[Partial congenital deficiency in factor V associated with an intestinal malabsorption syndrome due to lambliasis. A familial survey (author's transl)].

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.