Hautekeete M L, Degott C, Benhamou J P
Service d'Hépatologie, Hôpital Beaujon, Clichy, France.
Acta Clin Belg. 1990;45(5):311-26. doi: 10.1080/17843286.1990.11718105.
The term "microvesicular steatosis of the liver" refers to a variant form of hepatic fat accumulation whose histologic features contrast with the much more common macrovesicular steatosis. Microvesicular steatosis of the liver was originally described in association with conditions who share a number of biochemical and a limited number of clinical features: acute fatty liver of pregnancy, Reye's syndrome, Jamaican vomiting sickness, sodium valproate toxicity, high-dose tetracycline toxicity and certain congenital defects of urea cycle enzymes; they were thought to constitute an entity of "microvesicular fat diseases". In recent years the disease has been described in a wide variety of conditions: alcoholism, toxicity of several medications, delta hepatitis in South America and Central Africa, sudden childhood death, congenital defects of fatty acid beta oxidation, cholesterol ester storage disease, Wolman disease and Alpers syndrome. Not much is known regarding the pathogenesis of microvesicular steatosis but in many instances the primary defect could be a mitochondrial lesion, and inhibition of the mitochondrial beta oxidation of fatty acids has been the most frequently implicated defect. The different conditions associated with microvesicular steatosis are heterogenous in many aspects. Maintaining the concept of "microvesicular fat diseases" as a unique entity seems no longer justified.
“肝脏微泡性脂肪变性”一词指的是肝脏脂肪蓄积的一种变异形式，其组织学特征与更为常见的大泡性脂肪变性形成对比。肝脏微泡性脂肪变性最初是在与一些具有若干生化特征和少数临床特征的病症相关联的情况下被描述的：妊娠急性脂肪肝、瑞氏综合征、牙买加呕吐病、丙戊酸钠毒性、大剂量四环素毒性以及尿素循环酶的某些先天性缺陷；它们被认为构成了“微泡性脂肪疾病”这一实体。近年来，在多种病症中都描述了这种疾病：酒精中毒、多种药物的毒性、南美洲和中非的丁型肝炎、儿童猝死、脂肪酸β氧化的先天性缺陷、胆固醇酯贮积病、沃尔曼病和阿尔珀斯综合征。关于微泡性脂肪变性的发病机制所知甚少，但在许多情况下，主要缺陷可能是线粒体病变，而脂肪酸线粒体β氧化的抑制是最常涉及的缺陷。与微泡性脂肪变性相关的不同病症在许多方面是异质性的。将“微泡性脂肪疾病”概念维持为一个独特实体似乎不再合理。
