Vitamin E deficiency syndromes

Because a number of vitamin E deficiency syndromes are responsive to dietary selenium, the possibility of selenium functioning as a lipid antioxidant has been examined. Some very powerful selenium antioxidants are known (Woodbridge, 1959). In the vitamin E-deficient chick, dietary selenite inhibited in vivo and in vitro lipid peroxidation (Zalkin et al., 1960). Further, antioxygenic activity was found for various selenium compounds in simple lipid peroxidation reactions selenoamino acids were weak antioxidants. On the basis of this evidence and the known incorporation of selenium into tissue proteins, Zalkin et al. (1960) suggested that the antioxidants formed in the chick may be selenoamino acids and selenoproteins. Bieri (1961) and Bieri et al. (1961) have reported extensive evidence for inhibition of in vitro lipid peroxidation of liver, kidney, and heart tissue from selenium fed chickens. They also suggested that the lipid antioxidant may be a selenoprotein. Olcott et al. (1961) showed that selenomethionine is a stronger lipid antioxidant than methionine and that selenomethionine decomposes lipid peroxides. 

Persons at risk for EFA deficiency tend to be the same as those at risk for vitamin E deficiency. Some signs are shared by both defidencies. Premature infants may be at risk for EFA deficiency because of their low stores of lipids and their rapid growth, especially when they are fed diets that do not contain EFAs. For example, fats have been omitted from diets used to feed preterm infants (to avoid a variety of complications). EFA deficiency may develop later in life with fat malabsorption syndromes, EFA deficiency has presented in adults fed by total parenteral nutrition for longer periods, where EFAs had not been included in the liquid diet. 

Disorders associated with vitamin E deficiency (ataxia with vitamin E deficiency (AVED), chronic cholestatic liver disease, cystic fibrosis, chronic pancreatitis, short bowel syndrome, progressive systemic sclerosis,... 

The second pathologic finding, ceroid pigment, a product of autoxidation of unsaturated lipids, has been reported in the smooth muscle of the stomach, intestine, bronchial wall, and bladder of children djdng with cystic fibrosis of the pancreas, particularly if they were over 2 years of age (Blanc et al., 1958 Kerner and Goldbloom, 1960). It was less frequent in infants who died with biliary atresia, presumably because of their earlier death. These lesions, which also have been described in adults with the malabsorption syndrome (Pappenheimer and Victor, 1946 Braunstein, 1961) bear a close resemblance to the findings in the vitamin E-deficient animal where the amount of ceroid deposition can be correlated with the level of unsaturated fatty acids in the diet (Mason et al., 1946). 

The great diversity of syndromes which occur in different species when attempts are made to produce vitamin E deficiency has been commented on on many occasions. For this reason, as well as dealing with those syndromes in sheep and cattle in which it is well established that vitamin E has an etiological role, mention is made of the possible role vitamin E may have in the genesis of reproductive disorders and liver disease in cattle and sheep and in maintaining normal growth and function. 

There are few documented cases of harmful effects resulting from excessive intake of essential fatty acids in children. The syndrome related to vitamin E deficiency in premature infants described by Hassan et al (1966) was referred to earlier. Presumably, this could be exacerbated by increasing the level of polyunsaturated fat in the diet unless the levels of vitamin E were also increased at the same time. Johnson et al (1974) have provided some evidence that the occurrence of retrolental fibroplasia in premature infants is associated with vitamin E deficiency. 

Vitamin E deficiency was first described in children with fat malabsorption syndromes, principally abe-talipoproteinemia, cystic fibrosis, and cholestatic liver disease. Subsequently, humans with severe vitamin E deficiency with no known defect in lipid or lipoprotein metabolism were described to have a defect in the a-TTP gene. 

Vitamin E deficiency occurs only rarely in humans and almost never as a result of inadequate vitamin E intakes, therefore, interactions with other nutrients have not been well studied. There have been reports of vitamin E deficiency symptoms in persons with protein-calorie malnutrition. Vitamin E deficiency does occur as a result of genetic abnormalities in a-TTP and as a result of various fat malabsorption syndromes. Vitamin E supplementation halts the progression of the neurologic abnormalities caused by inadequate nerve tissue a-tocopherol and, in some cases, has reversed them. 

