[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.
D000293:Adolescent; D000328:Adult; D002648:Child; D005166:Factor V Deficiency / Q000150:complications* / Q000235:genetics / Q000628:therapy; D005260:Female; D005873:Giardiasis / Q000150:complications*; D006470:Hemorrhage / Q000209:etiology; D006801:Humans; D008286:Malabsorption Syndromes / Q000150:complications*; D008297:Male; D010375:Pedigree
Déficit congénital partiel en facteur V associé à une malabsorption intestinale due à une lambliase. Enquête familiale.