Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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Distinctive Features of Hepatic Steatosis in Children: Is It Primary or Secondary to Inborn Errors of Metabolism?

  • Karhan, Asuman Nur (Department of Pediatrics, Division of Gastroenterology, Mersin University Faculty of Medicine) ;
  • Hizarcioglu-Gulsen, Hayriye (Department of Pediatrics, Division of Gastroenterology, Hacettepe University Faculty of Medicine) ;
  • Gumus, Ersin (Department of Pediatrics, Division of Gastroenterology, Hacettepe University Faculty of Medicine) ;
  • Akcoren, Zuhal (Department of Pediatric Pathology, Hacettepe University Faculty of Medicine) ;
  • Demir, Hulya (Department of Pediatrics, Division of Gastroenterology, Hacettepe University Faculty of Medicine) ;
  • Saltik-Temizel, Inci Nur (Department of Pediatrics, Division of Gastroenterology, Hacettepe University Faculty of Medicine) ;
  • Orhan, Diclehan (Department of Pediatric Pathology, Hacettepe University Faculty of Medicine) ;
  • Ozen, Hasan (Department of Pediatrics, Division of Gastroenterology, Hacettepe University Faculty of Medicine)
  • Received : 2021.05.01
  • Accepted : 2021.08.29
  • Published : 2021.11.15
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Abstract

Purpose: The incidence of hepatic steatosis among children has been increasing; however, data distinguishing simple steatosis from a more complex disorder are lacking. Methods: This study identified the etiologies resulting in hepatic steatosis through a retrospective review of pediatric liver biopsies performed in the last 10 years. A total of 158 patients with hepatic steatosis proven by histopathological evaluation were enrolled in the study, and baseline demographic features, anthropometric measurements, physical examination findings, laboratory data, ultrasonographic findings, and liver histopathologies were noted. Results: The two most common diagnoses were inborn errors of metabolism (IEM) (52.5%) and nonalcoholic fatty liver disease/steatohepatitis (NAFLD/NASH) (29.7%). The three most common diseases in the IEM group were glycogen storage disorders, Wilson's disease, and mitochondrial disease. The rates of consanguineous marriage (75.6%; odds ratio [OR], 26.040) and positive family history (26.5%; OR, 8.115) were significantly higher (p=0.002, p<0.001, respectively) in the IEM group than those in the NAFLD/NASH group. Younger age (p=0.001), normal anthropometric measurements (p=0.03), increased aspartate aminotransferase levels (p<0.001), triglyceride levels (p=0.001), and cholestatic biochemical parameters with disrupted liver function tests, as well as severe liver destruction of hepatic architecture, cholestasis, fibrosis, and nodule formation, were also common in the IEM group. Conclusion: Parents with consanguinity and positive family history, together with clinical and biochemical findings, may provide a high index of suspicion for IEM to distinguish primary steatosis from the consequence of a more complex disorder.

Keywords

References

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