Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Hepatic glycogenosis in a patient with poorly controlled type 1 diabetes mellitus

혈당 조절이 불량한 제1형 당뇨병 환자에서 발생한 간의 당원축적증

  • Jin, Hye-Young (Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine) ;
  • Kang, Dae-Young (Department of Pathology, Chungnam National University Hospital, College of Medicine, Chungnam National University) ;
  • Choi, Jin-Ho (Division of Pediatric Endocrinology and Metabolism, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine)
  • 진혜영 (울산대학교 의과대학 소아과학교실) ;
  • 강대영 (충남대학교 의과대학 병리학교실) ;
  • 최진호 (울산대학교 의과대학 소아과학교실)
  • Received : 2009.05.06
  • Accepted : 2009.09.22
  • Published : 2009.11.15
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Abstract

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.

당뇨병 환자에서 간비대와 간 기능 이상이 동반될 경우 간 내 당원축적증과 비알콜성 지방간염을 감별해야 한다. 저자들은 인슐린 치료를 제대로 받지 않아 혈당 조절이 불량한 환자에서 저혈당과 간비대로 발현한 간 내 당원축적증을 경험하여 보고한다. 만 6세경 1형 당뇨병으로 진단받은 환아로 10세경 우상복부 통증과 반복되는 고혈당과 저혈당으로 내원하였으며, 당시 진찰상 둥근 얼굴, 우측 늑골연 하방 6 cm 정도에서 만져지는 간비대가 있었다. 검사상 소변 케톤 양성, 당화혈색소는 9.6%였다. 간 효소치가 증가되어 있었고, 혈중 콜레스테롤/중성지방 250/267 mg/dL였다. 바이러스성 간염, 자가면역성 간염, 대사성 간 질환에 대한 검사는 모두 정상이었다. 복부 초음파 및 컴퓨터 단층 촬영상 지방간과 간비대 소견을 보였다. 간 조직 검사상 PAS 염색에서 양성, diastase-PAS 염색에서는 음성으로 전형적인 당원축적증의 소견을 보였다. 고혈당을 보일 때마다 초속효성 인슐린을 투여하고 심한 저혈당의 증상이 동반된 경우 글루카곤을 투여하였으며 생옥수수 전분 가루를 간식으로 먹이면서 저혈당 횟수가 감소하여 퇴원하였다. 현재 외래에서 초속효성 인슐린으로 혈당 조절하면서 관찰 중이다. 제1형 당뇨병 환자에서 당원축적증은 간비대와 간 효소치의 상승을 야기할 수 있는 원인이다. 본 증례에서는 심한 저혈당이 반복되어 하루에도 수 차례 글루카곤을 투여하였으며, 생옥수수 전분 가루을 투여하면서 저혈당이 호전되었다. 엄격한 혈당 관리로 간 비대 및 간 기능 이상이 호전될 수 있으므로 적절한 인슐린 치료로 혈당을 조절하고 저혈당을 예방해야 하겠다.

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References

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