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首页> 外文期刊>Sao Paulo Medical Journal >Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy?
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Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy?

机译：小儿原因不明的脂肪变性：是原发性线粒体肝病吗？

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CONTEXT AND OBJECTIVE: In children, hepatic steatosis may be related to inborn errors of metabolism (IEMs) or to non-alcoholic fatty liver disease (NAFLD). The aim of this study was to assess and characterize steatosis of indeterminate cause through morphological and morphometric analysis of liver tissue. DESIGN AND SETTING: Cross-sectional study at the Departments of Pathology of Faculdade de Ciências Médicas, Universidade Estadual de Campinas (FCM-Unicamp) and Faculdade de Medicina de Botucatu, Universidade Estadual Paulista (FMB-Unesp). METHODS: Eighteen consecutive liver biopsies obtained from 16 patients of ages ranging from 3 months to 12 years and nine months that were inserted in a database in the study period were analyzed using optical microscopy and transmission electron microscopy. Through electron microscopy, the mitochondrial density and mean mitochondrial surface area were determined in hepatocytes. Ten patients ranging in age from 1 to 14 years were used as a control group. RESULTS: "Pure" steatosis was detected, unaccompanied by fibrosis or any other histological alteration. Microvesicular steatosis predominated, with a significant increase in mean mitochondrial surface area. CONCLUSION: Microvesicular steatosis may be related to primary mitochondrial hepatopathy, especially due to reduction of β-oxidation or partial stagnation of oxidative phosphorylation. For these reasons, this form of steatosis (which should not be called "pure") is likely to represent an initial stage in the broad spectrum of NAFLD. We have drawn attention to cases of steatosis in the pediatric group, in which the microvesicular form predominates, since this may be associated with mitochondrial disorders.
机译：背景与目的：在儿童中，肝脂肪变性可能与先天性代谢错误（IEM）或非酒精性脂肪肝疾病（NAFLD）有关。这项研究的目的是通过肝组织的形态学和形态计量学分析评估和鉴定不确定原因的脂肪变性。设计与地点：坎帕纳斯国立大学（FCM-Unicamp）的Faculdade deCiênciasMédicas病理学系和Paulista大学（FMB-Unesp）的Boculatu Medicina de Botucatu医院的横断面研究。方法：使用光学显微镜和透射电子显微镜对在研究期间插入数据库的16例3个月至12岁零9个月的患者进行的18例连续肝活检进行分析。通过电子显微镜，测定肝细胞中的线粒体密度和平均线粒体表面积。十名年龄在1至14岁之间的患者被用作对照组。结果：检测到“纯”脂肪变性，未伴有纤维化或任何其他组织学改变。微囊性脂肪变性为主，平均线粒体表面积显着增加。结论：微泡脂肪变性可能与原发性线粒体肝病有关，尤其是由于β-氧化减少或氧化磷酸化的部分停滞。由于这些原因，这种形式的脂肪变性（不应称为“纯”形式）很可能代表了NAFLD广泛领域的初始阶段。我们引起了对儿童的脂肪变性病例的关注，其中以微泡形式为主，因为这可能与线粒体疾病有关。

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