Digestive Center for Diagnosis and Treatment, Damascus, Syrian Arab Republic.
Division of Gastroenterology, Hepatology, and Nutrition, Vanderbilt University, Nashville, TN, USA.
Liver Int. 2021 Jul;41(7):1545-1555. doi: 10.1111/liv.14827. Epub 2021 Feb 28.
BACKGROUND & AIMS: Glycogenic hepatopathy (GH) in type 1 diabetes-mellitus (T1DM) is characterized by hepatomegaly and perturbations of liver chemistries (LC) that have not been well studied. Furthermore, misdiagnosis with other hepatic complications of T1DM, such as nonalcoholic fatty liver disease, has been described. We perform a systematic review of biopsy-proven GH reports in T1DM patients to identify LC patterns.
A systematic review identified reports of biopsy-proven GH in patients with T1DM. We excluded GH with other liver diseases, Mauriac syndrome, or GH without T1DM. Two reviewers screened and extracted studies and assessed their methodological quality. LC elevation magnitude, AST-to-ALT ratio, R-ratio to designate hepatocellular, cholestatic or mixed pattern of hepatic injury, and evolution of transaminases after glycemic control were analyzed.
A total of 192 patients were included, with median age of 20 years, 73% adults, 66% females, median duration of T1DM before diagnosis 10 years, median adult body mass index 21 kg/m , median HbA1c 12%, at least one episode of diabetic ketoacidosis 70%, and hepatomegaly 92%. ALT and AST showed moderate-to-severe elevation in 78% and 76%, respectively, AST/ALT >1 in 71% and hepatocellular to mixed pattern of hepatic injury in 81%. Transaminase improvement with glycemic control was the rule, regardless of other factors in multilinear regression analysis.
GH tends to have AST-predominant elevation with a median of 13 times the upper normal limit and R-ratio >2, which may distinguish it from other etiologies of AST-predominant LC elevation, and in the appropriate clinical context, may obviate invasive tests.
1 型糖尿病（T1DM）患者的糖原贮积性肝病（GH）的特征为肝肿大和肝脏化学指标（LC）的改变，但这些改变尚未得到充分研究。此外，曾有 T1DM 其他肝脏并发症（如非酒精性脂肪性肝病）的误诊描述。我们对 T1DM 患者活检证实的 GH 报告进行了系统回顾，以确定 LC 模式。
系统回顾确定了 T1DM 患者活检证实的 GH 报告。我们排除了 GH 合并其他肝脏疾病、Mauriác 综合征或 GH 不伴 T1DM 的报告。两名审阅者筛选和提取研究并评估其方法学质量。分析 LC 升高幅度、AST/ALT 比值、R-比值以指定肝细胞性、胆汁淤积性或混合性肝损伤模式，以及血糖控制后转氨酶的变化。
共纳入 192 例患者，中位年龄 20 岁，73%为成年人，66%为女性，诊断前 T1DM 中位病程 10 年，成人中位体重指数 21kg/m²，中位糖化血红蛋白 12%，至少有一次糖尿病酮症酸中毒发作 70%，肝肿大 92%。ALT 和 AST 分别有 78%和 76%表现为中重度升高，AST/ALT＞1 的比例为 71%，肝细胞性至混合性肝损伤模式的比例为 81%。血糖控制后，无论多线性回归分析中的其他因素如何，转氨酶的改善都是普遍规律。
GH 倾向于 AST 为主的升高，中位数为正常上限的 13 倍，R-比值＞2，这可能使其与其他以 AST 为主的 LC 升高的病因不同，在适当的临床情况下，可能无需进行侵入性检查。
