Karagiorga-Lagana M
Thalassemia Unit, The Aghia Sophia Children's Hospital, Athens, Greece.
J Pediatr Endocrinol Metab. 1998;11 Suppl 3:945-51.
Pregnancy in beta-thalassemic patients has become a not unusual event, especially in the last 10 years. The course and outcome of 19 pregnancies in 16 thalassemic women, followed in our unit, 12 with thalassemia major and 4 with thalassemia intermedia, were studied. Genetic counselling was provided and counselling regarding the planning or the continuation of the pregnancy was based mainly on cardiac performance at rest. Cardiac, endocrine and liver function were evaluated at baseline, monitored throughout pregnancy and reevaluated after delivery. Desferrioxamine treatment was discontinued as early as possible. During pregnancy the Hb level was maintained at about 10 g/dl in all women by transfusion. The course of pregnancy was essentially uneventful and elective Cesarean section was performed in all cases. The mean birth weight of the newborns was 3000 g. All babies were normal except for one with exomphalus. Pregnancy was well tolerated by the heart in all women and no endocrinological disorders were observed. In conclusion, pregnancies in beta-thalassemia can be safe for both mothers and their babies with careful selection and appropriate care.
β地中海贫血患者怀孕已变得并不罕见，尤其是在过去10年。我们对本单位收治的16名地中海贫血女性的19次妊娠过程及结局进行了研究，其中12例为重型地中海贫血，4例为中间型地中海贫血。提供了遗传咨询，关于妊娠计划或继续妊娠的咨询主要基于静息时的心脏功能。在基线时评估心脏、内分泌和肝功能，在整个孕期进行监测，并在产后重新评估。尽早停用去铁胺治疗。孕期通过输血使所有女性的血红蛋白水平维持在约10 g/dl。妊娠过程基本顺利，所有病例均行择期剖宫产。新生儿平均出生体重为3000 g。除1例脐膨出外，所有婴儿均正常。所有女性的心脏对妊娠耐受性良好，未观察到内分泌紊乱。总之，通过仔细选择和适当护理，β地中海贫血患者的妊娠对母亲及其婴儿都可以是安全的。
