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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifteen years after onset of a malabsorption syndrome, a 49-year-old man had sensory and oculomotor disorder with marked vitamin E deficiency. After 6 months of treatment with high parenteral doses of vitamin E, the neurologic signs slowly receded, but the patient died of gastrointestinal hemorrhage. Autopsy and sural nerve biopsy showed the changes in both central and peripheral nerves; these changes are considered characteristic of vitamin E deficiency.
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PMID:Neurologic disorder of vitamin E deficiency in acquired intestinal malabsorption. 650 27

A survey of vitamin D status in 152 patients with chronic gastrointestinal conditions and 104 patients with chronic liver diseases is presented. Mild deficiency was common and severe deficiency, as judged by plasma 25-OHD levels less than 8 nmol/l, was encountered in every disease category tested. In the gastrointestinal disease patients, deficiency was significantly more common in patients following gastroenterostomy than other gastric surgery, in patients with active Crohn's disease than in those with inactive disease and in patients with chronic pancreatitis or pancreatic carcinoma with cholestatic features than in those without cholestatic features. Deficiency was as common in patients with Crohn's disease who had not been treated surgically as in those who had. There was no significant correlation between plasma 25-OHD levels and any laboratory index of malabsorption or malnutrition except for serum albumin in the gastric surgery patients, haemoglobin and ESR in the Crohn's disease patients and albumin and vitamin E in the group of patients with gastrointestinal disorders taken as a whole. In the chronic liver disease patients, those with late primary biliary cirrhosis had lower plasma 25-OHD levels than those with histological Stage I and II disease who all had normal levels, and those with pruritus and jaundice were more commonly severely deficient. Whatever the underlying disease process, patients with other coincidental medical conditions were much more likely to be deficient as were patients with cholestasis. Evidence of secondary hyperparathyroidism and osteomalacia on bone histology indicated the clinical relevance of the vitamin D deficiency. This study showed no relationship between abnormal plasma vitamin D binding protein levels and vitamin deficiency.
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PMID:A survey of vitamin D deficiency in gastrointestinal and liver disorders. 654

Evidence that vitamin E is important for normal neurological function in humans is presented. First, in abetalipoproteinaemia early therapy with vitamin E delays and may prevent the development of the neurological complications, and in patients with established lesions treatment can arrest or reverse the neuropathy. Second, in other chronic disorders of fat absorption with severe vitamin E deficiency, neurological manifestations which are very similar to those described in untreated abetalipoproteinaemia can be improved by vitamin E. Vitamin E supplementation is therefore advisable for all patients with chronic fat malabsorption who have low serum vitamin E concentration. Serum vitamin E concentrations should also be measured in patients with spinocerebellar disorders, whatever the aetiology.
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PMID:Vitamin E and neurological function: abetalipoproteinaemia and other disorders of fat absorption. 655 2

In order to characterize the mechanism(s) causing vitamin E deficiency during chronic childhood cholestasis, we studied 6 vitamin E-deficient cholestatic children with clinical evidence of neurologic dysfunction (group A), 4 vitamin E-sufficient cholestatic children and young adults with normal neurologic status (group B), and 6 vitamin E-sufficient noncholestatic children (group C). Intestinal absorption of dl-alpha-tocopherol (assessed by an oral tolerance test) was markedly impaired (p less than 0.001) in group A compared with groups B and C, which did not differ from each other. Intraluminal total bile acid concentrations were markedly depressed in group A compared with age-matched controls (0.50 vs. 7.00 mM, p less than 0.001), whereas concentrations were low normal in group B. Intramuscular dl-alpha-tocopherol was well absorbed in 4 group A subjects and corrected abnormal hydrogen peroxide hemolysis. Our data suggest that low intraluminal bile acid concentrations result in malabsorption and deficiency of vitamin E in children with prolonged, severe cholestasis. Intact plasma transport and tissue uptake of vitamin E during cholestasis suggest that intramuscular vitamin E should be utilized for prevention and therapy of the neurologic abnormalities caused by vitamin E deficiency.
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PMID:Mechanism causing vitamin E deficiency during chronic childhood cholestasis. 661 8

To characterize differences in intestinal absorption of fat-soluble vitamins during cholestasis, intestinal absorption of vitamin E was compared with that of 25-hydroxyvitamin D in eight infants and young children with prolonged neonatal cholestasis. Oral tolerance tests were performed using 100 IU/kg/dose dl-alpha-tocopherol and 10 micrograms/kg/dose 25-hydroxyvitamin D. Mean vitamin E absorption was only 1.0% to 1.9% of that of control children, whereas 25-hydroxyvitamin D absorption was 22.5% to 25.1% of that of controls. Although intestinal absorption of both vitamins is impaired during cholestasis, the severity of vitamin E malabsorption far exceeds that of 25-hydroxyvitamin D.
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PMID:Comparison of vitamin E and 25-hydroxyvitamin D absorption during childhood cholestasis. 663 97

