Brenner B, Fishman A, Goldsher D, Schreibman D, Tavory S
Institute of Hematology, Rambam Medical Center, Haifa, Israel.
Am J Hematol. 1988 Mar;27(3):209-11. doi: 10.1002/ajh.2830270311.
An Israeli Arab family with type I antithrombin III (AT-III) deficiency with several affected symptomatic members in three generations is reported. The propositus presented with deep vein thrombosis and pulmonary emboli associated with gestation. The propositus infant presented at the age of 2 weeks with superior sagittal and rectus sinus thrombosis. Hereditary AT-III deficiency should be considered in infants with cerebral thrombosis, especially if they have a family history of thromboembolism. The role of prophylactic therapy by AT-III concentrates in these infants should be further assessed.
本文报道了一个患有I型抗凝血酶III(AT-III)缺乏症的以色列阿拉伯家庭,三代中有几名受影响的有症状成员。先证者出现与妊娠相关的深静脉血栓形成和肺栓塞。先证者婴儿在2周龄时出现上矢状窦和直窦血栓形成。对于患有脑血栓形成的婴儿,尤其是有血栓栓塞家族史的婴儿,应考虑遗传性AT-III缺乏症。AT-III浓缩物预防性治疗在这些婴儿中的作用应进一步评估。