高脂血症の経過中急性壊死性すい炎にて死亡した若年者糖尿病の１剖検例

An autopsy case of juvenile-onset diabetes in a patient who died of acute necrotizing pancreatitis following massive hyperlipidemia is reported. A 23-year-old woman was first admitted to the hospital in 1978 because of eruptive xanthoma which appeared at the age of 19. She had no family history of diabetes or hyperlipidemia but her parents were cousins. She was diagnosed as having type III hyperlipidemia and non-insulin-dependent diabetes mellitus. Both lipoprotein lipase (LPL) and hepatic triglyceride lipase (HTGL) activities were decreased. Restriction of energy intake resulted in marked improvemnt of the hyperglycemia and hyperlipidemia together with increases in LPL and HTGL activities. On the second and third admission, she was treated with glibenclamide and insulin. Through-out the course, levels of fasting blood glucose, triglyceride, cholesterol and broad beta band changed in parallel, while LPL and HTGL activities varied inversely with these parameters. On Sept 14, 1982, (age 27), she was admitted to the hospital because of severe abdominal pain and vomiting since the previous evening. On admission, she was stuporous, and blood pressure was 76/mmHg. The abdomen was distended and tender. Xanthoma was seen on the lower legs. The plasma was milky and the triglyceride level was 9, 000 mg/dl, cholesterol 1, 000 mg/dl, and chylomicron 8, 500 mg/dl. The blood glucose level was 556 mg/dl, urine ketone was positive, and the pH was 7.322. The urine amylase level was 39, 369 units. Treatments with continuous insulin infusion, fluids and heparin, and replacement transfusion were ineffective. She died on the next day. The autopsy revealed acute hemorrhgic pancreatitis. The pancreas was enlarged and there was hemorrhage and coagulation necrosis in the body and tail, and fat necrosis in the interlobular portions of the pancreatic head and adjacent adipose tissue. Veins and capillaries at the site of coagulation necrosis were filled with thrombi including cholesterol crystals. Lymphatics in the serosa of the stomach, and the small and large intestines were filled with chyle. The acute hemorrhgic pancreatitis was probably due to massive hyperchylomicronemia to which she was predisposed by the combination of subnormal LPL activity and diabetes mellitus, but the direct trigger of the terminal episode is unknown.