POLYCYSTIC OVARIES AND GLUCOSETOLERANCE IN HEPATIC GLYCOGEN STORAGE DISEASE

Abstract

Polycystic ovaries (PCO) are found in both lean and obese women in association wiih hyperinsulinism and insulin resistance. The hepatic glycogen storage diseases (GSDs) are an heterogeneous group of inherited disorders of carbohydrate metabolism characterised by hypoglycaemia, lactic acidosis, hyperlipidaemia and hyperuricaemia in which abnormalities of insulin secretion may be present. We therefore studied 16 female patients with GSDs by performing pelvic ultrasonography and oral glucose tolerance tests (1.75g/kg 10 maximum 75g glucose) during which samples were drawn every 20 minutes for 2 hours. Fasting could not be standardised and varied between 2 and 3 hours. 8 patients had glucose-6-phosphatase deficiency (GSD 1a); 6 had amylo-1,6-glucosidase deficiency (GSD III); I had phosphorylase deficiency (GSD VI); and I had phosphorylase b kinase deficiency (GSD IXa). 8 patients were pre-pubertal; mean age was 15.9 years (range 4.5-31.3). The group overall was not obese with age correcied mean body mass index being 111% (range 100-129). All patients except the two youngest (4.5 and 4.6 years) had ultrasonographic evidence of PCO (87.5%). 2 out of 10 adults were symptomatic with oligo- or secondary amenorrhoea. Basal plasma glucose levels varied from 0.4 to 7.9 mmol/l (mean 4.4) with peak values varying between 5.8 and 17.4 minol/l (mean 9.6). Mean basal insulin was 39.9 mU/l (range 3.4 to 106.2), with mean incremental insulin being 1454.7 mU/L (range 26.9 to 4249.5). The prevalence of PCO in this group is much greater than the 22% seen in the general adult female population. In particular, the early age of onset is quite striking. They display glucose intolerance with abnormal insulin secretion. This supports the hypothesis that insulin may be causative of PCO as well as having important implications for women with GSDs.