Distinctive Features of Hepatic Steatosis in Children: Is It Primary or Secondary to Inborn Errors of Metabolism?

Asuman Nur Karhan; Hayriye Hizarcioglu-Gulsen; Ersin Gumus; Zuhal Akçören; Hülya Demir; İnci Nur Saltik-Temizel; Diclehan Orhan; Hasan Özen

doi:10.5223/pghn.2021.24.6.518

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저자정보: Asuman Nur Karhan (Mersin University Faculty of Medicine) Hayriye Hizarcioglu-Gulsen (Hacettepe University Faculty of Medicine) Ersin Gumus (Hacettepe University Faculty of Medicine) Zuhal Akçören (Hacettepe University Faculty of Medicine) Hülya Demir (Hacettepe University Faculty of Medicine) İnci Nur Saltik-Temizel (Hacettepe University Faculty of Medicine) Diclehan Orhan (Hacettepe University Faculty of Medicine) Hasan Özen (Hacettepe University Faculty of Medicine)

저널정보: 대한소아소화기영양학회 Pediatric Gastroenterology, Hepatology & Nutrition Pediatric Gastroenterology, Hepatology & Nutrition 제24권 제6호

발행연도: 2021.11

수록면: 518 - 527 (10page)

DOI: 10.5223/pghn.2021.24.6.518

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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.

#Hepatic steatosis #Metabolism #Inborn errors #Nonalcoholic hepatic steatosis #Pediatric

참고문헌 (29)

참고문헌 신청

Hourigan SK / 2015 / The full spectrum of hepatic steatosis in children / Clin Pediatr(Phila) 54:635~642

Silva GH / 2011 / Steatosis of indeterminate cause in a pediatric group: is it a primary mitochondrial hepatopathy? / Sao Paulo Med J 129:217~223

Alfani R / 2018 / Pediatric fatty liver and obesity : not always justa matter of non-alcoholic fatty liver disease / Children(Basel) 5:169

Vos MB / 2017 / NASPGHAN clinical practice guideline for the diagnosis and treatment of nonalcoholic fatty liver disease in children: recommendations from the Expert Committee on NAFLD (ECON) and the North American Society of Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) / J Pediatr Gastroenterol Nutr 64:319~334

Brunt EM / 2001 / Nonalcoholic steatohepatitis : definition and pathology / Semin Liver Dis 21:3~16

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Asuman Nur Karhan

소속기관 Mersin University Faculty of Medicine

주요연구분야 의약학 > 임상의학 > 소아과학

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Hayriye Hizarcioglu-Gulsen