Endocrine Abstracts

Congenital Generalised Lipodystrophy (CGL) is characterised by an inability to store energy in adipose tissue. Integral to CGL is severe insulin resistance resulting in hyperinsulinaemia and premature Type 2 diabetes (DM). Sulphonylureas are ineffective in CGL whilst insulin therapy is complicated by insulin resistance and absence of subcutaneous fat. We explored the possibility of using Metformin and Pioglitazone (both insulin sensitising agents) in CGL.
PD presented aged 34yrs with deteriorating home blood glucose (HBG) (12-20mmol/l) (HbA1c 9.4%) following 16yrs of adequate control by diet. Examination revealed absent subcutaneous fat but no evidence of microvascular disease or hypertension. As sulphoylureas are ineffective in CGL Metformin therapy 500mg tds was introduced for a period of 3 months. This had no demonstrable effect on glycaemic control. Pioglitazone was added at 30mg od for 3 months with no effect on HbA1c (9.2%) or HBG.
PD has now been commenced on twice daily insulin therapy. Early improvement in HBG and HbA1c (8.0%) has been observed. In summary we present a case of CGL-associated diabetes in which insulin sensitising drugs (Pioglitazone in conjunction with Metformin) have been used for the first time. They have proved to be ineffective in improving blood glucose control in our patient.