Aubry P, Capdevielle P, Durand J P, Laroche R, André L J
Ann Med Interne (Paris). 1979 Dec;130(12):631-4.
Owren's disease is a rare hemorrhagic diathesis which can occur in infancy as a severe hemorrhagic disorder. It also appears in adult life when clinical manifestations are those of acquired deficiencies of other coagulation factors. A familial survey enables a definite diagnosis to be made as it demonstrates the presence of deficiency in factor V in one or several members of the family. Such a case is reported in a young adult with an associated intestinal malabsorption syndrome due to lambliasis. After administration of vitamin K the deficiency in factor V remained an isolated disorder, and the hemostatic anomaly was found in three other members of the family. The deficiency was a partial one, which explains why the Owren's disease only became evident during the course of the malabsorption syndrome due to lambliasis which caused a reduction in the level of vitamin K dependent factors II, VII, and X.
奥伦氏病是一种罕见的出血素质，在婴儿期可表现为严重的出血性疾病。它也会出现在成人期，临床表现为其他凝血因子获得性缺乏。家族调查有助于明确诊断，因为它能证明家族中一个或几个成员存在因子V缺乏。本文报告了一例年轻成人病例，其伴有因梨形鞭毛虫病导致的肠道吸收不良综合征。给予维生素K后，因子V缺乏仍为孤立性病症，且在该家族的其他三名成员中也发现了止血异常。这种缺乏是部分性的，这就解释了为什么奥伦氏病仅在因梨形鞭毛虫病引起的吸收不良综合征过程中才变得明显，该综合征导致了维生素K依赖因子II、VII和X水平降低。
