Protein S Deficiency Presenting as an Acute Postoperative Arterial Thrombosis in a Four-Year-Old Child

1999 ◽  
Vol 88 (3) ◽  
pp. 535-537
Author(s):  
A. Andrew Zimmerman ◽  
R. Scott Watson ◽  
Joseph K. Williams
1989 ◽  
Vol 61 (01) ◽  
pp. 144-147 ◽  
Author(s):  
A Girolami ◽  
P Simioni ◽  
A R Lazzaro ◽  
I Cordiano

SummaryDeficiency of protein S has been associated with an increased risk of thrombotic disease as already shown for protein C deficiency. Deficiencies of any of these two proteins predispose to venous thrombosis but have been only rarely associated with arterial thrombosis.In this study we describe a case of severe cerebral arterial thrombosis in a 44-year old woman with protein S deficiency. The defect was characterized by moderately reduced levels of total and markedly reduced levels of free protein S. C4b-bp level was normal. Protein C, AT III and routine coagulation tests were within the normal limits.In her family two other members showed the same defect. All the affected members had venous thrombotic manifestations, two of them at a relatively young age. No other risk factors for thrombotic episodes were present in the family members. The patient reported was treated with ASA and dipyridamole and so far there were no relapses.


1989 ◽  
Vol 62 (03) ◽  
pp. 1040-1040 ◽  
Author(s):  
P Sié ◽  
B Boneu ◽  
R Biermé ◽  
M L Wiesel ◽  
L Grunebaum ◽  
...  

2014 ◽  
Vol 6 (2) ◽  
pp. 175-179
Author(s):  
AK Choudhury ◽  
M Khalequzzaman ◽  
S Hasem ◽  
M Akhtaruzzaman ◽  
S Jannat

Stent thrombosis (ST) is one of the major complications that occur in percutaneous coronary interventions (PCIs) with stents. Various factors have been attributed to the development of ST, and several strategies have been recommended for its management. Protein C or protein S deficiencies may uncommonly be responsible for coronary arterial thrombosis. We report a young woman with recurrent stent thrombosis due to the deficiency of protein S. After coronary stenting, stent thrombosis occurred two times despite aggressive medical therapy. This report suggests that the deficiency of protein C or S should be born in mind in a young patient with recurrent thrombotic events, and that anticoagulants in addition to antiplatelet agents considered in the presence of their deficiency DOI: http://dx.doi.org/10.3329/cardio.v6i2.18364 Cardiovasc. j. 2014; 6(2): 175-179


1999 ◽  
Vol 88 (3) ◽  
pp. 535-537 ◽  
Author(s):  
A. Andrew Zimmerman ◽  
R. Scott Watson ◽  
Joseph K. Williams

1990 ◽  
Vol 58 (5) ◽  
pp. 461-468 ◽  
Author(s):  
Marie-Louise Wiesel ◽  
Jean-Luc Charmantier ◽  
Jean-Marie Freyssinet ◽  
Lélia Grunebaum ◽  
Simone Schuhler ◽  
...  

2017 ◽  
Vol 5 (1) ◽  
pp. 5
Author(s):  
Wei-Sheng Liao ◽  
Wei-Tsung Wu ◽  
Nai-Yu Chi ◽  
Wen-Hsien Lee ◽  
Chun-Yuan Chu ◽  
...  

Protein S deficiency is an inherited thrombophilia associated with an increased risk of venous thromboembolism. However, arterial thrombosis is a relative rare complication of protein S deficiency and the prognosis of these patients was worse than those without protein S deficiency in the literature. Herein we reported a 43-year-old male with protein S deficiency experiencing several times acute peripheral arterial thrombosis of left leg. Surgical thrombectomy was performed initially but later endovascular treatment (EVT) was suggested. Although EVT was successfully performed by catheter-directed thrombolysis (CDT), arterial thrombosis still recurred three months later. CDT was tried again but thrombosis could not be treated by this strategy anymore. Therefore, we used mechanical thrombectomy device (Rotarex system) and successfully regained the straight-line blood flow to the foot after the procedure. Peripheral echo showed patent flow after 6 months follow-up. In conclusion, arterial thrombosis is a relative rare complication of protein S deficiency and prognosis was not well in the literature, our case reminds physicians that Rotarex system is a safe and highly efficient device for acute PAOD even in the patients with hypercoagulable state.


