2例儿童肝脏铁过载：非酒精性脂肪性肝炎和青少年血色素沉着症
Iron Overload in the Liver of 2 Children: Nonalcoholic Steatohepatitis and Juvenile Hemochromatosis.
作者信息
Ünlüsoy Aksu Aysel, Caleffi Angela, Pietrangelo Antonello, Sari Sinan, Eğritaş Gürkan Ödül, Demirtaş Zeliha, Yilmaz Güldal, Dalgiç Buket
机构信息
Departments of *Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition ‡Pathology, Gazi University Faculty of Medicine, Ankara, Turkey †"Mario Coppo" Liver Research Center, Division of Internal Medicine 2 and Center for Hemochromatosis, University Hospital of Modena, Modena, Italy.
出版信息
J Pediatr Hematol Oncol. 2017 Aug;39(6):466-469. doi: 10.1097/MPH.0000000000000752.
BACKGROUND
Iron overload disorders are hereditary hemochromatosis and secondary etiologies other than hereditary hemochromatosis. We describe 2 boys presenting with iron overload. Juvenile hemochromatosis and nonalcoholic steatohepatitis (NASH) related iron overload are the genetic and secondary causes, respectively.
OBSERVATIONS
Both patients benefited from phlebotomy even if they had different etiologies.
CONCLUSIONS
In childhood, the diagnosis of iron overload syndromes is crucial because they do not confront us with obvious symptoms and findings. Early initiation of a phlebotomy program can prevent mortality. NASH might lead to iron overload and iron overload might aggravate the clinical course of NASH.
背景
铁过载疾病包括遗传性血色素沉着症以及遗传性血色素沉着症以外的继发性病因。我们描述了2名出现铁过载的男孩。青少年血色素沉着症和非酒精性脂肪性肝炎（NASH）相关的铁过载分别是其遗传和继发性病因。
观察结果
两名患者均从放血疗法中获益，尽管他们病因不同。
结论
在儿童时期，铁过载综合征的诊断至关重要，因为它们不会给我们带来明显的症状和体征。早期启动放血疗法可预防死亡。NASH可能导致铁过载，而铁过载可能会加重NASH的临床病程。
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