Acid deficiency

Health and Safety. The U.S. FDA has affirmed R%- and S(—)-maHc acid as substances that are generally recognized as safe (GRAS) as flavor enhancers, flavoring agents and adjuvants, and as pH control agents at levels ranging from 6.9% for hard candy to 0.7% for miscellaneous food uses (42). R%- and A(—)-maHc acid may not be used in baby foods. MaHc acid is also cleared to correct natural acid deficiencies in juice or wine (43). 

Wine Making. Wine making is one of the principal areas of tartaric acid use. There is a relationship between the size of the grape crop and its tartaric acid content when grapes are pressed. In poor harvest years, the tartaric acid content is low in good harvest years, the tartaric acid content is high. Thus, in poor harvest years, tartaric acid often is added to correct acid deficiencies in wine. 

Fohc acid is a precursor of several important enzyme cofactors required for the synthesis of nucleic acids (qv) and the metaboHsm of certain amino acids. Fohc acid deficiency results in an inabiUty to produce deoxyribonucleic acid (DNA), ribonucleic acid (RNA), and certain proteins (qv). Megaloblastic anemia is a common symptom of folate deficiency owing to rapid red blood cell turnover and the high metaboHc requirement of hematopoietic tissue. One of the clinical signs of acute folate deficiency includes a red and painhil tongue. Vitamin B 2 folate share a common metaboHc pathway, the methionine synthase reaction. Therefore a differential diagnosis is required to measure foHc acid deficiency because both foHc acid and vitamin B 2 deficiency cause... 

Folic acid deficiency Hyperthermia Phenylketonuria Rheumatic disease Virilizing tumors Drugs and chemicals Androgenic chemicals Angiotensin-converting enzyme inhibitors Captopril, enalapril Antibiotics... 

The 4-phosphopantetheine group of CoA is also utilized (for essentially the same purposes) in acyl carrier proteins (ACPs) involved in fatty acid biosynthesis (see Chapter 25). In acyl carrier proteins, the 4-phosphopantetheine is covalently linked to a serine hydroxyl group. Pantothenic acid is an essential factor for the metabolism of fat, protein, and carbohydrates in the tricarboxylic acid cycle and other pathways. In view of its universal importance in metabolism, it is surprising that pantothenic acid deficiencies are not a more serious problem in humans, but this vitamin is abundant in almost all foods, so that deficiencies are rarely observed. 

The anemias discussed in this chapter include iron deficiency anemia, anemia in patients witii chronic renal disease pernicious anemia, and anemia resulting from a folic acid deficiency. Table 45-1 defines these anemias. Drugp used in treatment of anemia are summarized in die Summary Drug Table Drugp Used in die Treatment of Anemia. 

Follow die diet recommended by die primary healdi care provider because diet and drug are necessary to correct a folic acid deficiency. 

An IV fat emulsion contains soybean or safflower oil and a mixture of natural triglycerides, predominately unsaturated fatty acids. It is used in the prevention and treatment of essential fatty acid deficiency. It also provides nonprotein calories for those receiving TPN when calorie requirements cannot be met by glucose. Examples of intravenous fat emulsion include Intralipid 10% and 20%, Liposyn II 10% and 20%, and Liposyn III 10% and 20%. Fat emulsion is used as a source of calories and essential fatty acids for... 

Small amounts of trans-unsamrated fatty acids are found in ruminant fat (eg, butter fat has 2-7%), where they arise from the action of microorganisms in the rumen, but the main source in the human diet is from partially hydrogenated vegetable oils (eg, margarine). Trans fatty acids compete with essential fatty acids and may exacerbate essential fatty acid deficiency. Moreover, they are strucmrally similar to samrated fatty acids (Chapter 14) and have comparable effects in the promotion of hypercholesterolemia and atherosclerosis (Chapter 26). 

The role of essential fatty acids in membrane formation is unrelated to prostaglandin formation. Prostaglandins do not reheve symptoms of essential fatty acid deficiency, and an essential fatty acid deficiency is not caused by inhibition of prostaglandin synthesis. 

