Origins and extinctions: paleontology in Chicago.
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Biomedical subjects
Publications and source records attributed to R A Kerr.
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In 60 thiamine deficient patients, the mean erythrocyte transketolase activity after activation by thiamine diphosphate cofactor in vitro, representing the apparent sum of holoenzyme and apoenzyme activities, was 0.609 (SD 0.166) U/g Hb before thiamine therapy and rose to 0.772 (SD 0.152) U/g Hb immediately after the administration of thiamine to the patients. The difference between these values, 0.163 (SD 0.130) U/g, is the mean activity of transketolase protein which can be activated by thiamine in vivo but not by thiamine diphosphate in vitro. This difference correlated with low initial erythrocyte transketolase activity in these patients, but not with their alcohol intake, liver function or diagnoses.
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Patients (n = 104) were judged to be thiamine deficient by the criteria of erythrocyte transketolase activity (ETK) less than 0.6 U/g of hemoglobin, or greater than 17% increase in this activity on addition of thiamine pyrophosphate in vitro (TPP effect). ETK activated by TPP in vitro (AETK) was related to ETK by a linear regression of slope greater than or equal to 1, implying that transketolose apoenzyme (apoTK) was constant or decreased as ETK decreased. For most patient groups the value of apoTK was 0.1 U/g and the slope 1.033 to 1.050. In the subgroup of non-vomiting drinkers with Wernicke's encephalopathy (WE), the slope of the linear regression of AETK on ETK was 1.21, so that apoTK decreased as ETK decreased. Comparison of these data is consistent with a difference in the TK of WE drinkers from that of others. Generally, any variation of TPP effect was due only to variation of ETK. We recommend measurement of ETK, without TPP effect, for the assessment of thiamine nutrition.
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This paper reports two studies carried out on patients with the Wernicke-Korsakoff (W-K) syndrome and control subjects. All had been mental hospital inpatients for at least 12 months. In the first study the changes in the thiamine-dependent enzyme erythrocyte transketolase (TK) which followed the administration of oral thiamine are described. Essentially the two patient samples responded similarly. In the second study patients who had been maintained on high-dose thiamine for several months stopped this treatment abruptly. The subsequent decline in TK is described. In W-K patients this decline was rapid and virtually complete at four weeks. In control subjects the decline was much more gradual. These findings offer support to other evidence suggesting a difference between TK in W-K and control subjects.
Two techniques were used to seek variants of human erythrocyte transketolase and to test for any association of the Wernicke-Korsakoff syndrome, a thiamin-deficiency disease, with a particular variant of this thiamin-dependent enzyme. Apparent Km values for the cofactor thiamin diphosphate were similar for patients and controls. However, isoelectric focussing separated erythrocyte transketolase into different isoenzymes characterized by pI values in the range 6.6-9.2. Six distinct patterns of isoenzymes were found in thirty-six healthy control subjects. The isoenzyme pattern for thirty-nine out of forty-two patients suffering from the Wernicke-Korsakoff syndrome was identical to a pattern found in only eight of thirty-six control subjects, a highly significant association (P less than 0.001). This association suggests that a variant transketolase and thiamin deficiency together contribute to the pathogenesis of the brain damage of the Wernicke-Korsakoff syndrome by some mechanism independent of apparent Km values for thiamin diphosphate.
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One hundred and seventy-eight subjects completed a questionnaire regarding ECT. The sample comprised three groups of approximately equal size: a group of patients who had received ECT, a group of visitors to ECT-treated psychiatric patients, and a group of visitors to non-ECT-treated psychiatric patients. Misconceptions about ECT were common throughout, particularly in the young, those giving films and television as a source of information and those visiting patients not receiving ECT. Fewer misconceptions occurred among those who were more highly educated or had experience of ECT either personally or via a visited friend or relative. Less fear of the procedure was expressed by those given the treatment and those who had the treatment explained to them by a doctor. Over half of the patient group denied having had ECT explained to them.
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