Prader-Willi syndrome (PWS) is due to genetic alterations on chromosome 15q11-q13, and represents the most common genetic cause of obesity. Weight excess associated with PWS is often massive, and its complications are the major causes of morbidity and mortality, including diabetes mellitus. Type 2 diabetes mellitus (T2DM) accounts for the vast majority of cases of PWS patients with diabetes. The relationship between the obese condition and the development of diabetes, however, is not clear and may be different than that seen in individuals with simple obesity. The bulk of evidence has demonstrated that PWS subjects exhibit a state of relative hypoinsulinemia, with higher insulin sensitivity, despite severe obesity. Data about differences in insulin secretion in PWS, however, are still conflicting. Other reports have shown that PWS subjects and BMI-matched controls have similar insulin levels and are similarly insulin resistant. T2DM may be present with the classic symptoms, but most PWS subjects are asymptomatic and diabetes-related complications are infrequent. In spite of this, periodic surveillance for T2DM should be undertaken, and evaluation of diabetes risk is recommended prior and during growth hormone (GH) therapy. Apart from diet and increased exercise, management of T2DM needs similar pharmacological agents as with nonsyndromic obesity-related diabetes.
|Title of host publication||Diabetes Associated with Single Gene Defects and Chromosomal Abnormalities|
|Publisher||S. Karger AG|
|Number of pages||6|
|Publication status||Published - Mar 21 2017|
ASJC Scopus subject areas
- Biochemistry, Genetics and Molecular Biology(all)
- Immunology and Microbiology(all)