Cases reported "Vitamin E Deficiency"

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21/85. Fat-soluble vitamin deficiency in pregnancy: a case report and review of abetalipoproteinemia.

    BACKGROUND: abetalipoproteinemia (ABL) is a metabolic disorder resulting in poor absorption of fat-soluble vitamins. CASE: Two pregnancies in a woman with ABL are reported, contrasting outcomes with subtherapeutic and normal vitamin levels. CONCLUSION: Fat-soluble vitamin levels in pregnancy are critical for many aspects of fetal development. This report details a congenital ophthalmologic finding that may be associated with vitamin a deficiency.
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22/85. Cerebellar syndrome in adult celiac disease with vitamin e deficiency.

    We studied a woman with adult onset celiac disease complicated by a cerebellar syndrome that progressed despite the resolution of the malabsorption symptoms with a gluten free diet. The patient presented vitamin e deficiency and the cerebellar symptoms improved with vitamin E therapy. This case supports the possible role of this deficiency in the development of the neurological complications of celiac disease.
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keywords = deficiency
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23/85. Serial somatosensory evoked potentials in a patient with familial hypobetalipoproteinemia, and vitamin e deficiency.

    Serial somatosensory evoked potentials (SEPs) to median and posterior tibial nerve stimulation in a case with familial heterozygous hypobetalipoproteinemia and vitamin e deficiency were investigated over a period of 4 years. In serial SEPs to posterior tibial nerve stimulation, interpeak latencies between N20 and P2 were delayed even in the early clinical stage, although the peak latency of N20 was normal. N20 latency was delayed when the patient noted paresthesia of the lower extremities. Interpeak latencies between N20 and P2 were progressively prolonged, and finally both peaks disappeared. Progressive dysfunctions of spinal posterior columns and peripheral somatosensory pathways were discovered by serial SEP studies.
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24/85. rhabdomyolysis after intramuscular iron-dextran in malabsorption.

    The case of a 59 year old white man who had chronic malabsorption and selective iga deficiency with severe iron deficiency is reported. In addition, he was deficient in vitamin E and selenium, important antioxidants which protect against lipid peroxidation. He was intolerant of oral iron and when treated with iron-dextran developed symptoms suggestive of polymyositis with evidence of rhabdomyolysis. It is suggested that free iron within iron-dextran activated free radicals, initiating lipid peroxidation and leading to polymyositis, rhabdomyolysis, and myoglobulinuria.
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keywords = deficiency
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25/85. Essential fatty acid deficiency mimicking porphyria cutanea tarda in a patient with chronic cholestasis.

    Essential fatty acid deficiency was documented in a 3-year-old boy with chronic cholestasis secondary to paucity of intrahepatic bile ducts (Alagille's syndrome). Dietary management had consisted almost exclusively of a proprietary formula with over 80% of the fat as medium-chain triglycerides. The bullous lesions involved mostly sun-exposed areas and were diagnosed initially as being compatible with acquired porphyria cutanea tarda. Improvement followed correction of the fatty acid abnormalities with a polyunsaturated fat supplement administered orally. We postulate that the association of fatty acid deficiency and abnormal vitamin E status contributed to skin damage, possibly involving photosensitizing compounds poorly cleared by the markedly cholestatic liver.
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ranking = 1.1882655280009
keywords = deficiency
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26/85. vitamin e deficiency in acquired fat malabsorption.

    Thirteen patients with adult-onset vitamin e deficiency due to fat malabsorption were investigated clinically and electrophysiologically. These patients had slightly or moderately decreased serum vitamin E (1.7-4.8 micrograms/ml, normal less than 6.0) or vitamin E/cholesterol ratio (0.21-0.31 mg/g, normal less than 0.35). Only one patient had typical neurological manifestations of vitamin e deficiency, which improved with supplementary vitamin E. The pathological findings in this patient were also compatible with vitamin e deficiency. This patient had poorly controlled diabetes mellitus due to advanced chronic pancreatitis. Reviewing previously reported cases of vitamin e deficiency with diabetes mellitus in chronic pancreatitis, the duration of deficiency until the onset of symptoms was shorter than in those cases without complications. Although adult patients with early, slight deficiency of vitamin E are generally asymptomatic, patients with diabetes mellitus tend to have early neurological symptoms. The vitamin E tolerance test should be used, because even in some patients with vitamin e deficiency due to malabsorption, the deficiency can be overcome by large oral doses of vitamin E.
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ranking = 2.3765310560018
keywords = deficiency
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27/85. Visual and somatosensory evoked potentials in vitamin e deficiency with cystic fibrosis.

    patients with cystic fibrosis (CF) and pancreatic malabsorption frequently have vitamin e deficiency. Affected patients may develop spinocerebellar degeneration with dysarthria, ataxia, proximal weakness, proprioceptive loss and areflexia. Of a highly selected group of 10 patients with vitamin E levels below 5 micrograms/ml (normal 5-20 micrograms/ml), 7 had abnormal neurological examinations, predominantly affecting vibration and joint position perception with some severely affected patients manifesting diminished visual acuity, tremor, ataxia and diffuse weakness. Evoked potential studies showed marked abnormalities in 3 patients, demonstrating deficits in the optic pathways and in the cervical cord dorsal column pathways. Evoked potential studies may supplement careful neurological examination in patients with CF before and after supplementation with vitamin E to evaluate their progression and response to treatment.
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ranking = 0.9902212733341
keywords = deficiency
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28/85. myoclonus and adult coeliac disease.

    A 56-year-old woman with coeliac disease developed myoclonus of cortical origin and palatal myoclonus with lesions of subcortical white matter on magnetic resonance imaging. myoclonus can thus be a prominent feature of coeliac disease encephalopathy. A slight vitamin e deficiency was found but does not satisfactorily explain this myoclonic encephalopathy, which remains of unknown origin.
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ranking = 0.19804425466682
keywords = deficiency
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29/85. Brown-bowel syndrome.

    Extensive brownish discoloration of the small bowel is rare and has been described mainly in association with malabsorption disorders. It is related to deficiency of the fat soluble vitamin E. Vitamin E is an antioxidant substance that prevents peroxidation of unsaturated fatty acids. vitamin e deficiency may result in oxidized lipids, which when polymerized with polysaccharides form the brown, PAS-positive pigment termed ceroid or lipofuscin. The deposition of pigmented granules in the effected tissues accounts for the brownish discoloration. We present three patients ultimately found to have "brown bowel syndrome (BBS)," in which the symptoms, preoperative findings, and respective diagnoses were different. In all three patients, serum Vitamin E levels were found to be extremely low.
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ranking = 0.39608850933364
keywords = deficiency
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30/85. Neuropathy secondary to vitamin e deficiency in acquired intestinal malabsorption.

    A patient with acquired intestinal malabsorption developed a motor-sensory polyneuropathy with a recurrent remittent course, normal CSF and reduced motor and sensory conduction velocities. Nerve biopsy showed axonal changes. serum DL-alpha-tocopherol was abnormally low. Six months supplementation with vitamin E was followed by normalization of DL-alpha-tocopherol serum levels and clinical and electrophysiological improvement.
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keywords = deficiency
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