The pregnancy outcome in patients with minor β-thalassemia

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Abstract

Background: β-thalassemia is the most common hereditary disease in Iran and more than 2 million carriers of the β-thalassemia mutant gene are living in this country.

Objective: To determine pregnancy outcome of women with β-thalassemia minor.

Materials and Methods: In this retrospective, case-control study in two universities affiliated hospitals in Shiraz, all pregnancies occurred between 2006 and 2008 were included. Patients were divided in two groups regarding the presence of β-thalassemia minor. Patients in case and control groups were matched according to maternal age, gestational age and number of previous pregnancies. Cesarean delivery, hypertensive disorders, gestational diabetes mellitus, premature rupture of membranes and preterm labor were recorded in each group and were compared using the χ ² or Fisher exact tests.

Results: Overall 510 β-thalassemia minor subjects and 512 healthy controls were studied. Cases with β-thalassemia minor had significantly higher prevalence of oligohydramnios (p<0.001) and cesarean section delivery (p=0.001). There was no significant difference regarding Apgar score in 1 ^st (p=0.65) and 5 ^th minute (p=0.25), IUGR (p=0.073), gestational diabetes mellitus (DM) (p=0.443) and preeclampsia (p=0.116) between two study groups.

Conclusion: β-thalassemia minor does not significantly influence the pregnancy outcome in the negative way.

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Most cited references 23

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Thalassemia in Iran: epidemiology, prevention, and management.

A Ehsani, Sirous Zeinali, M Rahiminejad … (2007)

To determine the prevalence and geographic distribution of thalassemia and to evaluate the success of the thalassemia prevention and treatment programs in Iran. Data were obtained from the National Thalassemia Registry of Iran, Iranian Blood Transfusion Organization, genetic laboratories involved in prenatal diagnosis, related pharmaceutical companies, and centers performing bone marrow transplantation for thalassemic patients. A total of 13,879 living patients have been registered, mostly from the northern and southern parts of Iran with the median age of 15 years. Twenty-three percent of patients were older than 20 years. The number of newly diagnosed cases has been decreased considerably after the start of the prevention program. Since the introduction of prenatal diagnosis, 2819 couples (2549 fetuses) have been tested, with only 6 false results. Elective abortion was not performed in 10 affected fetuses. Most common mutations detected were IVS II-1 and IVS I-5. In 2003, approximately 25% of the national blood products and 6 million vials of desferal were used for thalassemic patients. Overall, 340 patients have received allogeneic bone marrow transplantation, of those 46 patients deceased. Bloodborne infections have also been decreased significantly owing to the national screening of blood products for bloodborne viral infections. Owing to the national prevention program and provided special care, the age distribution of thalassemic patients in Iran is getting adapted to a full prevention and treatment program and life expectancy of these patients has been increased considerably. This shift in the age distribution of thalassemia, a traditionally considered pediatric disease, will face us with new challenges and the health care system should be prepared for this new face of thalassemia.

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The beta-thalassemia mutation spectrum in the Iranian population.

M. Karimi, H Najmabadi, N Amirizadeh … (2001)

Beta-thalassemia is the most common hereditary disease in Iran. More than two million carriers of beta-thalassemia live in Iran. Since the Iranian population is a mixture of different ethnic groups, it is necessary to determine the frequency and distribution of mutations in the different parts of the country. For this purpose, we divided Iran in to eight different regions according to the geographic and ethnic distribution of the population. Over a 10-year period 1,217 beta-thalassemia chromosomes of 164 affected patients and 889 unrelated carriers were studied using the amplification refractory mutation system-polymerase chain reaction technique. We detected 81% beta-thalassemia mutations in the studied chromosomes. IVS-II-I (G --> A) was the predominant mutation found in our study (34%). Its relative frequency in the north was much higher than other regions, and it lessened toward the south, where the IVS-I-5 (G --> C) mutation was more common. IVS-I-5 (G --> C) (7.55%), codons 8/9 (+ G) (4.76%), and IVS-I-110 (G --> A) (4.76%) were the other most common mutations. The results presented here can be used as a basis of prenatal diagnosis of beta-thalassemia in different regions of Iran.

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Fertility in female patients with thalassemia.

N Skordis, N Pavlides, M Koliou … (1997)

With recent therapeutic advances, thalassemic patients can now reach adulthood and attain reproductive capacity. Endocrine complications due to hemosiderosis 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 the frequency of fertility among our female thalassemic patients. Our population included 50 married women with thalassemia major (TM) and 12 with thalassemia intermedia (TI) who are regularly followed in our thalassemic centers. Of the 50 patients with TM, 7 had primary amenorrhea (PA), 9 had secondary amenorrhea (SA), and 34 had normal menstrual function (NM), as did all the patients with TI. Overall we had 62 women who were able to achieve 90 pregnancies and give birth to 87 healthy babies. Most of our patients became pregnant around the age of 25 years. Associated endocrine complications were rare except in the group of patients with PA, as expected. In all patients with PA and SA, the 17 pregnancies were induced (intercourse 10, insemination 3, IVF 4). In the patients with NM and TI, 66 pregnancies were achieved spontaneously and 7 following induction (insemination 3, IVF 4). There were four twin and one triple pregnancies, which all resulted in premature deliveries. Among the seven couples in which both partners had thalassemia major, sperm donation was used in 5 cases, ovum donation in one case, and one pregnancy was achieved spontaneously. These 90 pregnancies resulted in 69 full-term, 12 pre-term, 7 abortions and 2 stillbirths. No severe obstetric complication was observed except for two patients with preeclampsia. One patient with PA who carried the triple pregnancy developed severe cardiac failure, 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 transient increase of ESD and EDD during pregnancy, with return to normal after delivery. Labor was performed by Caesarian section in 26 births (26%) out of the 81 successful pregnancies. These collected data represent the largest number of pregnancies in thalassemic females reported so far and are clearly encouraging for the ultimate improvement of the quality of life in thalassemic patients.

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Author and article information

Journal

Journal ID (nlm-ta): Iran J Reprod Med

Journal ID (iso-abbrev): Iran J Reprod Med

Journal ID (publisher-id): IJRM

Title: Iranian Journal of Reproductive Medicine

Publisher: Research and Clinical Center for Infertility (Yazd, Iran )

ISSN (Print): 1680-6433

ISSN (Electronic): 2008-2177

Publication date (Print): Winter 2011

Volume: 9

Issue: 1

Pages: 9-14

Affiliations

[1 ]Department of Obstetrics and Gynecology, Shiraz University of Medical Sciences, Shiraz, Iran.

[2 ]Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

Author notes

Corresponding Author: Mehran Karimi, Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Email: karimim@sums.ac.ir

Article

Publisher ID: ijrm-9-009

PMC ID: 4212139

PubMed ID: 25356075

SO-VID: 450701b3-d5bd-4548-a836-5963bd1ec6f5

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution License, ( http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

The pregnancy outcome in patients with minor β-thalassemia

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Most cited references 23

Thalassemia in Iran: epidemiology, prevention, and management.

The beta-thalassemia mutation spectrum in the Iranian population.

Fertility in female patients with thalassemia.

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