Ebert R F, Bell W R
Thromb Res. 1985 Apr 15;38(2):121-8. doi: 10.1016/0049-3848(85)90054-4.
A congenital dysfibrinogenemia, fibrinogen Baltimore IV, has been found in a 56-year-old Caucasian man. Clinical laboratory studies disclosed a slightly prolonged prothrombin time, but were otherwise unremarkable. Release of fibrinopeptides by thrombin occurs normally, as does ligation of the fibrin polymer by Factor XIII. Approximately half of the isolated fibrin monomers polymerize normally, but the remainder polymerize at about 2% of the initial rate. The functional defect is thus limited to a decrease in the rate of fibrin monomer polymerization.
在一名56岁的白种男性中发现了一种先天性纤维蛋白原异常血症——巴尔的摩IV型纤维蛋白原。临床实验室研究显示凝血酶原时间略有延长,但其他方面并无异常。凝血酶释放纤维蛋白肽的过程正常,因子XIII连接纤维蛋白聚合物的过程也正常。大约一半分离出的纤维蛋白单体能正常聚合,但其余的聚合速度约为初始速度的2%。因此,功能缺陷仅限于纤维蛋白单体聚合速率的降低。