[4 cases of congenital afibrinogenemia (author's transl)].
Report on 4 members of one family with afibrinogenemia. 2 of them died of cerebral hemorrhage at the age of 10. The 2 surviving children are 8 and 10 years old. Plasmafibrinogen was determined in 69 members of the family. Only in the parents of 2 patients was plasmafibrinogen found to be slinghtly reduced. But in other members of the family fibrinogen was low. In our cases, too, the ADP induced aggregation of thrombocytes in plasma obtained with citrate was weaker than normal.
D000244:Adenosine Diphosphate; D000347:Afibrinogenemia / Q000150:complications / Q000235:genetics*; D002543:Cerebral Hemorrhage / Q000150:complications; D002648:Child; D002675:Child, Preschool; D002951:Citrates; D005340:Fibrinogen / Q000032:analysis; D006801:Humans; D007223:Infant; D010375:Pedigree; D010973:Platelet Adhesiveness
D002951:Citrates; D000244:Adenosine Diphosphate; D005340:Fibrinogen
Vier Fälle von kongenitaler Afibrinogenämie