Symptoms of Essential Fatty Acid Deficiency in Humans Include Skin Lesions Impairment of Lipid Transport... 

In adults subsisting on ordinary diets, no signs of essential fatty acid deficiencies have been reported. How-... 

All 20 of the amino acids present in proteins are essential for health. While comparatively rare in the Western world, amino acid deficiency states are endemic in certain regions of West Africa where the diet relies heavily on grains that are poor sources of amino acids such as tryptophan and lysine. These disorders include kwashiorkor, which results when a child is weaned onto a starchy diet poor in protein and marasmus, in which both caloric intake and specific amino acids are deficient. 

Deficiency of fohc acid itself—or deficiency of vitamin Bi2, which leads to functional fohc acid deficiency—affects cells that are dividing rapidly because they have a large requirement for thymidine for DNA synthesis. ChnicaUy, this affects the bone marrow, leading to megaloblastic anemia. 

Folic acid deficiency is also related to megaloblastic anemia. Tetrahydrobiopterin is a co-factor for phenylalanine, tyrosine, and tryptophane hydroxilases — enzymes... 

Anemia from vitamin B12 or folic acid deficiency is treated effectively by replacing the missing nutrient. 

Folic add (plasma) 3.1-12.4 ng/mL or mcg/L (7-28 nmol/L) Used to determine folic acid deficiency. 

Anemia from vitamin BI2 or folic acid deficiency is treated effectively by replacing the missing nutrient. Both folic acid and vitamin B12 are essential for erythrocyte production and maturation. Replacing these factors allows for normal DNA synthesis and, consequently, normal erythropoiesis. 

When treating folic acid deficiency, an initial daily dose of 1 mg/day by mouth typically is effective. Absorption of folic acid generally is rapid and complete. However, patients with malabsorption syndromes may require larger doses (up to 5 mg/day). Similar to vitamin B12 deficiency, resolution of symptoms and reticulocytosis is prompt, occurring within days of commencing therapy. Hgb will start to rise after 2 weeks of therapy and may take from 2 to 4 months to resolve the deficiency completely. Afterwards, if the underlying deficiency is corrected, folic acid replacement can be discontinued. However, in cases where folic acid is consumed rapidly or absorbed poorly, chronic replacement may be required. 

In patients with folic acid deficiency, methylmalonic acid may be normal, and homocysteine may be high. Monitor hemoglobin periodically, and reevaluate patients who fail to normalize hemoglobin levels after 2 months of therapy. 

Intravenous lipid emulsions are used as an energy source in PN and to prevent or treat essential fatty acid deficiency. 

The essential fatty acids in humans are linoleic acid (C-18 2 N-6) and a-linolenic acid (C18 3 N-3). Arachidonic acid (C20 4 N-6) is also essential but can be synthesized from linoleic acid. Administration of 2% to 4% of total daily calories as linoleic acid should be adequate to prevent essential fatty acid deficiency in adults (e.g., infusion of 500 mL of 20% intravenous lipid emulsion once weekly).7 Biochemical evidence of essential fatty acid deficiency can develop in about 2 to 4 weeks in adult patients receiving lipid-free PN, and clinical manifestations generally appear after an additional... 

Essential fatty acid deficiency Deficiency of linoleic acid, linolenic acid, and/or arachidonic acid, characterized by hair loss, thinning of skin, and skin desquamation. Long-chain fatty acids include trienes (containing three double-bonds [e.g., 5,8,11-eicosatrienoic acid, or Mead acid trienoic acids) and tetraenes (containing four doublebonds [e.g., arachidonic acid]). Biochemical evidence of essential fatty acid deficiency includes a trieneitetraene ratio greater than 0.4 and low linoleic or arachidonic acid plasma concentrations. 

Hyland, K., and Clayton, P. T. (1990). Aromatic amino acid deficiency in twins. J. Inherited Metab. Dis. 13 301-304. 

Thompson, A. J., E. T. Thome et al. (2004). Complementation of notabilis, an abscisic acid-deficient mutant of tomato Importance of sequence context and utility of partial complementation. Plant Cell Environ. 27(4) 459 171. 

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