INS and Glycogen storage disease due to glycogenin deficiency: This is supported by hyperandrogenism and PCOS described in patients with insulinoma (41, 42), and in hyperinsulinemic patients with glycogen storage disease (43), and by increased prevalence of hyperandrogenism and PCOS-like ovarian appearances in patients with type 1 diabetes, attributed to supraphysiological exogenous insulin in peripheral circulation (44, 45).