Vitamin E deficiency is seen rarely in humans. However, there may be a risk of vitamin E deficiency in premature infants because the placenta does not transfer a-tocopherol to the fetus in adequate amounts. When it occurs in older children and adults, it is usually a result of lipoprotein deficiencies or a lipid malabsorption syndrome. These include patients with abetalipoproteinemia or homozygous hypobeta-lipoproteinemia, those with cholestatic disease, and patients receiving total parenteral nutrition. There is also an extremely rare disorder in which primary vitamin E deficiency occurs in the absence of lipid malabsorption. This disorder is a rare autosomal recessive neurodegenerative disease caused by mutations in the gene for a-TTP. This disorder is known as ataxia with vitamin E deficiency (AVED). Patients with AVED have extraordinary low plasma vitamin E concentrations (<5pgml ) and have an onset between 4 and 18 years, with progressive development of peripheral neuropathy,... 

Water-soluble vitamins removed by hemodialysis (HD) contribute to malnutrition and vitamin deficiency syndromes. Patients receiving HD often require replacement of water-soluble vitamins to prevent adverse effects. The vitamins that may require replacement are ascorbic acid, thiamine, biotin, folic acid, riboflavin, and pyridoxine. Patients receiving HD should receive a multivitamin B complex with vitamin C supplement, but should not take supplements that include fat-soluble vitamins, such as vitamins A, E, or K, which can accumulate in patients with renal failure. 

Deficiency of vitamin E is characterized by low serum tocopherol levels and a positive hydrogen peroxide hemolysis test. This deficiency is believed to occur in patients with biliary, pancreatic, or intestinal disease that is characterized by excessive steatorrhea. Premature infants with a high intake of fatty acids exhibit a deficiency syndrome characterized by edema, anemia, and low tocopherol levels. This condition is reversed by giving vitamin E. 

Vitamin E is a generic term that represents four tocopherols and four tocotrienols of varying biological potency. The term tocopherol correctly refers to the methyl-substituted derivatives of to-col and is not synonymous with the term vitamin E. The tocopherols and tocotrienols may be referred to collectively as tocochromanols. Many of the diverse deficiency syndromes observed in animals experimentally deprived of vitamin E can be explained by the vitamin s acting as an antioxidant in stabilizing unsaturated lipids in biological membranes. 

It should be noted that deficiency states for some vitamins (e.g., pantothenic acid) are practically unknown in human beings. In such cases, deficiency states may be simulated by feeding the subject an appropriate vitamin antagonist. In another series of situations, vitamin deficiencies can be brought about by interfering with their absorption intentionally or may be the result of a disease process. Thus, fat-soluble vitamin deficiency may develop in cases of fat malabsorption syndromes (steatorrhea) sprue, pancreatic insufficiency, and bile duct obstruction. 

A severe and chronic deficiency of vitamin E is associated with a characteristic neurological syndrome with typical clinical, neuropathological, and electrophysio-logical abnormalities in both humans and experimental animals. Chronic vitamin E... 

Pathological syndromes may result in muscular spasm, as seen in the exertional myopathies, or weakness, as seen in hyperkalemic periodic paralysis (HYPP). Similarly, infectious diseases may result in muscular rigidity (C. tetani infection (tetanus)) or paralysis (C. botulinum intoxication (botulism)). Overt rhabdomyolysis may result from the ingestion of the coccidiostats monensin, rumensin and lasalocid, or one of a number of plant mycotoxins. Dietary deficiencies of selenium or vitamin E have also been described as having severe deleterious effects on skeletal muscle health. 

Vitamin E is used as a dietary supplement and for the treatment of deficiency syndromes. 

Money DFL. 1970. Vitamin E and selenium deficiencies and their possible etiological role in the sudden death in infants syndrome. N Z Med J 71 32-34. 

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