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镰状细胞血红蛋白病合并妊娠期间预防性输注正常红细胞。

Prophylactic transfusions of normal red blood cells during pregnancies complicated by sickle cell hemoglobinopathies.

作者信息

Cunningham F G, Pritchard J A, Mason R, Chase G

出版信息

Am J Obstet Gynecol. 1979 Dec 1;135(7):994-1003. doi: 10.1016/0002-9378(79)90825-1.

DOI:10.1016/0002-9378(79)90825-1
PMID:507138
Abstract

Prophylactic transfusions of normal donor red cells were administered during 37 pregnancies to women with sickle cell anemia, sickle cell-hemoglobin C disease, or sickle cell-beta thalassemia disease. Once the diagnosis was confirmed, the transfusions were administered intermittently throughout the rest of the pregnancy in such amounts and at such frequencies that no more than 60% of the circulating red cells contained hemoglobin S and the hematocrit was above 25. The maternal mortality rate was zero and maternal morbidity as the consequence of the sickle cell hemoglobinopathy was minimal. The perinatal mortality rate was appreciably reduced when compared to that previously observed without prophylactic transfusions but perinatal morbidity was still excessive. Evidence that the intrauterine environment was compromised, in spite of the transfusions consisted of an increased frequency of growth-retarded fetuses, of meconium staining of amnionic fluid, and of ominous decelerations of fetal heart rate. Morbidity from the transfusions was troublesome. Nonetheless, it is concluded tentatively that both the mother with a sickle cell hemoglobinopathy and her fetus are likely to benefit from prophylactic transfusions of normal donor red cells administered during one pregnancy according to the protocol employed in this study.

摘要

在37例患有镰状细胞贫血、镰状细胞-血红蛋白C病或镰状细胞-β地中海贫血病的孕妇孕期,输注了正常供者的红细胞进行预防性输血。一旦确诊,在孕期剩余时间内间歇性输血,输血的量和频率应使循环红细胞中不超过60%含有血红蛋白S,且血细胞比容高于25。孕产妇死亡率为零,镰状细胞血红蛋白病导致的孕产妇发病率极低。与之前未进行预防性输血时相比,围产期死亡率明显降低,但围产期发病率仍然过高。尽管进行了输血,但仍有证据表明子宫内环境受到损害,表现为生长受限胎儿的发生率增加、羊水胎粪污染以及胎儿心率出现不祥减速。输血引起的发病率令人困扰。尽管如此,初步得出结论,患有镰状细胞血红蛋白病的母亲及其胎儿,按照本研究采用的方案,在一次孕期进行正常供者红细胞预防性输血可能会受益。

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Prophylactic transfusions of normal red blood cells during pregnancies complicated by sickle cell hemoglobinopathies.镰状细胞血红蛋白病合并妊娠期间预防性输注正常红细胞。
Am J Obstet Gynecol. 1979 Dec 1;135(7):994-1003. doi: 10.1016/0002-9378(79)90825-1.
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Pregnancy and sickle cell hemoglobinopathies: results with and without prophylactic transfusions.妊娠与镰状细胞血红蛋白病:预防性输血与未输血的结果
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Prophylactic red-cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study.镰状细胞病孕妇的预防性红细胞输血。一项随机合作研究。
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