui s diseases). 

Heinie, R.A., Levy, R.I., Fredrickson, D.S. and Gorlin, R. Lipid and carbohydrate abnormalities in patients with angiographically documented coronary artery disease. 

Formulas tend to contain isolates as the protein source to eliminate or reduce the presence of carbohydrates that ate the cause of flatulence and abnormal stools. Care is taken to provide adequate nutrition and to use proteins processed in such a way as to minimize or eliminate any antinutritional factors. The formulation of a typical soy-based infant formula is also given in Table 17. 

The citric acid cycle is the final common pathway for the aerobic oxidation of carbohydrate, lipid, and protein because glucose, fatty acids, and most amino acids are metabolized to acetyl-CoA or intermediates of the cycle. It also has a central role in gluconeogenesis, lipogenesis, and interconversion of amino acids. Many of these processes occur in most tissues, but the hver is the only tissue in which all occur to a significant extent. The repercussions are therefore profound when, for example, large numbers of hepatic cells are damaged as in acute hepatitis or replaced by connective tissue (as in cirrhosis). Very few, if any, genetic abnormalities of citric acid cycle enzymes have been reported such ab-normahties would be incompatible with life or normal development. 

Postma and Stock [81] showed that HPr or E-I mutants were unable to grow on PTS carbohydrates suggesting that transport without phosphorylation did not take place in apparent contradiction with the studies presented above. The explanation may be that facilitated diffusion via PTS carriers is observed only in abnormal situations, carbohydrate being transported by the incorrect PTS carrier (galactose via the mannose carrier) or transport via a mutated carrier. Efflux, which also reflects facilitated diffusion, is more common for PTS carriers. 

Ketosis An abnormal increase of ketone bodies present in conditions of reduced or disturbed carbohydrate metabolism. 

In addition to these interconversions, the metabolism of fat and the metabolism of carbohydrate are inseparably related. This fact is most clearly demonstrated by the appearance of such abnormal products of fat oxidation as the so-called ketone bodies in the blood and urine whenever the supply of carbohydrate is deficient or in cases where the organism is unable to metabolize this foodstuff. Whether ketonuria results because the metabolism of fat must occur concomitantly with that of D-glucose (ketolysis), or whether the presence of D-glucose prevents any fat breakdown because it is preferentially oxidized (antiketogenesis) is still a moot question. 

Diabetes mellitus (DM) is a group of metabolic disorders characterized by hyperglycemia and abnormalities in carbohydrate, fat, and protein metabolism. It results from defects in insulin secretion, insulin sensitivity, or both. Chronic microvascular, macrovascular, and neuropathic complications may ensue. 

Disease state-specific formulations are designed to meet specific nutrient requirements and to manage metabolic abnormalities. Unfortunately, scientific and clinical research supporting their efficacy is minimal, except for low carbohydrate formulations supplemented with specific fatty acids and antioxidants for patients with acute respiratory distress syndrome. Oral supplements are not intended for tube feeding. They are sweetened to improve taste and are therefore hypertonic. 

H2. Heller, P., Weinstein, H. G., West, M., and Zimmerman, H. J., Enzymes in anemia A study of abnormalities of several enzymes of carbohydrate metabolism in the plasma and erythrocytes in patients with anemia, with preliminary observations of bone marrow enzymes. Ann. Internal Med. 53, 898-913 (1900). 

Alcohol consumption is very difficult to assess. There is widespread belief that individuals underreport their intake and there are no reliable laboratory tests available for definitive diagnosis of alcohol abuse. A combination of abnormalities in the plasma activity of gamma-glutamyl transferase (GGT or yGT), AST and reduction in erythrocyte mean cell volume (MCV) maybe useful and all are routine lab. tests. A potential marker of interest is carbohydrate-deficient transferrin (CDT) which is an abnormal isoform of serum transferrin arising due to defects in the attachment of carbohydrate chains to the protein core. Unfortunately, CDT is a somewhat specialized test, not performed by most laboratories. Other markers which have attracted some research interest are ethyl sulphate and ethyl glucuronide. Excretion in the urine of these metabolites occurs for up to 50 hours after binge drinking so they offer a useful index of recent heavy alcohol intake. 

Viral protease cleaves precursor proteins into proteins required for viral replication. The inhibitors of this protease (saquinavir, ritonavir, indinavir, and nelfinavir) represent abnormal proteins that possess high antiviral efficacy and are generally well tolerated in the short term. However, prolonged administration is associated with occasionally severe disturbances of lipid and carbohydrate metaboUsm Biotransformation of these drugs involves cytochrome P450 (CYP 3A4) and is therefore subject to interaction with various other drugs inactivated via this route. 

Biotin (vitamin B ) is widespread in foods and is also synthesized by intestinal bacteria. It is a coenzyme for the carboxylation of pyruvate, acetyl-coenzyme-A (CoA), propionyl CoA, and /1-methyl-crotonyl CoA and is involved in fatty acid formation and in energy release from carbohydrates. In humans deficiencies only occur in patients with an abnormal gut flora and manifests itself as exfoliative dermatitis and alopecia. 

Diabetes mellitus is a heterogeneous group of disorders characterized by abnormalities in carbohydrate, protein, and lipid metabolism. The central disturbance in diabetes mellitus is an abnormality in insulin production or action or both, although other factors can be involved. Hyperglycemia is a common end point for all types of diabetes mellitus and is the parameter that is measured to evaluate and manage the efficacy of diabetes therapy. 

In diabetes mellitus, there is either insulin deficiency or insulin resistance in peripheral tissues which lead to hyperglycemia and glycosuria. Insulin corrects the various abnormalities of carbohydrate metabolism by its action on various tissues. 

Ketone A negative result is typical. Positive results can indicate physiological stress (e.g., fasting, pregnancy). Ketoacidosis and other abnormalities of carbohydrate, lipid, and amino acid metabolism may yield high levels of ketones in the urine and positive strip results. 

Iourin O, Mattu TS, Mian N, Keir G, Winchester B, Dwek RA, Rudd PM (1996) The identification of abnormal glycoforms of serum transferrin in carbohydrate deficient glycoprotein syndrome type I by capillary zone electrophoresis. Glycoconj J 13 1031-1042... 

In animals, manganese deficiency results in wide-ranging disorders, e.g,. impaired growth, abnormal skeletal structure, disturbances of reproduction, and defective lipid and carbohydrate metabolism. Although overt manganese deficiency has nol been induced in humans, some forms of epilepsy... 

In plants, the meristematic tissues in general are particularly rich in potassium, as are other metabolically active regions, such as buds, young leaves, and ioot tips. Potassium deficiency may produce both gross and microscopic changes in the structure of plants. Effects of deficiency reported include leaf damage, high or low water content of leaves, decreased photosynthesis, disturbed carbohydrate metabolism, low protein content and other abnormalities. 

Martinez, J., Keane, P. M., Gilman, P. B., and Palascak, J. E. (1983). The abnormal carbohydrate composition of the dysfibrinogenemia associated with liver disease. 

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