4.3 Review

New insights and therapies for the metabolic consequences of Alstrom syndrome

Journal

CURRENT OPINION IN LIPIDOLOGY
Volume 20, Issue 4, Pages 315-320

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MOL.0b013e32832dd51a

Keywords

Alstrom syndrome; dyslipidaemia; insulin resistance

Funding

  1. The National Clinical Service Group
  2. Alstrom syndrome UK
  3. Torbay Hispital, UK
  4. Birmingham Children's Hospital, UK

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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 Alstrom 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 Alstrom families from childhood and adolescence. Recent findings Studies of body fat distribution, insulin resistance, glucose metabolism and dyslipidaemia in Alstrom 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 Alstrom syndrome and suggest novel approaches to understanding diabetes mellitus, hyperlipidaemias and heart disease in the general population.

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