Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The purpose of this report is to present an overview on vitamin E distribution, requirements, absorption and biochemical and nutritional aspects. A continuous interest in biochemical functions is recently developed and vitamin E certainly plays an important role throughout the body. The best known of its effects and still actively considered in recent years is the role as an important biological antioxidant. The red blood cell is an ideal model for studying the antioxidant role of vitamin E in cell membranes. Nutritional deprivation is a rare occurrence in developed countries. In prematurely delivered newborns the deficiency is due to marginal stores and to transient malabsorption but it can also be iatrogenic. In infants and adults vitamin E deficiency does occur in syndromes characterized by increased consumption or reduced absorption. Various gastrointestinal disorders induce, with steatorrhoea, marked alteration of vitamin E levels. Cystic fibrosis (CF), the commonest cause of pancreatic insufficiency during the first decades of life, is of particular interest. The fat malabsorption, often severe, may not well respond to pancreatic therapy and the hepatobiliary disease, increased in frequency with improved survival, induce a further reduction in intestinal bile salt concentration. Several manifestations have been attributed to vitamin E deficiency in CF and, although overt neurological complications seem to be relatively uncommon, it is recommended to maintain an adequate supplementation.
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PMID:[Vitamin E: physiology and pathology]. 328 49

Brown bowel syndrome (BBS) is a rare disease accompanied by deposits of lipofuscin predominantly in smooth muscle cells of the gastrointestinal tract. To determine whether cells other than smooth muscle cells show pigment deposition we studied biopsies of the stomach, small and large intestine, and thyroid gland of a 52-year-old male with malabsorption syndrome. Light and electron microscopy found lipofuscin pigment in vascular endothelial cells and in smooth muscle cells of the muscularis mucosae, arterioles and venules of the gastrointestinal tract and thyroid gland. All other cells of bowel and thyroid gland, such as epithelial cells, Schwann cells, nerve fibers, fibroblasts, macrophages and leukocytes, showed no such pigment inclusions. Intracellular lipofuscin deposition is thought to be caused by a deficiency of vitamin E. Such a deficiency existed in our patient and was attributed to a six year history of malabsorption syndrome with complete atrophy of the villi of the intestinal mucosa. Lipofuscin pigmentation has been reported in numerous organs of patients with BBS but not, until now, in the thyroid gland or in endothelial cells of lymph vessels. The origin of lipofuscin pigment is not known exactly. The prevailing opinion is that it derives from degenerating mitochondria. Our findings, however, suggest that autophagocytotic processes may also play a role in the formation of lipofuscin.
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PMID:Brown bowel syndrome with manifestation in the gastrointestinal tract and thyroid gland. 336 51

Two patients with the syndrome of Ramsay Hunt (dyssynergia cerebellaris myoclonica, DCM), associated with malabsorption due to adult coeliac disease, are reported. Both presented with progressive cerebellar ataxia, action myoclonus, and epilepsy. One had gastrointestinal symptoms (recurrent diarrhea and weight loss which responded satisfactorily to a gluten-free diet), but the other did not. In both patients, jejunal biopsy revealed subtotal villous atrophy; serum folate and vitamin E level were also reduced. Neither a gluten-free diet nor vitamin supplements improved the neurological picture. However, some symptomatic relief was afforded by treatment with clonazepam, sodium valproate, carbamazepine, and piracetam. It could be argued that the association between these two disorders is coincidental. However, since we have found this combination in 2 of 14 consecutive cases with DCM, a causal relationship seems likely, although the underlying mechanism remains unknown. Patients with the Ramsay Hunt syndrome should be investigated for malabsorption, and also undergo small intestinal biopsy.
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PMID:Ramsay Hunt syndrome and coeliac disease: a new association? 847 11

Studies in patients with abetalipoproteinaemia, other chronic and severe fat malabsorptive states and a selective defect in vitamin E absorption, together with neuropathological studies in the vitamin E deficient human, monkey and rat indicate that vitamin E is important for normal neurological function. Appropriate vitamin E supplementation is, therefore, advisable for all patients with chronic fat malabsorption who have low serum vitamin E concentrations. Serum vitamin E concentrations should also be measured in patients with spinocerebellar disorders, whatever the aetiology.
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PMID:Vitamin E--its role in neurological function. 354 Sep 25

There is increasing evidence that vitamin E is essential for normal neurological function. In abetalipoproteinemia, which is the most severe deficiency state known in man, development of the associated spinocerebellar syndrome can be prevented by early vitamin E therapy. A neurological disorder similar to that seen in abetalipoproteinemia, comprising progressive ataxia, hyporeflexia, and proprioceptive loss, has been described in children and adults with chronic fat malabsorption and vitamin E deficiency. The neuropathological changes in such patients resemble those seen in vitamin E-deficient monkeys. Recent reports suggest that spinocerebellar degeneration may be caused by a selective defect of vitamin E absorption without other evidence of gastrointestinal disease.
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PMID:Vitamin E and the nervous system. 355 52

We report the cases of two adult patients with cystic fibrosis affecting the pancreas and liver, who also had severe vitamin E deficiency and neurologic disease. The most prominent clinical features were abnormal eye movements, diminished reflexes, decreased vibratory and position sense, ataxia, and muscle weakness. Treatment with intramuscular injections of vitamin E partially corrected the neurologic deficits. Vitamin E absorption tests documented severe malabsorption, which was later alleviated by the addition of dessicated ox bile to the regimen of alpha-tocopheryl acetate. These studies suggest that a decreased intraluminal concentration of bile salts is an important factor in the development of severe vitamin E deficiency and in the poor response to oral replacement therapy that is seen in some patients with cystic fibrosis.
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PMID:Vitamin E deficiency and neurologic disease in adults with cystic fibrosis. 359 48

