Type III glycogenosis with deposition of urate and amyloid

A case of a 44-year-old man with hepatic form of glycogenosis was presented. The patient had abdominal distension and muscular weakness. The glucose tolerance test showed a diabetic pattern, though he had hypoglycemia in fasting state. The fructose tolerance test showed an ability of conversion from fructose to glucose. The double glucagon test showed no rise of blood glucose in fasting state but a rise 2 hours after meal. These symptoms and laboratory data supported the clinical diagnosis of type III glycogenosis. At autopsy, glycogen was markedly deposited in the liver, and slightly in the kidneys and heart. The glycogen pooled in the hepatic cells histochemically showed a normal reaction to several glycogen stainings. Electron microscopy by using Thiéry's method revealed that the pooled glycogen particles were clearly arranged as rosettes measuring 1,000A in largest diameter composed of clustered monoparticulates. There were marked hyalinization of the islets of Langerhans containing amyloid. As to its pathogenesis, this change can be interpreted as a morphological expression of the hypofunction of beta-cells ascribed to long-standing hypoglycemia.