New insights and therapies for the metabolic consequences of Alström syndrome.

PURPOSE OF REVIEW: Recent studies have begun to evaluate the heterogeneity of insulin resistance in syndromes associated with type 2 diabetes, dyslipidaemia and associated cardiac, renal and hepatic consequences. These insights are of particular importance in Alström syndrome in which all of these conditions coexist from a young age with considerable morbidity and reduction in life expectancy. Clear definition of the phenotype in the syndrome may clarify biochemical pathways of crucial importance in propensity to diabetic complications and heart disease in the general population. This review will focus on ways in which more effective treatments can be put in place for Alström families from childhood and adolescence. RECENT
FINDINGS: Studies of body fat distribution, insulin resistance, glucose metabolism and dyslipidaemia in Alström syndrome will be discussed as well as genotypic and phenotypic variation in the syndrome in the context of recent metabolic studies in insulin resistance. Potential therapies including low-carbohydrate diet, niaspan and incretins will be considered.
SUMMARY: These new insights will encourage early introduction of protective nutrition, exercise and drug therapies in Alström syndrome and suggest novel approaches to understanding diabetes mellitus, hyperlipidaemias and heart disease in the general population.