Gulino F A, Vitale S G, Fauzia M, Cianci S, Pafumi C, Palumbo M A
Bratisl Lek Listy. 2013;114(9):523-5. doi: 10.4149/bll_2013_109.
It was studied the clinical management and the medical outcomes of 6 pregnancies in 5 women affected by Beta Thalassemia major, based on last guidelines and pharmacological treatments.
Paediatric Department and Department of Obstetrics and Gynaecology of the University of Catania.
These patients were taken among a group of 116 women affected by beta-thalassemia major divided into three subgroups, according to the characteristics of their menstrual cycle: 1) women with primitive amenorrhoea, 2) women with secondary amenorrhoea and 3) women with normal menstruation. Only one woman, affected by primitive amenorrhoea, needed the induction of ovulation. An accurate and detailed pre-pregnancy assessment was effected before each conception. This was constituted by a series of essays, including checks for diabetes and hypothyroidism, for B and C hepatitis and for blood group antibodies. Moreover were evaluated: cardiac function, rubella immunity and transaminases. Other pregnancy monitoring, and cares during labour and delivery were effected according to usual obstetrics practice.
All the women were in labour when they were 38 week pregnant, and the outcome were six healthy babies born at term. There were no complications related to the pregnancy and to the immediate outcome after delivery.
The improvements of current treatments, especially in the management of iron deposits, the prolongation of survival rate, will result in a continuous increase of pregnancies in thalassemic women. Pregnancy is now a real possibility for women affected by such disease. Although numerous complications can occur, vigilant monitoring by both experienced obstetricians and hematologists can lead to successful pregnancy outcomes (Tab. 1, Fig. 1, Ref. 16).
根据最新指南和药物治疗方法，研究5例重型β地中海贫血女性患者6次妊娠的临床管理及医疗结局。
卡塔尼亚大学儿科与妇产科。
这些患者选自116例重型β地中海贫血女性患者，根据月经周期特点分为三个亚组：1）原发性闭经女性；2）继发性闭经女性；3）月经正常女性。仅1例原发性闭经女性需要诱导排卵。每次受孕前均进行了准确而详细的孕前评估。评估包括一系列检查，如糖尿病、甲状腺功能减退、B型和C型肝炎及血型抗体检查。此外，还评估了心脏功能、风疹免疫力和转氨酶。其他孕期监测以及分娩时和分娩后的护理均按照常规产科操作进行。
所有女性在怀孕38周时分娩，结局为6名足月出生的健康婴儿。无与妊娠及分娩后近期结局相关的并发症。
当前治疗方法的改进，尤其是在铁沉积管理方面，以及生存率的延长，将导致地中海贫血女性妊娠次数持续增加。对于受此类疾病影响的女性来说，怀孕现在已成为现实。尽管可能会出现许多并发症，但经验丰富的产科医生和血液科医生的密切监测可带来成功的妊娠结局（表1、图1、参考文献16）。
