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Alström Syndrome International
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High versus low carbohydrate meal effects on serum triglyceride and blood glucose levels in 5 patients with Alström Syndrome.R.B. Paisey, K. Williams, C. Warren, P. Taylor The severe insulin resistance and sometimes severe hypertriglyceridaemia in Alström Syndrome present a clinical management challenge. We obtained South Devon Healthcare Ethics Committee permission and informed consent to study metabolic response to meals in 5 Alström Syndrome patients age 18-33 years, BMI 28-34 kilograms per metre square. On day 1 all 5 patients aimed for a 700 kilocalorie, 75 per cent fat breakfast, and on day 2 a 700 kilocalorie 77 per cent carbohydrate breakfast. Peak 3 hour serum triglyceride increases after the high fat meal were median 66 per cent, interquartile range 57.5 per cent, and peak 2 hour median blood glucose response was –3 per cent, interquartile range 10.3 per cent. Corresponding figures for the high carbohydrate meal were triglycerides 12 per cent, interquartile range 29.7 per cent, glucose 23.8 per cent, interquartile range 30.9 per cent. The two patients with normal glucose tolerance had no rise in blood glucose 2 or 3 hours after the high carbohydrate meal, but the three with frank diabetes had 25, 36 and 81 per cent increases in blood glucose. Even in patients with severe insulin resistance syndrome, the metabolic responses to high fat and high carbohydrate meals were heterogenous, but indicated loss of glucose control after high carbohydrate in those with frank diabetes. |
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Last Modified: June 23, 2008 |