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Glycogenic hepatopathy in a Korean girl with poorly controlled type 1 diabetes mellitus

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주제분류소아과학

초록

Glycogenic hepatopathy (GH) is a rare complication of type 1 diabetes mellitus. We report the case of a 13-year-old diabetic female with poorly controlled blood sugar levels who presented with abdominal pain and distention 1 month in duration. She exhibited tender hepatomegaly, an elevated lipid profile, and elevated serum transaminase levels. Her liver histology was consistent with GH. The pathophysiology and/or underlying genetic background of GH remains unclear. The optimum treatment for GH is optimal glycemic control, and the prognosis is favorable. Clinicians should be aware of the possibility of GH and observe the clinical response to optimal glycemic control prior to invasive investigation.

Abstract

Glycogenic hepatopathy (GH) is a rare complication of type 1 diabetes mellitus. We report the case of a 13-year-old diabetic female with poorly controlled blood sugar levels who presented with abdominal pain and distention 1 month in duration. She exhibited tender hepatomegaly, an elevated lipid profile, and elevated serum transaminase levels. Her liver histology was consistent with GH. The pathophysiology and/or underlying genetic background of GH remains unclear. The optimum treatment for GH is optimal glycemic control, and the prognosis is favorable. Clinicians should be aware of the possibility of GH and observe the clinical response to optimal glycemic control prior to invasive investigation.

참고문헌 (20)

  1. Sperling M, "Pediatric endocrinology", pp.376~ (2008), Elsevier Saunders
  2. Daneman D, "Type 1 diabetes", Lancet, 367, pp.847~858 (2006)
  3. El-Karaksy HM, "Prevalence of hepatic abnormalities in a cohort of Egyptian children with type 1 diabetes mellitus", Pediatr Diabetes, 11, pp.462~470 (2010)
  4. Al-Hussaini AA, "Prevalence of hepatopathy in type 1 diabetic children", BMC Pediatr, 12, pp.160~ (2012)
  5. 차재황, "Three cases of glycogenic hepatopathy mimicking acute and relapsing hepatitis in type I diabetes mellitus", Clinical and Molecular Hepatology, 19(4), pp.421~425 (2013), 대한간학회
  6. 정인혁, "1형 당뇨병 여아에서 간 글리코겐증에 의한 간 기능이상 1례", Annals of Pediatirc Endocrinology & Metabolism, 14(2), pp.174~178 (2009), 대한소아내분비학회
  7. 진혜영, "Hepatic glycogenosis in a patient with poorly controlled type 1 diabetes mellitus", Korean Journal of Pediatrics, 52(11), pp.1279~1282 (2009), 대한소아과학회
  8. 이승환, "혈당 조절이 불량한 제1형 당뇨병 환자에서 발현된 당원축적증 1예", Diabetes and Metabolism Journal, 30(1), pp.82~86 (2006), 대한당뇨병학회
  9. 박진아, "혈당 조절 후 호전된 당뇨병성 당원축적증에 의한 간비대 1예", Endocrinology and Metabolism, 19(2), pp.223~228 (2004), 대한내분비학회
  10. Mauriac P, "Gros ventre, hepatomegalie, troubles de las croissance chez les enfants diabetiques traits depuis plusieurs annes parl’insuline", Gaz Hebd Med Bordeaux, 26, pp.402~410 (1930)
  11. Abaci A, "Hepatic glycogenosis: a rare cause of hepatomegaly in Type 1 diabetes mellitus", J Diabetes Complications, 22, pp.325~328 (2008)
  12. Hudacko RM, "Clinical resolution of glycogenic hepatopathy following improved glycemic control", J Diabetes Complications, 22, pp.329~330 (2008)
  13. Munns CF, "Hepatic glycogenosis: reversible hepatomegaly in type 1 diabetes", J Paediatr Child Health, 36, pp.449~452 (2000)
  14. Roach PJ, "Glycogen and its metabolism", Curr Mol Med, 2, pp.101~120 (2002)
  15. Tomihira M, "Intermittent and recurrent hepatomegaly due to glycogen storage in a patient with type 1 diabetes: genetic analysis of the liver glycogen phosphorylase gene (PYGL)", Diabetes Res Clin Pract, 65, pp.175~182 (2004)
  16. Chatila R, "Hepatomegaly and abnormal liver tests due to glycogenosis in adults with diabetes", Medicine, 75, pp.327~333 (1996)
  17. Sheth SG, "Nonalcoholic steatohepatitis", Ann Intern Med, 126, pp.137~145 (1997)
  18. Sweetser S, "The bright liver of glycogenic hepatopathy", Hepatology, 51, pp.711~712 (2010)
  19. Murata F, "A case of glycogenic hepatopathy developed in a patient with new-onset fulminant type 1 diabetes: the role of image modalities in diagnosing hepatic glycogen deposition including gradient-dual-echo MRI", Endocr J, 59, pp.669~676 (2012)
  20. van den Brand M, "Glycogenic hepatopathy: a rare cause of elevated serum transaminases in diabetes mellitus", Neth J Med, 67, pp.394~396 (2009)

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