Pregnancy outcome in congenital dyserythropoietic anemia type I

Hanna Shalev, Galit Perez Avraham, Reli Hershkovitz, Amalia Levy, Eyal Sheiner, Itai Levi, Hannah Tamary

    Research output: Contribution to journalArticlepeer-review

    9 Scopus citations

    Abstract

    Objectives: Congenital dyserythropoietic anemia type I (CDA I) is a rare inherited disease characterized by moderate to severe macrocytic anemia and abnormal erythroid precursors with nuclear chromatin bridges and spongy heterochromatin. Moderate to severe maternal anemia is a recognized independent risk factor for low birth weight (LBW) and complicated delivery. The aim of the study was to review the outcome of pregnancies in women with CDA I. Methods: The clinical and laboratory records of 28 spontaneous pregnancies in six Bedouin women with CDA I were reviewed. The results were compared with findings from a retrospective review of a large population-based registry including all pregnancies in Bedouin women during the same 15-yr period. Results: Eighteen pregnancies in women with CDA I (64%) were complicated. One pregnancy was aborted spontaneously in the first trimester and one resulted in a non-viable fetus (stillborn at 26 wk). Cesarean section (CS) was performed in 10 pregnancies (36%). Eleven of the 26 newborns (42%) had a LBW: six were born prematurely and five were small for gestational age. The odds ratio for CS in women with CDA I compared with healthy Bedouin women was 4.5 [95% confidence interval (CI) 1.2-10.3], and for a LBW infant, 5.5 (95% CI 2.4-12.3). Careful follow-up was associated with significantly better fetal outcome (P = 0.05). Conclusions: Pregnancies in women with CDA I are at high risk for delivery-related and outcome complications. To improve fetal outcome, women with CDA I should be carefully monitored during pregnancy.

    Original languageEnglish
    Pages (from-to)317-321
    Number of pages5
    JournalEuropean Journal of Haematology
    Volume81
    Issue number4
    DOIs
    StatePublished - 1 Oct 2008

    Keywords

    • CDA I
    • Low birth weight
    • Pregnancy
    • Pregnancy outcome
    • Preterm delivery

    ASJC Scopus subject areas

    • Hematology

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