Research Article Details

Article ID: A09033
PMID: 31897838
Source: Eur J Pediatr
Title: Causes of secondary non-alcoholic fatty liver disease in non-obese children below 10 years.
Abstract: This study aimed to detect etiologies and histopathological features of non-alcoholic fatty liver disease (NAFLD) in Egyptian children <&#8201;10&#160;years from hepatologist perspectives. Infants and children below 10&#160;years of age with biopsy-proven fatty liver over a 6-year period were included. NAFLD activity score was used to detect the presence of non-alcoholic steatohepatitis (NASH). The study included 66 cases whose age ranged between 5&#160;months and 10&#160;years. Transaminases were elevated in 60% patients. Glycogen storage disease (GSD) was the most common diagnosis (33.3%) followed by hepatitis C virus (HCV) (10.6%) and Chanarin-Dorfman syndrome (CDS) (9.1%). The cause of steatosis could not be identified in 28.8% of cases. There was a higher prevalence of secondary causes of NAFLD in patients <&#8201;10&#160;years. Liver histopathological examination revealed preserved lobular architecture in 75.7% with minimal-to-mild fibrosis in 79%. Steatosis was macrovesicular in all specimens (severe steatosis in 39.4%). Four patients had NASH. Higher degree of steatosis was associated with more severe fibrosis (P&#8201;=&#8201;0.01).Conclusion: GSD was the commonest cause of secondary NAFLD in Egyptian children <&#8201;10&#160;years followed by HCV and CDS with higher degrees of steatosis in younger patients. The degree of fibrosis was significantly related to the degree of steatosis.What is Known:&#8226; Primary non-alcoholic fatty liver disease (NAFLD) is rare in children aged less than 10&#160;years.&#8226; Secondary causes of NAFLD should be considered in patients who do not have traditional risk factors.What is New:&#8226; Glycogen storage disease, hepatitis C virus, and Chanarin-Dorfman syndrome are the commonest causes of secondary NAFLD in Egyptian children (<&#8201;10&#160;years) with higher degrees of steatosis in younger patients.&#8226; The degree of liver fibrosis is significantly related to the degree of steatosis.
DOI: 10.1007/s00431-019-03551-0