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1型糖尿病患者糖原性肝病改善但血糖控制仅有极小相应改善:病例报告与文献综述

Improvement of Glycogenic Hepatopathy With Minimal Corresponding Improvement of Glycemic Control in a Person With Type 1 Diabetes: Case Report and Literature Review.

作者信息

Tessa Tonleu Joselyn, Reyes Nicolas A, Hillerson Natalie D, Horton William B

机构信息

Division of Endocrinology and Metabolism, University of Virginia School of Medicine, Charlottesville, Virginia.

Department of Pathology, University of Virginia School of Medicine, Charlottesville, Virginia.

出版信息

AACE Clin Case Rep. 2024 Dec 17;11(2):113-116. doi: 10.1016/j.aace.2024.12.004. eCollection 2025 Mar-Apr.

Abstract

BACKGROUND/OBJECTIVE: Glycogenic hepatopathy is characterized by diffuse glycogen accumulation in hepatocytes that leads to hepatomegaly and elevated transaminases. Notably, the condition is reversible as improving glycemic control has been shown to resolve glycogenic hepatopathy and provide symptomatic relief.

CASE REPORT

A 30-year-old female with longstanding and poorly-controlled type 1 diabetes presented to her primary care physician for a routine follow-up visit. Routine lab work demonstrated hyperglycemia and elevated liver enzymes (alkaline phosphatase of 180 U/L, aspartate aminotransferase of 111 U/L, and alanine aminotransferase of 101 U/L). At laboratory reassessment 3 weeks later, liver function tests remained elevated and hepatic ultrasound was unrevealing. She was referred to gastroenterology for further evaluation and laboratory tests for viral and autoimmune hepatitis were negative while magnetic resonance imaging of the abdomen was unremarkable. Given the nondiagnostic work-up, liver biopsy was performed and pathology was consistent with glycogenic hepatopathy. She was referred to Endocrinology for improved glycemic control; however, liver enzymes normalized over the next several months despite minimal improvement in glycemic control. She was eventually transitioned to a closed-loop automated insulin delivery system and started dulaglutide for management of obesity. Subsequent A1c values significantly improved and liver enzymes remained within normal limits.

DISCUSSION

This case raises awareness of an under recognized complication of type 1 diabetes and challenges conventional thinking about factors leading to its resolution.

CONCLUSION

Further investigation into the underlying pathophysiology of glycogenic hepatopathy is needed.

摘要

背景/目的:糖原性肝病的特征是肝细胞内弥漫性糖原蓄积,可导致肝肿大和转氨酶升高。值得注意的是,这种情况是可逆的,因为改善血糖控制已被证明可缓解糖原性肝病并减轻症状。

病例报告

一名30岁的1型糖尿病女性患者,长期血糖控制不佳,前往其初级保健医生处进行常规随访。常规实验室检查显示血糖升高和肝酶升高(碱性磷酸酶为180 U/L,天冬氨酸转氨酶为111 U/L,丙氨酸转氨酶为101 U/L)。3周后进行实验室复查时,肝功能检查仍升高,肝脏超声检查未发现异常。她被转诊至胃肠病科进行进一步评估,病毒和自身免疫性肝炎的实验室检查均为阴性,腹部磁共振成像也无明显异常。鉴于检查结果未能明确诊断,遂进行了肝活检,病理结果与糖原性肝病相符。她被转诊至内分泌科以改善血糖控制;然而,尽管血糖控制改善甚微,但在接下来的几个月里肝酶恢复了正常。她最终改用闭环自动胰岛素输注系统,并开始使用度拉鲁肽治疗肥胖症。随后糖化血红蛋白值显著改善,肝酶仍保持在正常范围内。

讨论

该病例提高了对1型糖尿病一种未被充分认识的并发症的认识,并挑战了关于导致其缓解的因素的传统观念。

结论

需要对糖原性肝病的潜在病理生理学进行进一步研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2a2/11973649/a28778caceb2/gr1.jpg

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