Skip Navigation Links.
Collapse <span class="m110 colortj mt20 fontw700">Volume 12 (2024)</span>Volume 12 (2024)
Collapse <span class="m110 colortj mt20 fontw700">Volume 11 (2023)</span>Volume 11 (2023)
Collapse <span class="m110 colortj mt20 fontw700">Volume 10 (2022)</span>Volume 10 (2022)
Collapse <span class="m110 colortj mt20 fontw700">Volume 9 (2021)</span>Volume 9 (2021)
Collapse <span class="m110 colortj mt20 fontw700">Volume 8 (2020)</span>Volume 8 (2020)
Collapse <span class="m110 colortj mt20 fontw700">Volume 7 (2019)</span>Volume 7 (2019)
Collapse <span class="m110 colortj mt20 fontw700">Volume 6 (2018)</span>Volume 6 (2018)
Collapse <span class="m110 colortj mt20 fontw700">Volume 5 (2017)</span>Volume 5 (2017)
Collapse <span class="m110 colortj mt20 fontw700">Volume 4 (2016)</span>Volume 4 (2016)
Collapse <span class="m110 colortj mt20 fontw700">Volume 3 (2015)</span>Volume 3 (2015)
Collapse <span class="m110 colortj mt20 fontw700">Volume 2 (2014)</span>Volume 2 (2014)
Collapse <span class="m110 colortj mt20 fontw700">Volume 1 (2013)</span>Volume 1 (2013)
American Journal of Medical Case Reports. 2019, 7(2), 27-28
DOI: 10.12691/AJMCR-7-2-4
Case Report

Protein S Deficiency Presenting as Stroke and Arterial Thrombosis in a Patient with Beckwith Weidemann Syndrome

Tanveer Mir1, , Anita Choudhary1, Anupam Mehar1, Harris Younas1 and Sabah Ambreen2

1Department of Internal Medicine Detroit Medical Center Wayne State University, Detroit, USA

2Hamdard Institute of Medical Sciences, Internal Medicine, Delhi, India

Pub. Date: February 24, 2019

Cite this paper

Tanveer Mir, Anita Choudhary, Anupam Mehar, Harris Younas and Sabah Ambreen. Protein S Deficiency Presenting as Stroke and Arterial Thrombosis in a Patient with Beckwith Weidemann Syndrome. American Journal of Medical Case Reports. 2019; 7(2):27-28. doi: 10.12691/AJMCR-7-2-4

Abstract

Protein S deficiency is a rare thrombophilia, autosomal dominant disorder leading to VTE especially thrombosis at an uncommon place. Arterial thrombosis and strokes are very rare presentations. We are presenting a case of 19 years old lady with Beckwith Weidemann syndrome who presented with arterial thrombosis with a stroke one month prior to thrombosis. Patient was successfully managed with intravenous heparin and thrombectomy and discharged on Apixaban for life long treatment.

Keywords

protein S deficiency, stroke, arterial thrombosis

Copyright

Creative CommonsThis work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

References

[1]  Munts AG, van Genderen PJ, Dippel DW, et al. Coagulation disorders in young adults with acute cerebral ischemia. J Neurol 1998; 245:21.
 
[2]  Douay X, Lucas C, Caron C, et al. Antithrombin, protein C and protein S levels in 127 consecutive young adults with ischemic stroke. Acta Neurol Scand 1998; 98: 124.
 
[3]  Ken-Dror G, Cooper JA, Humphries SE, et al. Free protein S level as a risk factor for coronary heart disease and stroke in a prospective cohort study of healthy United Kingdom men. Am J Epidemiol 2011; 174:958
 
[4]  Esmon CT. Protein S and protein C Biochemistry, physiology, and clinical manifestation of deficiencies. Trends Cardiovasc Med 1992; 2: 214.
 
[5]  Kenneth A Bauer, MD, et al, Uptodate.com 2019.
 
[6]  Koppelman SJ, Hackeng TM, Sixma JJ, Bouma BN. Inhibition of the intrinsic factor X activating complex by protein S: evidence for a specific binding of protein S to factor VIII. Blood 1995; 86: 1062.
 
[7]  Dahlbäck B. C4b-binding protein: a forgotten factor in thrombosis and hemostasis. Semin Thromb Hemost 2011; 37: 355.
 
[8]  Girolami A, Simioni P, Lazzaro AR, Cordiano I. Severe arterial cerebral thrombosis in a patient with protein S deficiency (moderately reduced total and markedly reduced free protein S): a family study. Thromb Haemost 1989; 61: 144.
 
[9]  Schwarz HP, Fischer M, Hopmeier P, et al. Plasma protein S deficiency in familial thrombotic disease. Blood 1984; 64:1297.
 
[10]  Comp PC, Nixon RR, Cooper MR, Esmon CT. Familial protein S deficiency is associated with recurrent thrombosis. J Clin Invest 1984; 74: 2082.
 
[11]  Gandrille S, Borgel D, Sala N, et al. Protein S deficiency: a database of mutations--summary of the first update. Thromb Haemost 2000; 84: 918.
 
[12]  Coller BS, Owen J, Jesty J, et al. Deficiency of plasma protein S, protein C, or antithrombin III and arterial thrombosis. Arteriosclerosis 1987; 7: 456.
 
[13]  Green D, Otoya J, Oriba H, Rovner R. Protein S deficiency in middle-aged women with stroke. Neurology 1992; 42: 1029.
 
[14]  Arterial thrombosis and protein S deficiency Horowitz IN1, Galvis AG, Gomperts ED. J Pediatr. 1992 Dec; 121(6): 934-7.