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Course of pregnancy and birth in a patient with Bernard-Soulier syndrome – a case report

  • Gohar Rahimi , Solvejg Rellecke , Peter Mallmann and Frank Nawroth
Published/Copyright: May 1, 2005
Journal of Perinatal Medicine
From the journal Volume 33 Issue 3

Abstract

Bernard-Soulier syndrome is a rare autosomal recessive bleeding disorder characterized by platelet dysfunction, the presence of giant platelets, and a prolonged bleeding time. Absent or reduced expression of the platelet glycoprotein (GP) Ib-IX receptor results in the Bernard-Soulier syndrome and is characterized by severe bleeding and macrothrombocytopenia. To our knowledge only ten pregnant patients with this disease have been previously reported. In some patients the pregnancy course was uneventful while in others post-partum hemorrhage and resulting hysterectomy was the most common complication. We present a primiparous female with Bernard-Soulier syndrome who had delayed post-partum hemorrhage and review the literature.

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Corresponding author: ENDOKRINOLOGIKUM Hamburg, Centre for Hormone and Metabolic Diseases, Gynecological Endocrinology and Reproductive Medicine, Lornsenstr. 4-6, 22767 Hamburg Germany. E-mail:

References

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Published Online: 2005-05-01
Published in Print: 2005-05-01

© by Walter de Gruyter Berlin New York

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