Plasma vitamin E levels were found to be decreased (less than 0.5 mg) in thalassemia and in 17 out of 20 patients with Gaucher's disease, where the levels were two standard deviations below the normal mean value. In the latter, the decrease in vitamin E levels correlated with the severity of the clinical expression of the disease and correlated inversely with the degree of hepatosplenomegaly and serum tartrate-resistant acid phosphatase activity. In both diseases, there was no evidence for intestinal malabsorption of the lipid-soluble vitamin. In spite of the different etiology, pathophysiology, and clinical expression, severe vitamin E deficiency could result in both diseases by a common mechanism. In thalassemia, rapid consumption of vitamin E occurs while neutralizing oxidative damage in the pathological erythrocyte membranes and in other tissues. In Gaucher's disease, lysosomal accumulation of glucocerebroside may stimulate phagocytes into a maintained "respiratory burst" with excessive production of oxygen free radicals, resulting in increased utilization and eventual deficiency of vitamin E. Efficacy of antioxidant therapy was evaluated by administration of vitamin E with and without canthaxanthin, which has similar antioxidant properties to beta-carotene, to patients with beta-thalassemia. The results showed increased serum vitamin E levels and a decrease in the extent of erythrocyte lipid membrane peroxidation, while no significant changes occurred in hemoglobin levels and in transfusion requirements.
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PMID:Vitamin E deficiency due to increased consumption in beta-thalassemia and in Gaucher's disease. 675 58

Evidence has accumulated that vitamin E is essential for normal neurological function. It is a fat-soluble vitamin, and, therefore, deficiency states are most likely to occur in patients with fat malabsorption. If such patients have low serum vitamin E concentrations, it is important to initiate appropriate vitamin E therapy with the aim of restoring and maintaining normal serum vitamin E concentrations and preventing long-term neurological disability. This is likely to become a more frequent problem with the increasing survival of patients with chronic fat malabsorptive states. As a corollary, it would also seem reasonable to measure serum vitamin E concentrations in all patients with spinocerebellar disorders, whatever the supposed cause.
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PMID:Use of vitamin E for prevention and treatment of spinocerebellar disorders. 685 66

In summary, we propose the following scheme (Figure 5) to describe the role of peroxidation in the pathophysiology of SCA. Sickle erythrocytes are more susceptible to peroxidation than are normal erythrocytes. This increased susceptibility to peroxidation is, in part, due to decreased blood vitamin E levels and abnormal membrane phospholipid organization induced by sickling. The peroxidative damage of sickle erythrocytes may accelerate or contribute to loss of cell deformability and to chronic hemolysis. Peroxidative damage can produce abnormal cellular properties, such as potassium leak and reduced filterability, and contribute to formation of ISCs. Increased red cell rigidity can initiate episodes of capillary obstruction, leading to vasoocclusive painful crises and to tissue infarction. Liver dysfunction as well as increased production of bilirubin secondary to hemolysis could result in bile sludging and decreased secretion of bile salts into the intestinal lumen. Reduced bile salt secretion leads to partial fat and vitamin E malabsorption. Vitamin E deficiency enhances red cell susceptibility to peroxidation and promotes a vicious cycle in SCA. Although we have not studied factors that might initiate peroxidative damage, sickle hemoglobin and excess body iron should be considered as potential sources. Our studies suggest that vitamin E supplementation to sickle-cell patients could be of clinical benefit.
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PMID:Peroxidation, vitamin E, and sickle-cell anemia. 695 61

Brownish discoloration of the muscular layers of the small intestine has been described in association with deficiency of fat soluble vitamin E occurring in various disorders with malabsorption. A patient presenting with diarrhea ultimately found to have "brown bowel syndrome" is reported with serum levels of vitamin E before and during treatment. A brief review of the nature and significance of ceroid pigmentation of the intestine is included; however, the pathophysiology of the brown bowel syndrome remains to be fully elucidated.
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PMID:The "brown bowel syndrome": a case report. 713 38

The mechanisms and consequences of Vitamin E deficiency were studied in 12 children presenting with chronic cholestasis. Preliminary results indicate that: (1) Vitamin E serum levels are lowest in children with the long-lasting cholestasis and in children in whom fat malabsorption is deepest. (2) Signs of neurologic dysfunction involving peripheral nerves, cerebellum, eye movements and retina were present in 7 children. (3) In vitro study of RBC showed increased hemolysis with oxidating agents and in physiological saline. Degree of hemolysis was inversely related to the serum level of vitamin E. (4) Increased platelet aggregation was observed in 8 patients; 6 of these also had low levels of serum Vitamin E.
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PMID:[Vitamin E and cholestasis in children]. 714 9


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