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Hepatic glycogenosis in a patient with poorly controlled type 1 diabetes mellitus

Korean Journal of Pediatrics 2009;52(11):1279-1282.
Published online November 15, 2009.
Hepatic glycogenosis in a patient with poorly controlled type 1 diabetes mellitus
Hye Young Jin1, Dae-Young Kang2, Jin-Ho Choi1
1Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, Asan Medical Center Children’s Hospital, University of Ulsan College of Medicine, Seoul, Korea
2Department of Pathology, Chungnam National University Hospital, College of Medicine, Chungnam National University, Daejeon, Korea
혈당 조절이 불량한 제1형 당뇨병 환자에서 발생한 간의 당원축적증
진혜영1, 강대영2, 최진호1
1울산대학교 의과대학 소아과학교실
2충남대학교 의과대학 소아과학교실
Jin-Ho Choi, Email: jhc@amc.seoul.kr
Hepatomegaly and liver dysfunction might develop in patients with diabetes mellitus due to glycogen deposition or nonalcoholic steatohepatitis. We experienced a case of hepatic glycogenosis in a patient with type 1 diabetes mellitus who presented with recurrent hypoglycemia, suggesting impairment of glycogenolysis and gluconeogenesis. A 10-year-old girl with a 4-year history of type 1 diabetes mellitus was admitted because of recurrent hypoglycemia and abdominal pain in the right upper quadrant. She had Cushingoid features and hepatomegaly that extended 6 cm below the right costal margin. Laboratory data and radiologic examination revealed elevated liver enzyme levels due to fatty liver. Periodic acid-Schiff (PAS) staining revealed intense glycogen deposition in the cytoplasm of the hepatocytes and PAS reactivity was lost with diastase treatment. At 2 months after administration of glucagon injection and uncooked cornstarch between meals and at bedtime, the hypoglycemic episodes and liver dysfunction improved. It is important to distinguish hepatic glycogenosis from steatohepatitis, because it is possible to prevent excessive hepatic glycogen storage in hepatic glycogenosis cases by strictly controlling blood glucose level and by glucagon administration. To prevent severe hypoglycemic symptoms accompanied by hepatic glycogenosis, we suggest that uncooked cornstarch, which is effective in maintaining blood glucose level, can also be administered.
Key Words: Starch, Glucagon, Hypoglycemia, Diabetes mellitus, Type 1

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