Glycogenosis is common in nonalcoholic fatty liver disease and is independently associated with ballooning, but lower steatosis and lower fibrosis.
Glycogenosis is common in nonalcoholic fatty liver disease and is independently associated with ballooning, but lower steatosis and lower fibrosis.
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DOI：
10.1111/liv.14773
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发表时间：
2021-05
期刊：
影响因子：
--
通讯作者：
NASH Clinical Research Network
中科院分区：
文献类型：
--
作者：
Allende DS;Gawrieh S;Cummings OW;Belt P;Wilson L;Van Natta M;Behling CA;Carpenter D;Gill RM;Kleiner DE;Yeh MM;Chalasani N;Guy CD;NASH Clinical Research Network
糖原的合成和储存是肝细胞的正常功能。然而，糖原沉积症在非酒精性脂肪性肝病(NAFLD)中尚未得到很好的描述。非酒精性脂肪肝肝组织中的血糖生成分为“无”、“局灶性”(占≥肝细胞的50%)或“弥漫性”(占肝细胞的50%)。评估与糖原沉积症相关的临床和病理变量。对2047例肝活检进行了前瞻性分析。在成人和儿童中，54%的病例存在任何糖原增多症；约三分之一的病例存在弥漫性糖原增多症。多因素Logistic回归分析显示，糖原增多症患者年龄偏大(P=0.03)，女性偏高(P=5.04)，血糖偏高(P=5.01)，胰岛素使用率较高(P=5.02)。成年人往往有较低的脂肪变性评分(P=0.006)和较低的纤维化阶段(P=4.005)；然而，出乎意料的是，他们也倾向于有更多的肝细胞损伤，包括气球膨胀(P=9.003)。多因素Logistic回归分析显示，糖原增多症患儿多为西班牙裔(P=0.03)，体重较轻(P=0.002)，甘油三酯升高(P=0.001)，空腹血糖较高(P=0.007)。有糖原沉积症的儿科患者的脂肪变性也比无糖原沉积症的患者少(P<；2.001)。血糖生成症在成人和儿童NAFLD中很常见，并与胰岛素抵抗的临床特征有关。糖原沉积症在组织学上的识别很重要，因为它可能被误解为气球状，当弥漫性时，必须避免与糖原储存障碍或糖源性肝病混淆。新观察到的糖原增多和肝细胞损伤增加，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。
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.
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