Cox Brian K, Guindi Maha, Hutchings Danielle, Kim Stacey A, Waters Kevin M, Larson Brent K
Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Los Angeles, CA 90048, United States of America.
Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, 8700 Beverly Blvd., Los Angeles, CA 90048, United States of America.
Ann Diagn Pathol. 2023 Jun;64:152130. doi: 10.1016/j.anndiagpath.2023.152130. Epub 2023 Mar 7.
This study examines the clinical-pathological profiles of patients with glycogenic hepatopathy in a contemporary cohort of patients at an adult acute care hospital.
Liver biopsies with glycogenic hepatopathy were retrieved from the departmental surgical pathology database, the histological findings were studied, and the clinical findings were reviewed.
Five cases of glycogenic hepatopathy were found, including cases associated with type 1 diabetes mellitus (n = 1), type 2 diabetes mellitus (n = 1), corticosteroids (n = 2), and anorexia (n = 2, including the patient with type 1 diabetes). AST and ALT were normal to mildly elevated (13-115 U/L and 7-126 U/L, respectively). Trace ascites was present in two patients. Hepatomegaly was only present in the patient with type 1 diabetes at the time of diagnosis.
Four of five cases were associated with etiologies other than type 1 diabetes, which is widely reported as the most common etiology of glycogenic hepatopathy. This study suggests that etiologies currently only rarely recognized may actually be more common causes of glycogenic hepatopathy than type 1 diabetes in a contemporary adult population. It is important not only to recognize that these rarely reported causes of glycogenic hepatopathy may be underrecognized, but that the clinical presentation may also be mild.
本研究在一家成人急症医院的当代患者队列中，考察糖原性肝病患者的临床病理特征。
从科室手术病理数据库中检索出有糖原性肝病的肝活检样本，研究组织学结果，并回顾临床结果。
发现5例糖原性肝病病例，包括与1型糖尿病相关的病例（n = 1）、2型糖尿病相关的病例（n = 1）、皮质类固醇相关的病例（n = 2）以及厌食相关的病例（n = 2，包括1型糖尿病患者）。谷草转氨酶（AST）和谷丙转氨酶（ALT）正常至轻度升高（分别为13 - 115 U/L和7 - 126 U/L）。两名患者有微量腹水。仅1型糖尿病患者在诊断时有肝肿大。
五例中有四例与1型糖尿病以外的病因相关，而1型糖尿病被广泛报道为糖原性肝病最常见的病因。本研究表明，在当代成年人群中，目前仅很少被认识到的病因实际上可能比1型糖尿病更常见地导致糖原性肝病。不仅要认识到这些很少被报道的糖原性肝病病因可能未得到充分认识，而且其临床表现可能也很轻微，这一点很重要。
