Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P17174 (aspartate aminotransferase)
 14,872 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The unexpected autopsy finding of Wernicke encephalopathy in three children who died after prolonged enteral feeding prompted us to examine the incidence of thiamine deficiency in three high-risk pediatric populations. We also measured riboflavin and pyridoxine activity in the same groups. We used activated enzyme assays (erythrocyte transketolase, glutathione reductase, aspartate aminotransferase) to assess tissue stores of the dependent vitamin cofactors (thiamine (vitamin B1), riboflavin (vitamin B2), and pyridoxine (vitamin B6), respectively). Using our own reference ranges based on data from 80 healthy adults and children, we prospectively investigated the B vitamin status of three groups of children: (1) 27 patients who were fed solely by nasogastric tube for more than 6 months, (2) 80 children admitted to a pediatric intensive care unit for more than 2 weeks, and (3) 6 children receiving intensive chemotherapy. The upper limits for stimulated enzyme activity in control subjects were unaffected by age or gender (16% for transketolase, 63% for glutathione reductase, 123% for aspartate aminotransferase). Using these limits, 10 (12.5%) of 80 patients receiving intensive care and 4 of 6 patients receiving chemotherapy were thiamine deficient. Elevated levels returned to normal after thiamine supplementation. No patients were pyridoxine deficient, but 3 (3.8%) of the 80 patients receiving intensive care and 1 of the 6 patients receiving chemotherapy were also riboflavin deficient. We conclude that unrecognized thiamine deficiency is common in our pediatric intensive care and oncology groups. This potentially fatal but treatable disease can occur in malnourished patients of any age and is probably underdiagnosed among chronically ill children. Our findings may be applicable to other high-risk pediatric groups.
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PMID:Thiamine, riboflavin, and pyridoxine deficiencies in a population of critically ill children. 140 85

A 27-year-old woman developed Wernicke's encephalopathy in the 18th week of her pregnancy after 11 weeks of vomiting accompanied by weight loss of 21 kg and moderately abnormal liver function tests. The patient recovered after thiamine therapy but the fetus was lost. Review of the literature published during the last 25 years revealed an additional 14 cases of Wernicke's encephalopathy complicating hyperemesis gravidarum. All patients vomited for at least 4 weeks. Six of the 15 patients (40%) had aspartate aminotransferase values > 100 U/l, much higher than the rate reported in previous series of patients with hyperemesis gravidarum (7%). This suggests the need for parenteral thiamine supplementation in patients with severe hyperemesis gravidarum lasting more than 3 weeks, especially those with abnormal liver function, and supports the hypothesis that the hepatic abnormality plays a pathogenetic role in the development of Wernicke's encephalopathy in hyperemesis gravidarum.
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PMID:Wernicke's encephalopathy in hyperemesis gravidarum: association with abnormal liver function. 818 22