Giant Cell Hepatitis With Autoimmune Hemolytic Anemia: A Case Report and Review of Pediatric Literature
Giant Cell Hepatitis With Autoimmune Hemolytic Anemia: A Case Report and Review of Pediatric Literature
复制标题
巨细胞肝炎伴自身免疫性溶血性贫血：一例报告及儿科文献综述
DOI：
10.1177/0009922810379501
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发表时间：
2011
影响因子：
1.6
通讯作者：
R. Dębski
中科院分区：
文献类型：
--
作者：
S. Raj;T. Stephen;R. Dębski
巨细胞性肝炎是一种罕见的进行性肝病的临床组织病理学描述。GCH主要见于患有特发性胆汁淤积的新生儿，在婴儿期后年龄组的发生率极低。GCH通常具有暴发性和侵袭性的临床过程，需要多种免疫抑制剂和/或肝移植。它与自身免疫性溶血性贫血（AHA）的关联是一个独特的实体（GCH-AHA），在儿科年龄组中报告了29例病例，包括我们的患者。我们描述了一个6个月大的白人女性谁提出了最初与AHA其次是GCH。我们成功地用强的松和硫唑嘌呤治疗了她，她目前在3岁时病情缓解。
Giant cell hepatitis (GCH) is a clinicohistopathologic description for an unusual form of progressive liver disease. GCH is mostly seen as a finding in neonates with idiopathic cholestasis, and its occurrence in the postinfantile age group is extremely rare. GCH often has a fulminant and aggressive clinical course requiring multiple immunosuppressive agents and/or liver transplantation. Its association with autoimmune hemolytic anemia (AHA) is a distinct entity (GCH-AHA) with 29 reported cases in the pediatric age group, including our patient. We describe a 6-month-old Caucasian female who presented initially with AHA followed by GCH. We successfully treated her with prednisone and azathioprine, and she is currently in remission at 3 years of age.