Fertility in female patients with thalassemia

Citation
N. Skordis et al., Fertility in female patients with thalassemia, J PED END M, 11, 1998, pp. 935-943
Citations number
22
Categorie Soggetti
Endocrinology, Nutrition & Metabolism
Journal title
JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM
ISSN journal
0334018X → ACNP
Volume
11
Year of publication
1998
Supplement
3
Pages
935 - 943
Database
ISI
SICI code
0334-018X(199812)11:<935:FIFPWT>2.0.ZU;2-Q
Abstract
With recent therapeutic advances, thalassemic patients can now reach adulth ood and attain reproductive capacity. Endocrine complications due to hemosi derosis and especially hypogonatotropic hypogonadism, which present either with sexual infantilism and primary amenorrhea or with secondary amenorrhea , are common in thalassemic women. The aim of this study was to estimate th e frequency of fertility among our female thalassemic patients. Our populat ion included 50 married women with thalassemia major (TM) and 12 with thala ssemia intermedia (TI) who are regularly followed in our thalassemic center s. Of the 50 patients with TM, 7 had primary amenorrhea (PA), 9 had seconda ry amenorrhea (SA), and 34 had normal menstrual function (NM), as did all t he patients with TI. Overall we had 62 women who were able to achieve 90 pr egnancies and give birth to 87 healthy babies. Most of our patients became pregnant around the age of 25 years. Associated endocrine complications wer e rare except in the group of patients with PA, as expected. In all patient s with PA and SA, the 17 pregnancies were induced (intercourse 10, insemina tion 3, IVF 4). In the patients with NM and TI, 66 pregnancies were achieve d spontaneously and 7 following induction (insemination 3, IVF 4). There we re four twin and one triple pregnancies, which all resulted in premature de liveries. Among the seven couples in which both partners had thalassemia ma jor, sperm donation was used in 5 cases, ovum donation in one case, and one pregnancy was achieved spontaneously. These 90 pregnancies resulted 69 ful l-term, 12 pre-term, 7 abortions and 2 stillbirths. No severe obstetric com plication was observed except for two patients with preeclampsia. One patie nt with PA who carried the triple pregnancy developed severe cardiac failur e, which was successfully treated. Transfusion requirements were increased during pregnancy. Discontinuation of desferrioxamine resulted in elevation of ferritin levels during the second and third trimesters of pregnancy and after delivery. Nine patients who were examined with cardiac echo had a tra nsient increase of ESD and EDD during pregnancy, with return to normal afte r delivery. Labor was performed by Caesarian section in 26 births (26%) out of the 81 successful pregnancies. These collected data represent the large st number of pregnancies in thalassemic females reported so far and are cle arly encouraging for the ultimate improvement of the quality of life in tha lassemic patients.