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Pregnancy, delivery and puerperium in hemophilia carrier
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Abstract
Among inherited bleeding disorders only hemophila A and B are recessive X-linked conditions. Bleeding diathesis affects males while females are mostly asymptomatic carriers of the hemophilia trait. There are however clinical conditions such as delivery and puerperium when hemophilia carriers are at inceased risk of hemorrhage. Wherever possible (i.e. knowledge of family history of hemophilia), in order to avoid bleeding complications genetic testing to confirm the status of suspected carrier should be performed in advance of planned pregnancy. The task of the obstetrician in charge of a pregnant hemophilia carrier is to recommend prenatal diagnosis to confirm or exclude hemophilia in early pregnancy and then to consider maternal and foetal risks and benefits as basis for selection of the most appropriate mode of delivery (cesarean versus natural delivery). As hemophilia carriers are at increased risk of postpartum hemorrhage, in puerperium they should be under comprehensive gynecological and hematological monitoring for factor VIII/IX levels and hemostatic treatment (factor VIII/IX concentrate) should be administered whenever necessary. A coagulation screen and appropriate factor levels assay should be performed on cord blood samples of all male infants delivered by hemophilia carriers. All at risk neonates should be carefully observed for sings of bleeding.
Abstract
Among inherited bleeding disorders only hemophila A and B are recessive X-linked conditions. Bleeding diathesis affects males while females are mostly asymptomatic carriers of the hemophilia trait. There are however clinical conditions such as delivery and puerperium when hemophilia carriers are at inceased risk of hemorrhage. Wherever possible (i.e. knowledge of family history of hemophilia), in order to avoid bleeding complications genetic testing to confirm the status of suspected carrier should be performed in advance of planned pregnancy. The task of the obstetrician in charge of a pregnant hemophilia carrier is to recommend prenatal diagnosis to confirm or exclude hemophilia in early pregnancy and then to consider maternal and foetal risks and benefits as basis for selection of the most appropriate mode of delivery (cesarean versus natural delivery). As hemophilia carriers are at increased risk of postpartum hemorrhage, in puerperium they should be under comprehensive gynecological and hematological monitoring for factor VIII/IX levels and hemostatic treatment (factor VIII/IX concentrate) should be administered whenever necessary. A coagulation screen and appropriate factor levels assay should be performed on cord blood samples of all male infants delivered by hemophilia carriers. All at risk neonates should be carefully observed for sings of bleeding.
Keywords
hemophilia carrier, pregnancy, delivery, prenatal diagnostics, replacement therapy
About this articleTitle
Pregnancy, delivery and puerperium in hemophilia carrier
Journal
Hematology in Clinical Practice
Issue
Article type
Review paper
Pages
14-22
Published online
2016-08-03
Page views
1656
Article views/downloads
9711
DOI
Bibliographic record
Hematologia 2016;7(1):14-22.
Keywords
hemophilia carrier
pregnancy
delivery
prenatal diagnostics
replacement therapy
Authors
Magdalena Górska-Kosicka
Edyta Odnoczko
Jerzy Windyga
