Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy?
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 t...
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| Format: | Article |
| Language: | English |
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Associação Paulista de Medicina
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| Series: | São Paulo Medical Journal |
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| Online Access: | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802011000400004&lng=en&tlng=en |
| _version_ | 1811261165874970624 |
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| author | Gustavo Henrique Silva Gabriel Hessel Kunie Iabiku Rabello Coelho Cecília Amélia Fazzio Escanhoela |
| author_facet | Gustavo Henrique Silva Gabriel Hessel Kunie Iabiku Rabello Coelho Cecília Amélia Fazzio Escanhoela |
| author_sort | Gustavo Henrique Silva |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-12T18:59:26Z |
| format | Article |
| id | doaj.art-2bbbaba4dc22487d954b564e5cb86fef |
| institution | Directory Open Access Journal |
| issn | 1806-9460 |
| language | English |
| last_indexed | 2024-04-12T18:59:26Z |
| publisher | Associação Paulista de Medicina |
| record_format | Article |
| series | São Paulo Medical Journal |
| spelling | doaj.art-2bbbaba4dc22487d954b564e5cb86fef2022-12-22T03:20:13ZengAssociação Paulista de MedicinaSão Paulo Medical Journal1806-9460129421722310.1590/S1516-31802011000400004S1516-31802011000400004Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy?Gustavo Henrique SilvaGabriel Hessel0Kunie Iabiku Rabello Coelho1Cecília Amélia Fazzio Escanhoela2Universidade Estadual de CampinasUniversidade Estadual PaulistaUniversidade Estadual de CampinasCONTEXT 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802011000400004&lng=en&tlng=enFatty liverMitochondria, liverAnatomyInfant, newbornMetabolism, inborn errors |
| spellingShingle | Gustavo Henrique Silva Gabriel Hessel Kunie Iabiku Rabello Coelho Cecília Amélia Fazzio Escanhoela Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy? São Paulo Medical Journal Fatty liver Mitochondria, liver Anatomy Infant, newborn Metabolism, inborn errors |
| title | Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy? |
| title_full | Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy? |
| title_fullStr | Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy? |
| title_full_unstemmed | Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy? |
| title_short | Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy? |
| title_sort | steatosis of indeterminate cause in a pediatric group is it a primary mitochondrial hepatopathy |
| topic | Fatty liver Mitochondria, liver Anatomy Infant, newborn Metabolism, inborn errors |
| url | http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31802011000400004&lng=en&tlng=en |
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