与雌激素治疗相关的无低纤维蛋白原血症的纤维蛋白原储存病
Fibrinogen storage disease without hypofibrinogenemia associated with estrogen therapy.
作者信息
Simsek Z, Ekinci O, Cindoruk M, Karakan T, Degertekin B, Akyol G, Unal S
机构信息
Gastroenterology Department, Gazi University, Faculty of Medicine, Ankara, Turkey.
出版信息
BMC Gastroenterol. 2005 Nov 15;5:36. doi: 10.1186/1471-230X-5-36.
BACKGROUND
Cytoplasmic inclusion bodies within hepatocytes may have different etiologies, including the Endoplasmic Reticulum Storage Diseases (ERSDs). ERSD is a pathological condition characterized by abnormal accumulation of proteins destined for secretion in the endoplasmic reticulum of hepatocytes; it may be congenital (primary) or acquired (secondary). Fibrinogen storage disease is a form of ERSD.
CASE PRESENTATION
We present a case of fibrinogen storage disease secondary to estrogen replacement therapy. Its causal relationship to the drug is shown by histological, immunohistochemical and ultrastructural studies of paired liver biopsies obtained during and after the drug therapy.
CONCLUSION
The liver biopsies of patients with idiopathic liver enzyme abnormalities should be carefully evaluated for cytoplasmic inclusion bodies and, although rare, fibrinogen deposits.
背景
肝细胞内的细胞质包涵体可能有不同病因，包括内质网储存疾病（ERSDs）。ERSD是一种病理状态，其特征是肝细胞内质网中分泌性蛋白质异常蓄积；它可能是先天性（原发性）的，也可能是后天获得性（继发性）的。纤维蛋白原储存病是ERSD的一种形式。
病例报告
我们报告一例继发于雌激素替代疗法的纤维蛋白原储存病病例。在药物治疗期间及之后获取的配对肝脏活检组织的组织学、免疫组化和超微结构研究显示了其与药物的因果关系。
结论
对于特发性肝酶异常患者的肝脏活检，应仔细评估细胞质包涵体以及虽然罕见但存在的纤维蛋白原沉积。
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