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Glycogenic Hepatopathy: Thinking Outside the Box

机译:糖原性肝病:跳出框框思考

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Glycogenic hepatopathy (GH) remains underrecognized in adults as most clinicians mistake it for the more common hepatic abnormality associated with uncontrolled diabetes mellitus in this age group, non-alcoholic fatty liver disease. This is also complicated by the fact that both entities are indistinguishable on liver ultrasound. We herein describe a similar predicament in which a young adult female presented with bilateral upper quadrant abdominal pain, tender hepatomegaly, lactic acidosis and a >10-fold increase in liver enzymes, which worsened after the administration of high-dose steroids. Despite intravenous normal saline resuscitation, serum transaminitis persisted in a fluctuating manner. Ultimately, a liver biopsy confirmed GH. Biochemically, GH is driven by high amounts of both circulating glucose and insulin or by the administration of high-dose steroids. Improving glycemic control is the mainstay of treatment for GH. However, in our case, improvement in glycated hemoglobin of just 0.6% was enough to achieve symptomatic relief, supporting recent claims of the involvement of other identified factors in disease development.
机译:糖原性肝病(GH)在成人中仍未得到充分认识,因为大多数临床医生将其误认为是该年龄组非酒精性脂肪肝疾病中与不受控制的糖尿病相关的更常见的肝脏异常。这两个事实在肝脏超声检查中无法区分,这也使情况变得复杂。我们在本文中描述了类似的困境,其中年轻成年女性表现出双侧上腹腹痛,肝肿大,乳酸性酸中毒和肝酶增加> 10倍,在服用大剂量类固醇后恶化。尽管进行了静脉生理盐水复苏,但血清转氨性疾病仍以波动的方式持续存在。最终,肝活检证实为GH。生化方面,GH由大量循环葡萄糖和胰岛素驱动,或由高剂量类固醇驱动。改善血糖控制是GH治疗的主要手段。然而,在我们的案例中,糖化血红蛋白仅改善0.6%就足以实现症状缓解,这支持了最近有关其他已确定因素参与疾病发展的主张。

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