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伪装成急性胰腺炎的糖原性肝病。

Glycogenic Hepatopathy Masquerading as Acute Pancreatitis.

作者信息

Adams Steven H, Bernshteyn Michelle, Masood Umair, Corines James, Manocha Divey

机构信息

Pathology, State University of New York Upstate Medical University, Syracuse, USA.

Internal Medicine, State University of New York Upstate Medical University, Syracuse, USA.

出版信息

Cureus. 2021 Feb 17;13(2):e13397. doi: 10.7759/cureus.13397.

DOI:10.7759/cureus.13397
PMID:33758698
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7978395/
Abstract

Glycogenic hepatopathy (GH), defined histologically by hepatocytic glycogen accumulation without fatty change or fibrosis, is a benign reversible condition. It presents clinically as hepatomegaly with elevated liver enzymes in young diabetic (type 1) patients with poor glycemic control. We report a case of a 20-year-old female with a history of poorly controlled type 1 diabetes mellitus (T1DM) and prior pancreatitis who presented with sharp epigastric pain and hepatomegaly. She was found to have diabetic ketoacidosis with elevated lipase and amylase. Though at first her symptoms were erroneously attributed to pancreatitis, a liver biopsy showing glycogenated nuclei led to a diagnosis of GH.

摘要

糖原性肝病(GH)在组织学上定义为肝细胞糖原蓄积,无脂肪变性或纤维化,是一种良性可逆性疾病。在血糖控制不佳的年轻糖尿病(1型)患者中,临床上表现为肝肿大伴肝酶升高。我们报告一例20岁女性,有1型糖尿病(T1DM)控制不佳病史及既往胰腺炎病史,出现上腹部剧痛和肝肿大。发现她患有糖尿病酮症酸中毒,脂肪酶和淀粉酶升高。起初她的症状被错误地归因于胰腺炎,但肝脏活检显示核内糖原沉积,从而诊断为GH。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e5a/7978395/0f98e39004ae/cureus-0013-00000013397-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e5a/7978395/63fb9f4473f7/cureus-0013-00000013397-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e5a/7978395/0f98e39004ae/cureus-0013-00000013397-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e5a/7978395/63fb9f4473f7/cureus-0013-00000013397-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5e5a/7978395/0f98e39004ae/cureus-0013-00000013397-i02.jpg

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Type 1 Diabetes Mellitus with Diabetic Ketoacidosis Presenting with Transient Severe Fatty Liver and Subsequent Liver Dysfunction.1型糖尿病合并糖尿病酮症酸中毒伴发短暂性严重脂肪肝及随后的肝功能障碍。
Intern Med. 2024 Dec 15;63(24):3339-3344. doi: 10.2169/internalmedicine.3488-24. Epub 2024 May 9.
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Hepatopathy Associated With Type 1 Diabetes: Distinguishing Non-alcoholic Fatty Liver Disease From Glycogenic Hepatopathy.1型糖尿病相关肝病:非酒精性脂肪性肝病与糖原性肝病的鉴别
Front Pharmacol. 2021 Oct 25;12:768576. doi: 10.3389/fphar.2021.768576. eCollection 2021.

本文引用的文献

1
Glycogenic hepatopathy: A narrative review.糖原性肝病:一篇叙述性综述。
World J Hepatol. 2018 Feb 27;10(2):172-185. doi: 10.4254/wjh.v10.i2.172.
2
Clinical, Biochemical, and Histopathology Features of Patients With Glycogenic Hepatopathy.糖原贮积性肝病患者的临床、生化和组织病理学特征。
Clin Gastroenterol Hepatol. 2017 Jun;15(6):927-933. doi: 10.1016/j.cgh.2016.11.038. Epub 2016 Dec 30.
3
Glycogenic hepatopathy, an underdiagnosed cause of relapsing hepatitis in uncontrolled type 1 diabetes mellitus.糖原性肝病,1型糖尿病控制不佳时复发性肝炎的一个未被充分诊断的病因。
Saudi Med J. 2017 Jan;38(1):89-92. doi: 10.15537/smj.2017.1.15934.
4
Glycogenic hepatopathy in young adults: a case series.年轻成年人的糖原性肝病:病例系列
Rev Esp Enferm Dig. 2016 Oct;108(10):673-676. doi: 10.17235/reed.2016.3934/2015.
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The bright liver of glycogenic hepatopathy.糖原累积性肝病的明亮肝脏。
Hepatology. 2010 Feb;51(2):711-2. doi: 10.1002/hep.23402.
6
Glycogenic hepatopathy: an underrecognized hepatic complication of diabetes mellitus.糖原性肝病:一种未被充分认识的糖尿病肝脏并发症。
Am J Surg Pathol. 2006 Apr;30(4):508-13. doi: 10.1097/00000478-200604000-00012.