Women with Turners syndrome (TS) have been reported to have 11.5% relative risk of type 1 diabetes mellitus (DM) and 4.3% relative risk of type 2 DM. However, it is now evident that the traditional categorisation of DM may not be appropriate in this condition as the defect of glucose homeostasis often presents in young non-obese women. In fact, the pattern of insulin secretion in TS seems more likely to be due to beta cells dysfunction or insufficiency, which is reminiscent of maturity onset diabetes of the young but so far assessment of established diabetes in TS has not taken place.
Objective: To treat TS patient with established diabetes with an insulin secretagogue targeting first phase of insulin response (FPIR)
Methods: We measured FPIR to intravenous glucose overload (IVGTT) in eight diabetic TS patients and tested the effect of Repaglinide which was chosen for its property to target FPIR. Patients underwent two weeks of wash-out from other anti diabetic agents and Repaglinide has been administrated for 1114 weeks.
Results: All women exhibited impaired FPIR at baseline with improvement after treatment with Repaglinide. Results on glucose homeostasis are shown in Table1. The drug has been generally well tolerated, diarrhoea has been observed as side effect, only in two cases.
|Fasting BM (mmol/l)||10.6±5.2||7.3±2.5|
|Fasting Insulin (mUI/l)||11±6.3||14.6±7.9|
Conclusion: TS patients exhibit a particular form of diabetes which cant be included the categories of IDDM and NIDDM. Repaglinide appears to be a very effective treatment of TS related diabetes and formal comparison to other agents is now indicated.