Allende Daniela S, Gawrieh Samer, Cummings Oscar W, Belt Patricia, Wilson Laura, Van Natta Mark, Behling Cynthia A, Carpenter Danielle, Gill Ryan M, Kleiner David E, Yeh Mathew M, Chalasani Naga, Guy Cynthia D
Department of Pathology, Cleveland Clinic, Cleveland, OH, USA.
Department of Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
Liver Int. 2021 May;41(5):996-1011. doi: 10.1111/liv.14773. Epub 2021 Jan 7.
BACKGROUND/AIMS: Glycogen synthesis and storage are normal hepatocyte functions. However, glycogenosis, defined as excess hepatocyte glycogen visible by routine H&E light microscopy, has not been well characterized in nonalcoholic fatty liver disease (NAFLD).
Glycogenosis in NAFLD liver biopsies was graded as "none", "focal" (in <50% of hepatocytes), or "diffuse" (in ≥50% of hepatocytes). Clinical and pathological variables associated with glycogenosis were assessed. 2047 liver biopsies were prospectively analysed.
In adults and children, any glycogenosis was present in 54% of cases; diffuse glycogenosis was noted in approximately 1/3 of cases. On multiple logistic regression analysis, adults with glycogenosis tended to be older (P = .003), female (P = .04), have higher serum glucose (P = .01), and use insulin (P = .02). Adults tended to have lower steatosis scores (P = .006) and lower fibrosis stages (P = .005); however, unexpectedly, they also tended to have more hepatocyte injury including ballooning (P = .003). On multiple logistic regression analysis, paediatric patients with glycogenosis were more likely to be Hispanic (P = .03), have lower body weight (P = .002), elevated triglycerides (P = .001), and a higher fasting glucose (P = .007). Paediatric patients with glycogenosis also had less steatosis (P < .001) than those without.
Glycogenosis is common in adult and paediatric NAFLD, and is associated with clinical features of insulin resistance. Glycogenosis is important to recognize histologically because it may be misinterpreted as ballooning, and when diffuse, confusion with glycogen storage disorders or glycogenic hepatopathy must be avoided. The newly observed dichotomous relationship between glycogenosis and increased liver cell injury but decreased steatosis and fibrosis requires further study.
背景/目的：糖原合成与储存是正常肝细胞的功能。然而，糖原贮积病（定义为通过常规苏木精-伊红染色光镜可见的肝细胞糖原过多）在非酒精性脂肪性肝病（NAFLD）中尚未得到充分表征。
将NAFLD肝活检中的糖原贮积病分为“无”、“局灶性”（<50%的肝细胞中出现）或“弥漫性”（≥50%的肝细胞中出现）。评估与糖原贮积病相关的临床和病理变量。对2047例肝活检进行前瞻性分析。
在成人和儿童中，54%的病例存在任何糖原贮积病；约1/3的病例有弥漫性糖原贮积病。多因素逻辑回归分析显示，患有糖原贮积病的成人往往年龄较大（P = 0.003）、为女性（P = 0.04）、血糖较高（P = 0.01）且使用胰岛素（P = 0.02）。成人的脂肪变性评分往往较低（P = 0.006），纤维化分期也较低（P = 0.005）；然而，出乎意料的是，他们的肝细胞损伤（包括气球样变）也往往更多（P = 0.003）。多因素逻辑回归分析显示，患有糖原贮积病的儿科患者更可能是西班牙裔（P = 0.03）、体重较低（P = 0.002）、甘油三酯升高（P = 0.001）且空腹血糖较高（P = 0.）。患有糖原贮积病的儿科患者的脂肪变性也比未患该病的患者少（P < 0.001）。
糖原贮积病在成人和儿童NAFLD中很常见，且与胰岛素抵抗的临床特征相关。糖原贮积病在组织学上很重要，因为它可能被误诊为气球样变，当为弥漫性时，必须避免与糖原贮积症或糖原性肝病混淆。新观察到的糖原贮积病与肝细胞损伤增加但脂肪变性和纤维化减少之间的二分关系需要进一步研究。
