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Cerebral Sinus Thrombosis During Pregnancy Associated with Protein S Deficiency: Report of a Rare Case

Year 2014, Volume: 21 Issue: 4, 307 - 309, 01.08.2014

Abstract

Hereditary thrombophylic mutations are seen in 10% of the general population and many cases are diagnosed as a result of complications that occur during pregnancy. Of these, Protein S deficiency is a rare hereditary trombophilia with severe thrombogenic features. Pregnancies accompanying protein S deficiency often result in loss of fetus. In this paper, we present the case of a protein S deficiency patient who eventually had a live birth despite the cerebral sinus thrombosis in the 30th week of her pregnancy and discuss the case with references to the relevant literature. Key words: Protein S Deficiency; Hereditary Thrombophilia; Cerebral Sinus Trombosis; Pregnancy.

References

  • Rezende SM, Simmonds RE, Lane DA. Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S-C4b binding protein complex. Blood. 2004; 103(4):1192-201.
  • Buchanan GS, Rodgers GM, Ware Branch D. The inherited thrombophilias: genetics, epidemiology, and laboratory evaluation. Best Pract Res Clin Obstet Gynaecol. 2003;17(3):397-411.
  • Marlar RA, Neumann A. Neonatal purpura fulminans due to homozygous protein C or protein S deficiencies. Semin Thromb Hemost. 1990;16(4):299–309.
  • Bowles L, Cohen H. Inherited thrombophilias and anticoagulation in pregnancy. Best Pract Res Clin Obstet Gynaecol. 2003;17(3):471–489.
  • Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology. Investigation and management of heritable thrombophilia. Br J Haematol 2001;114(3):512-28.
  • Wypasek E, Undas A. Protein C and protein S deficiency - practical diagnostic issues. Adv Clin Exp Med. 2013;22(4):459-67.
  • Forestier F, Daffos F, Rainaut M, Solé Y, Amiral J. Vitamin K dependent proteins in fetal hemostasis at mid trimester of pregnancy. Thromb Haemost. 1985;24:53(3):401-3
  • Alvis-Miranda HR, Milena Castellar-Leones S, Alcala-Cerra G, Rafael Moscote-Salazar L. Cerebral sinus venous thrombosis. J Neurosci Rural Pract. 2013;4(4):427-38.
  • Romualdi E, Dentali F, Rancan E, Squizzato A, Steidl L, Middeldorp S et al.. Anticoagulant therapy for venous thromboembolism during pregnancy: a systematic review and a meta-analysis of the literature. J Thromb Haemost. 2013;11(2):270-81.

Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu

Year 2014, Volume: 21 Issue: 4, 307 - 309, 01.08.2014

Abstract

Herediter trombofilik mutasyonlar genel popülasyonun %10'unda görülür ve bir çok olgu gebelik sırasında ortaya çıkan komplikasyonlar sonucunda tanı alır. Bunlardan Protein S eksikliği nadir görülen ve şiddetli trombojenik özellikte bir herediter trombofiliadır. Protein S eksikliği eşlik eden gebelikler genelde fetal kayıpla sonuçlanır. Bu yazıda canlı doğumla son bulan gebeliği sırasında, 30. gebelik haftasında serebral sinüs trombozu geçiren ve tetkiklerinde protein S eksikliği saptanan bir olgu sunularak konu literatür bilgileri eşliğinde tartışıldı. Anahtar kelimeler: Protein S Eksikliği; Herediter Trombofilia; Serebral Sinüs Trombozu; Gebelik.

References

  • Rezende SM, Simmonds RE, Lane DA. Coagulation, inflammation, and apoptosis: different roles for protein S and the protein S-C4b binding protein complex. Blood. 2004; 103(4):1192-201.
  • Buchanan GS, Rodgers GM, Ware Branch D. The inherited thrombophilias: genetics, epidemiology, and laboratory evaluation. Best Pract Res Clin Obstet Gynaecol. 2003;17(3):397-411.
  • Marlar RA, Neumann A. Neonatal purpura fulminans due to homozygous protein C or protein S deficiencies. Semin Thromb Hemost. 1990;16(4):299–309.
  • Bowles L, Cohen H. Inherited thrombophilias and anticoagulation in pregnancy. Best Pract Res Clin Obstet Gynaecol. 2003;17(3):471–489.
  • Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology. Investigation and management of heritable thrombophilia. Br J Haematol 2001;114(3):512-28.
  • Wypasek E, Undas A. Protein C and protein S deficiency - practical diagnostic issues. Adv Clin Exp Med. 2013;22(4):459-67.
  • Forestier F, Daffos F, Rainaut M, Solé Y, Amiral J. Vitamin K dependent proteins in fetal hemostasis at mid trimester of pregnancy. Thromb Haemost. 1985;24:53(3):401-3
  • Alvis-Miranda HR, Milena Castellar-Leones S, Alcala-Cerra G, Rafael Moscote-Salazar L. Cerebral sinus venous thrombosis. J Neurosci Rural Pract. 2013;4(4):427-38.
  • Romualdi E, Dentali F, Rancan E, Squizzato A, Steidl L, Middeldorp S et al.. Anticoagulant therapy for venous thromboembolism during pregnancy: a systematic review and a meta-analysis of the literature. J Thromb Haemost. 2013;11(2):270-81.
There are 9 citations in total.

Details

Primary Language Turkish
Journal Section Articles
Authors

Murat Bulanık This is me

Zeynep Özcan Dağ This is me

Demet Demiray This is me

Ersel Dağ This is me

Yavuz Şimşek This is me

Publication Date August 1, 2014
Published in Issue Year 2014 Volume: 21 Issue: 4

Cite

APA Bulanık, M., Dağ, Z. Ö., Demiray, D., Dağ, E., et al. (2014). Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu. Journal of Turgut Ozal Medical Center, 21(4), 307-309.
AMA Bulanık M, Dağ ZÖ, Demiray D, Dağ E, Şimşek Y. Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu. J Turgut Ozal Med Cent. August 2014;21(4):307-309.
Chicago Bulanık, Murat, Zeynep Özcan Dağ, Demet Demiray, Ersel Dağ, and Yavuz Şimşek. “Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu”. Journal of Turgut Ozal Medical Center 21, no. 4 (August 2014): 307-9.
EndNote Bulanık M, Dağ ZÖ, Demiray D, Dağ E, Şimşek Y (August 1, 2014) Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu. Journal of Turgut Ozal Medical Center 21 4 307–309.
IEEE M. Bulanık, Z. Ö. Dağ, D. Demiray, E. Dağ, and Y. Şimşek, “Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu”, J Turgut Ozal Med Cent, vol. 21, no. 4, pp. 307–309, 2014.
ISNAD Bulanık, Murat et al. “Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu”. Journal of Turgut Ozal Medical Center 21/4 (August 2014), 307-309.
JAMA Bulanık M, Dağ ZÖ, Demiray D, Dağ E, Şimşek Y. Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu. J Turgut Ozal Med Cent. 2014;21:307–309.
MLA Bulanık, Murat et al. “Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu”. Journal of Turgut Ozal Medical Center, vol. 21, no. 4, 2014, pp. 307-9.
Vancouver Bulanık M, Dağ ZÖ, Demiray D, Dağ E, Şimşek Y. Gebelite Protein-S Eksikliği İle İlişkili Serebral Sinüs Trombozu: Nadir Bir Olgu Sunumu. J Turgut Ozal Med Cent. 2014;21(4):307-9.