Lui David Tw, Woo Yu-Cho, Chow Wing-Sun, Lee Chi-Ho, Lee Alan Ch, Leung Eunice Kh, Tan Kathryn Cb, Lam Karen Sl, Lam Joanne Ky
Department of Medicine, University of Hong Kong, Queen Mary Hospital, Hong Kong.
Medicine (Baltimore). 2019 Apr;98(17):e15296. doi: 10.1097/MD.0000000000015296.
Deranged liver function is a common finding among patients with diabetes mellitus. We report a case of liver biopsy-proven glycogenic hepatopathy (GH) in a patient with long-standing poorly controlled type 1 diabetes (DM1), presented with recurrent transaminitis.
A 28-year-old Chinese woman was noted to have deranged liver function with transaminases elevated to more than 15 times the upper limit of normal.
She had underlying long-standing poorly controlled DM1. Blood tests including hepatitis serology and autoimmune panel were negative. Liver biopsy confirmed the diagnosis of GH, showing an increase in glycogen deposition with intact liver parenchymal architecture, and no inflammation or significant fibrosis.
Her glycemic control was optimized.
Her transaminase levels normalized upon subsequent follow-up with improved glycemic control.
GH is suspected when transaminase flare occurs in patients with poorly controlled DM1, usually with exaggerated hemoglobin A1c levels, especially after drug-induced, viral, autoimmune and metabolic liver diseases are excluded. The gold standard of diagnosis is liver biopsy. When diagnosis of GH is ascertained, the mainstay of treatment is to optimize glycemic control. Typically, the transaminases may become normal within days to months after improvement of glycemic control. Compared to non-alcoholic fatty liver disease, GH is associated with favorable prognosis and runs a benign course, making this differentiation clinically important.
肝功能紊乱在糖尿病患者中很常见。我们报告一例经肝活检证实为糖原性肝病（GH）的病例，该患者为长期血糖控制不佳的1型糖尿病（DM1）患者，表现为反复转氨酶升高。
一名28岁中国女性被发现肝功能紊乱，转氨酶升高至正常上限的15倍以上。
她患有潜在的长期血糖控制不佳的DM1。包括肝炎血清学和自身免疫指标在内的血液检查均为阴性。肝活检确诊为GH，显示糖原沉积增加，肝实质结构完整，无炎症或明显纤维化。
优化了她的血糖控制。
在随后的随访中，随着血糖控制改善，她的转氨酶水平恢复正常。
当血糖控制不佳的DM1患者出现转氨酶升高时，尤其是糖化血红蛋白水平过高，且排除药物性、病毒性、自身免疫性和代谢性肝病后，应怀疑GH。诊断的金标准是肝活检。确诊GH后，主要治疗方法是优化血糖控制。通常，血糖控制改善后数天至数月内转氨酶可能恢复正常。与非酒精性脂肪性肝病相比，GH预后良好，病程良性，因此这种鉴别在临床上很重要。
