伴有新生儿胆汁淤积症和广泛血色素沉着症的β地中海贫血:病例报告

Chung Gang Jung, Jang Hoon Lee, Y. Kim
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引用次数: 0

摘要

新生儿胆汁淤积症由各种形式的肝损伤引起,病因复杂。其中,原发性溶血病导致的重症胆汁淤积症病例十分罕见。在此,我们报告了一例地中海贫血引起的严重溶血导致胆管损伤的病例,患者出生后不久即出现胆汁淤积,并持续了4个月以上。此外,从生化和组织学角度观察到肝脏病理完全恢复。因此,临床医生在新生儿胆汁淤积症的鉴别诊断中,应将溶血病作为一种罕见病因,并根据病例进展情况考虑保守治疗的可取性。
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Beta Thalassemia Presenting with Neonatal Cholestasis and Extensive Hemosiderosis: A Case Report
Neonatal cholestasis is caused by various forms of liver injury and has a complex etiological background. Among these, cases of severe cholestasis due to primary hemolytic disease are rare. Herein, we report a case in which thalassemia-induced severe hemolysis caused bile duct injury by hemosiderosis, with cholestasis occurring shortly after birth and lasting for >4 months. In addition, complete recovery of liver pathology was observed both biochemically and histologically. Hence, clinicians should consider hemolytic disease as a rare cause of neonatal cholestasis in the differential diagnosis of neonates as well as the advisability of conservative treatment based on case progression.
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