Vitamin E (tocopherol) concentrations in blood plasma were determined in 48 infants and correlated with their nutritional status. Infants were divided into two groups as following: group I (n : 12) estimated well-nourished, and group II (n : 36) appreciated undernourished. Clinical nutritional status was evaluated according to their weight, height and skinfold thickness of triceps percentiles. Plasma vitamin E levels were analysed by a modification of the spectrophotometric micro-technique of Fabiank et al. (using 0.2 ml of plasma). There was difference in serum tocopherol levels between two groups: 1.21 (0.21) mg/dl: mean (+/- SEM) in group I in front of 1.84 (0.18) mg/dl in group II (0.10 greater than p greater than 0.05). On the other hand, vitamin E concentrations were correlated with the skinfold thickness of triceps percentiles by a logarithm curve: y = 2.25-0.31 1n X (r: 0.35, p less than 0.02). Probably, serum vitamin E levels do not reflect the tissue store status in undernourished infants without malabsorption.
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PMID:[Serum vitamin E in well-nourished and malnourished infants]. 366 50

In order to study the frequency of biochemical vitamin E deficiency in chronic alcohol-induced pancreatitis, we measured plasma vitamin E and total blood lipids in 44 patients with chronic pancreatitis and 83 control subjects (44 normal controls; 39 Crohn's disease controls). Mean plasma vitamin E and mean ratio vitamin E/total blood lipids, a more sensitive indicator of vitamin E status, were significantly lower in chronic pancreatitis when compared with either control group. A low vitamin E/total lipids ratio was found in 75% of patients with pancreatitis. Within the chronic pancreatitis group, mean plasma vitamin E and the ratio vitamin E to total lipids were significantly lower in those with steatorrhoea (23 patients--pancreatic steatorrhoea subgroup) than in those without (21 patients--pancreatic non-steatorrhoea subgroup). 91% of the pancreatic steatorrhoea subgroup had a low vitamin E/total lipids ratio. However, patients without pancreatic steatorrhoea also had significantly lower levels of plasma vitamin E and the ratio vitamin E/total lipids when compared to controls. We conclude that biochemical vitamin E deficiency is common in chronic alcohol-induced pancreatitis, particularly in patients with steatorrhoea, and that factors other than fat malabsorption may be responsible for vitamin E deficiency in pancreatic non-steatorrhoea.
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PMID:Biochemical vitamin E deficiency in chronic pancreatitis. 369 79

Although secondary vitamin E deficiency causes a reversible neurologic disorder in children with chronic cholestasis, the effect of this deficiency state on other organ systems is unknown. We studied the effects of vitamin E therapy on selected gastrointestinal and hepatic functions in five children with chronic cholestasis and well-documented biochemical and neurologic evidence of vitamin E deficiency. After 2 to 3 years of oral or parenteral vitamin E therapy, there was no improvement in fecal fat losses, severity of vitamin E malabsorption (as measured by an oral vitamin E tolerance test) or total serum fatty acid concentrations. Serial analyses of liver function blood tests demonstrated a marked decline in fasting serum cholylglycine concentrations during 18 to 31 months of vitamin E therapy, while other liver function tests showed no consistent changes. We conclude that vitamin E deficiency does not appear to alter intestinal absorption of fat or vitamin E; however, vitamin E deficiency may further impair already compromised hepatic function during pathologic conditions such as cholestasis.
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PMID:Correction of vitamin E deficiency in children with chronic cholestasis. II. Effect on gastrointestinal and hepatic function. 379 2

A male born to first cousins presented at 12 months with hypocalcemic convulsions, rickets, epistaxis due to vitamin K deficiency, and extremely low serum levels of beta-carotene and vitamin A. Liver function was altered moderately (glutamic-oxaloacetic transaminase, 55 U/L; glutamic-pyruvic transaminase, 37 U/L; lactate dehydrogenase, 255 U/L; alkaline phosphatase, 437 U/L). To correct the deficiencies, 8,000 IU vitamin D/day, 10,000 IU vitamin A/day, and intramuscular administration of vitamin K1 were required. At 9 years, he presented signs of neuromuscular affection, and the serum vitamin E level (measured for the first time) was extremely low. Classic lipid malabsorption syndromes (abetalipoproteinemia, chronic cholestasis, mucoviscidosis, coeliac disease, Whipple's disease) were excluded by appropriate examinations. Composition of duodenal bile acids was characterized by undetectable levels of cholic acid metabolites, and only chenodeoxycholic acid metabolites were present. Serum total bile acid concentration was normal, with an atypical low cholic acid/chenodeoxycholic acid ratio and abnormal presence of 3 beta-OH-delta 5-cholenic acid and 6-OH-bile acids. Urinary bile acid composition was also characterized by elevated 6-OH-bile acids. Known enzymopathies of the bile acid synthetic pathway were excluded (cerebrotendinous xanthomatosis, cerebro-hepato-renal syndrome of Zellweger, coprostanic acidemia). Bile acid pool sizes were determined by using stable isotopes: cholic acid pool size [2.90 (N, 32 +/- 16) microM/kg] and chenodeoxycholic acid pool size [10.8 (N, 32.6 +/- 9.9) microM/kg] were extremely low; fractional turnover rates of both bile acids were in a normal range.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Malabsorption of liposoluble vitamins in a child with bile acid deficiency. 379 31


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