1992 ◽  
Vol 121 (6) ◽  
pp. 934-937 ◽  
Author(s):  
Ira N. Horowitz ◽  
Antonio G. Galvis ◽  
Edward D. Gomperts

1998 ◽  
Vol 79 (04) ◽  
pp. 802-807 ◽  
Author(s):  
Bengt Zöller ◽  
Pablo García de Frutos ◽  
Björn Dahlbäck

SummaryReduced fibrinolytic capacity due to increased plasminogen activator inhibitor-1 (PAI-1) activity in plasma is a common finding in patients with coronary heart disease or venous thromboembolism, although its clinical significance is debated. Recently, a dimorphism in the PAI-1 promoter (4G-5G) has been reported and homozygosity for the 4G allele is associated with increased transcription and higher PAI-1 levels. Homozygous 4G genotype has been suggested to be a risk factor for myocardial infarction. In the present study, the 4G-5G dimorphism was determined in 349 individuals from 21 thrombophilic families with hereditary protein S deficiency and in 140 unrelated healthy controls. Among the 143 protein S deficient individuals, there was no relationship between deep or superficial venous thrombosis and the PAI-1 dimorphism. However, 26% (12/46) of individuals having protein S deficiency combined with homozygosity for the 4G allele had suffered pulmonary embolism as compared to 7% (7/97) of protein S deficient individuals carrying at least one 5G allele (p = 0.0019). In protein S deficient individuals, arterial thrombosis was found to be associated with smoking and 4G homozygosity. No association was found between the PAI-1 dimorphism and arterial or venous thromboembolism in family members without protein S deficiency. In conclusion, the PAI-1 genotype affects the phenotypic expression of thrombophilia in protein S deficient individuals.


1990 ◽  
Vol 64 (02) ◽  
pp. 206-210 ◽  
Author(s):  
C F Allaart ◽  
D C Aronson ◽  
Th Ruys ◽  
F R Rosendaal ◽  
J H van Bockel ◽  
...  

SummaryProtein S is the vitamin K dependent cofactor of activated protein C. It has an important role in the regulation of blood coagulation and fibrinolysis. Hereditary protein S deficiency is associated with familial venous thrombophilia. Since a few patients with arterial occlusions have been reported to be protein S deficient, it is speculated that hereditary protein S deficiency may be also a risk factor for the development of arterial thrombosis. In a group of 37 consecutive patients with arterial occlusive disease presenting before the age of 45, three patients were found heterozygous for hereditary protein S deficiency. None of the patients had a protein C deficiency or an antithrombin III deficiency. Family investigations showed a clear association between the hereditary deficiency and venous thrombosis, but a relation between the deficiency and arterial thrombosis was less obvious. A review of previous literature on patients with arterial thrombosis and protein S deficiency revealed that more extensive studies are needed to demonstrate whether or not hereditary protein S deficiency is a risk factor for the development of arterial thrombosis.


Lupus ◽  
2019 ◽  
Vol 28 (7) ◽  
pp. 903-905 ◽  
Author(s):  
A Plana-Pla ◽  
I Bielsa Marsol ◽  
C Ferrandiz Foraster

Protein S deficiency is rare in systemic lupus erythematosus (SLE) and is generally associated with the presence of antiphospholipid (APL) antibodies. Lack of protein S can cause skin necrosis, but when it does it is generally in response to warfarin exposure. In this article, we describe the case of a patient who had not received warfarin and without APL antibodies who developed extensive skin necrosis due to protein S deficiency. It is important to investigate protein S deficiency in patients with lupus and extensive skin ulcers as it is a sign of arterial thrombosis and venous thromboembolism.


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