Jeong Hwal Rim, Shim Young Seok, Kim Young Bae, Lee Hae Sang, Hwang Jin Soon
Department of Pediatrics, Ajou University School of Medicine, Suwon, Korea.
Department of Pathology, Ajou University School of Medicine, Suwon, Korea.
Ann Pediatr Endocrinol Metab. 2014 Mar;19(1):49-52. doi: 10.6065/apem.2014.19.1.49. Epub 2014 Mar 31.
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.
糖原性肝病（GH）是1型糖尿病的一种罕见并发症。我们报告了一例13岁的糖尿病女性患者，其血糖控制不佳，出现持续1个月的腹痛和腹胀。她表现出肝脏压痛、肝肿大、血脂升高和血清转氨酶水平升高。她的肝脏组织学检查结果符合糖原性肝病。糖原性肝病的病理生理学和/或潜在遗传背景仍不清楚。糖原性肝病的最佳治疗方法是优化血糖控制，预后良好。临床医生应意识到糖原性肝病的可能性，并在进行侵入性检查之前观察优化血糖控制后的临床反应。
