系统性原发性淀粉样变性缺乏因子7:一例罕见病例

F. Dursun, Erdal Akyar, G. Uygun, Z. Başlar, B. Çobanoğlu
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引用次数: 1

摘要

简介:已知在全身性原发性淀粉样变性中存在单独的和联合的因子缺乏。在这些病例中,已知最常见的因子缺乏是孤立的因子X缺乏。其他因素缺乏相对较少。在全身性原发性淀粉样变性病例中,很少发生孤立性因子VII缺乏。病例报告:一名58岁男性患者,先前以全身性水肿、疲劳、瘙痒为主诉到另一个卫生中心就诊,有蛋白尿,然后在蛋白尿病因肾活检后诊断为全身性原发性淀粉样变性。患者实验室检查显示凝血酶原时间延长和因子VII缺乏。患者对原发性淀粉样变性和因子VII缺乏症的治疗反应良好。讨论:在全身性原发性淀粉样变性病例中,如果凝血酶原时间延长的病因不涉及肝脏疾病、使用华法林或吸收不良,医生应始终牢记罕见的因子缺乏,如因子7缺乏,以及常见的因子缺乏。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Factor VII Deficiency in Systemic Primary Amyloidosis: A Rare Case
Introduction: Isolated and combined factor deficiencies are known to occur in systemic primary amyloidosis. The most common factor deficiency known in these cases is isolated factor X deficiency. Other factor deficiencies are relatively less frequent. Isolated factor VII deficiency occurs very rarely in cases of systemic primary amyloidosis.Case report: A 58-year-old male patient previously presenting to another health center with complaints of generalized edema, fatigue, and itching had proteinuria and then he was diagnosed with systemic primary amyloidosis after the renal biopsy for proteinuria etiology. The patient’s laboratory tests showed prolongation of prothrombin time and factor VII deficiency. The patient responded well to the treatment for primary amyloidosis and factor VII deficiency.Discussion: In cases of systemic primary amyloidosis, if the etiology of prolonged prothrombin time involves no liver disease, warfarin use, or malabsorption, physicians should always keep in mind rare factor deficiencies such as factor VII deficiency, along with common factor deficiencies.
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