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

During the recent resurgence of interest in the clinical uses of vitamin E, one of the major foci of attention has been the neurologic role of vitamin E in humans. Studies in patients with secondary vitamin E deficiency, caused by fat malabsorption disorders and total parenteral nutrition lacking an adequate supply of vitamin E, have elucidated a clinical disorder and histologic lesions of the nervous system and muscle that closely resemble those of experimental vitamin-E-deficient animal models. Investigations of the primary form of human vitamin E deficiency, the isolated vitamin E deficiency syndrome, have further substantiated the relationship between neurologic dysfunction and human vitamin E deficiency. It is now clear that vitamin E is an essential nutrient necessary for the optimal development and maintenance of the integrity and function of the human nervous system and skeletal muscle. The task for future study is to determine the mechanism by which vitamin E deficiency causes degeneration of selective regions of the nervous system and to investigate possible benefits of vitamin E supplementation in other neurologic disorders. In addition, further study of the isolated vitamin E deficiency syndrome promises to teach us more about normal physiologic mechanisms of vitamin E absorption and transport in humans.
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PMID:Vitamin E deficiency and neurologic disease. 306 Jan 70

A progressive neurological syndrome with cerebellar signs, abnormal proprioception, areflexia and Babinski response was observed in a child with chronic intestinal malabsorption. There was no ophtalmoplegia or retinitis pigmentosa. Electromyography and biopsy showed no axonopathy or myopathy. Two other members of the family were also affected. The serum Vitamin E corrected the serum Vitamin E levels within a few months and led to secondary neurological improvement. The authors underline the importance of searching for Vitamin E deficiency and its cause in patients, especially children, with signs of spino cerebellar degeneration. Substitative therapy may have a favorable influence on the neurological condition even when administered late.
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PMID:[Neurological manifestations in relation to vitamin E deficiency, caused by a defect of biliary acid synthesis]. 320 25

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

A 62-year-old man and his maternal uncle had a selective vitamin E deficiency without generalized fat malabsorption. A progressive neurological disorder comprising ataxia, areflexia, and loss of proprioception developed in their sixth and seventh decades. The vitamin E deficiency is thought to be due to abnormally accelerated utilization, excretion, or degradation of the vitamin. This adult-onset spinocerebellar syndrome is due to vitamin E deficiency not caused by malabsorption.
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PMID:Adult-onset spinocerebellar syndrome with idiopathic vitamin E deficiency. 347 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

Four patients had accumulation of ceroid in smooth muscle (lipofuscinosis), which indicated severe or uncontrolled malabsorption, with confirmed vitamin E deficiency in three cases. The distribution of the pigment was systematic, and there seemed to be an association between malabsorption syndrome and vitamin E deficiency. Vitamin E supplementation seems to be indicated in such patients, and it is suggested that studies of smooth muscle function should be made in cases of heavy accumulation of ceroid.
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PMID:Accumulation of ceroid in smooth muscle indicates severe malabsorption and vitamin E deficiency. 362 1

The clinical, biochemical, and histological features of 27 children with syndromic paucity of the interlobular bile ducts are described. All presented in the first 5 months of life, 21 with jaundice, two with spontaneous bleeding due to vitamin K malabsorption in addition to jaundice, two with pruritus, and two with failure to thrive. Interlobular bile ducts were abundant in liver biopsies from five (18% of cases) in the first 6 months of life. The degree of portal fibrosis and cellular infiltrate was mild in all except three patients. Clinically significant heart lesions occurred in 52% but only 22% had peripheral pulmonary stenosis. Characteristic facial appearances were present in only 70%; embryotoxon and vertebral anomalies were present in 56 and 33%, respectively. Two infants died of cardiovascular complications, one of alimentary bleeding and one of progressive liver disease. Complications of vitamin K deficiency occurred in 15%, vitamin D deficiency in 30%, and vitamin E deficiency in 37%. Survivors at ages of 19 months to 16.5 years had considerable morbidity with pruritus occurring in 70%, jaundice in 48%, xanthomas in 30%, 74% having hepatomegaly and 63% splenomegaly. All had abnormal biochemical tests of liver function, 90% had growth retardation, and 50% developmental delay. We conclude that differentiation from extrahepatic biliary atresia can be difficult if biliary flow cannot be demonstrated. Prevention of fat-soluble vitamin deficiency is essential. Further research is required to decrease the morbidity associated with this syndrome in infancy.
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PMID:Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty; severe morbidity throughout early childhood. 368 72

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

We describe nine patients with fat malabsorption in whom a spectrum of vitamin E deficiency was present. Early deficiency was generally asymptomatic, and intermediate deficiency produced some impairment. Ataxia, weakness, reflex changes, impaired vision, and pigment retinopathy were associated with chronic, advanced deficiency. In the last group, delayed central somatosensory conduction and amplitude reduction of the electroretinogram were present. In adults, a severe vitamin E deficiency state existed for more than 5 years before producing measurable neurologic damage. The clinical picture is less homogeneous than previously suggested, and electrophysiologic abnormalities need not predate clinical dysfunction.
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PMID:The spectrum of neurologic disorder from vitamin E deficiency. 371 53


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