Abstract
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. 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). 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. "Pure" steatosis was detected, unaccompanied by fibrosis or any other histological alteration. Microvesicular steatosis predominated, with a significant increase in mean mitochondrial surface area. 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.
Highlights
In children, the presence of steatosis is generally related to inborn errors of metabolism (IEMs)
Considerable variation was found in the degree of steatosis, as well as in the percentage of microvesicular steatosis compared with macrovesicular steatosis
The patients were allocated into two groups: the first group was composed of patients with predominantly (> 50%) microvesicular steatosis (n = 11); and the second group was composed of patients with predominantly (≤ 50%) macrovesicular steatosis (n = 7)
Summary
The presence of steatosis is generally related to inborn errors of metabolism (IEMs). Some forms of the disease present essentially as liver disease with hepatomegaly, jaundice, vomiting, lethargy and abnormalities of liver function When these diseases are ruled out after clinical and laboratory investigation, non-alcoholic fatty liver disease (NAFLD) should be considered. This term, which was initially used for adults, has become more inclusive.[1] It is currently used to describe all age groups, even children.[2,3] In more recent years, several studies on childhood[4] and adolescent hepatic steatosis in cases of NAFLD have been conducted, especially in relation to obese children,[5,6,7,8,9,10,11,12,13] since the prevalence of NAFLD is higher in these types of children. The major features are a higher rate in male children, with serum alanine aminotransferase (ALT) levels that are usually higher than serum aspartate aminotransferase (AST) levels, and with hypertriglyceridemia and vague abdominal pain (which is usually the main reason for seeking clinical evaluation).[14,15,16]
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More From: Sao Paulo medical journal = Revista paulista de medicina
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