[Intra-osseus hemorrhagic lesions in congenital afibrinogenemia. A new case (author's transl)].
Congenital afibrinemia is a rare disease, transmitted by an autosomal recessive mode, and due to deficient fibrinogen. Osseous involvement is exceptional, since only 2 cases have been reported. We describe a third case. The roentgenographic features are well limited radiolucent lesions, arising in the medullary space, and slightly expanding. These lesions are related to fibrous cysts, resulting from intraosseous hemorrhages. Spontaneous resolution may occur: the absence of fibrin deposits and blood clot probably favours resorption.
D000347:Afibrinogenemia / Q000150:complications / Q000151:congenital*; D001845:Bone Cysts / Q000000981:diagnostic imaging; D001847:Bone Diseases / Q000000981:diagnostic imaging / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D005269:Femur / Q000000981:diagnostic imaging; D006470:Hemorrhage / Q000000981:diagnostic imaging / Q000209:etiology*; D006801:Humans; D006811:Humerus / Q000000981:diagnostic imaging; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011859:Radiography; D012075:Remission, Spontaneous
Lésions hémorragiques intra-osseuses des afibrinémies congénitales. A propos d'un nouveau cas.