Search

Background: Alström syndrome is an autosomal recessive condition characterized by obesity, insulin resistance and hypertriglyceridaemia. Responses to fat and carbohydrate ingestion are important in planning dietetic advice and may help to explain the mechanism of metabolic disorder in the syndrome., Methods: After a 12-h fast, five Alström subjects received a 3.1 MJ (742 kcal), 75.8% fat breakfast on day 1, and a 3.3 MJ (794 kcal), 77.5% carbohydrate breakfast on day 2. Serum glucose, triglyceride and insulin levels were measured at baseline, and 2 and 3.5 h post-meal. Abdominal computerized tomography in three subjects and magnetic resonance imaging in one demonstrated distribution of abdominal fat., Results: Body fat was distributed subcutaneously, as well as viscerally. There were no changes in serum glucose, insulin or triglycerides after the high fat meal. Triglycerides remained stable after the high carbohydrate meal but glucose and log insulin levels increased [8.4 +/- 4.1 to 13.4 +/- 6.9 mmol L(-1) (P < 0.05) and 2.6 +/- 0.27 to 3.15 +/- 0.42 pmol L(-1) (P < 0.05), respectively]., Conclusions: Dietetic advice in Alström syndrome must include calorie restriction to reduce obesity, which is predominantly subcutaneous. This study has shown that low carbohydrate advice may prove more effective than fat restriction in control of hyperglycaemia and hyperinsulinism. A single high energy meal does not exacerbate hypertriglyceridaemia.

It had been suggested that long-term lowering of blood glucose by sulfonylureas in non-insulin-dependent (NIDD) diabetes is not due to a sustained increase in insulin secretion. We have re-examined this question. Thirteen nonobese NIDD patients not controlled on diet alone were studied prospectively on treatment with chlorpropamide for over 3 mo. Of these, 9 were also studied after 1 yr. Improvement in glucose tolerance was associated with an increase in fasting and postglucose serum insulin and C-peptide concentration. We conclude that at least for over 1 yr chlorpropamide increases insulin secretion. After 3 and 12 mo the fasting proinsulin percentage of immunoreactive insulin was increased.