Protein S for Portal Vein Thrombosis in Cirrhotic Patients Waiting for Liver Transplantation

Portal vein thrombus (PVT) is a challenge in liver transplantation. How PVT develops in cirrhotic patients who already have coagulopathy is unclear. This study aimed to investigate possible contributing factors to PVT in cirrhotic patients. A total of 349 cirrhotic patients who waited liver transplantation were included in this study and 48 of them had PVT. For all the patients, the mean age was 53.5 ± 9.0 year old, and 75.9% of the patients were male. There were 233 (66.8%) patients who had either hepatitis B or C. The mean Model For End-Stage Liver Disease (MELD) score was 16.4 ± 7.5. Eighteen of 48 patients with PVT and 145 of 301 patients without PVT received liver transplantation. Multivariate analysis showed that low protein S level (hazard ratio = 2.46, p = 0.017) was the only independent risk factor for PVT development. Protein S deficiency also demonstrated prognostic value on short-term survival, not only for cirrhotic patients awaiting liver transplantation (69.9% versus 84.1% at 1 year survival, p = 0.012), but also for the patients having liver transplantation (70.4% versus 84.8% at 1 year survival, p = 0.047). In conclusion, protein S level was an independent risk factor for PVT development in decompensated cirrhotic patients, and protein S deficiency was also a prognostic factor for the patients waiting for liver transplantation.

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Free Protein S Deficiency Is a Risk Factor for Venous Thrombosis

Thrombosis and Haemostasis ◽

10.1055/s-0038-1665408 ◽

1997 ◽

Vol 78 (05) ◽

pp. 1343-1346 ◽

Cited By ~ 54

Author(s):

Elena M Faioni ◽

Carla Valsecchi ◽

Alessandra Palla ◽

Emanuela Taioli ◽

Cristina Razzari ◽

...

Keyword(s):

Risk Factor ◽

Relative Risk ◽

Venous Thrombosis ◽

Protein S ◽

Unexpected Finding ◽

Reference Ranges ◽

Protein S Deficiency ◽

Men And Women ◽

Free Protein ◽

S Deficiency

SummaryA recent study suggests that protein S deficiency is not a risk factor for venous thrombosis. Since this unexpected finding would have important clinical implications if confirmed, we performed a case-control study with the aim to determine the prevalence of protein S deficiency in patients with thrombosis and in healthy individuals taken from the general population and the relative risk of thrombosis in protein S-deficient patients. Free protein S concentration was measured in 327 consecutive patients with at least one venous thrombotic episode and in 317 age- and sex-matched control individuals. Different normal reference ranges were obtained and adopted for men and women. Protein S deficiency was found in 3.1% (95% Cl: 1.5-5.2) of patients and in 1.3% of controls (95% Cl: 0.3-2.8). Ten patients and 4 control subjects had protein S deficiency, which determined a relative risk of thrombosis (sex- and age-adjusted odds ratio) of 2.4 (95% Cl: 0.8-7.9). When men and women were analyzed separately, the risk was 5.0 (95% CI: 0.6-43.6) and 1.6 (95% Cl: 0.4-6.7) respectively. PS-deficient men had more thrombotic episodes than women and later in life. Multivariate analysis established that sex was an independent determinant of the number of episodes, as was age, while PS deficiency was not. However sex and PS deficiency status were both determinants of age at first thrombotic episode.

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Portal vein thrombosis caused by protein C and protein S deficiency associated with cytomegalovirus infection

The Journal of Pediatrics ◽

10.1016/s0022-3476(95)70355-1 ◽

1995 ◽

Vol 126 (4) ◽

pp. 586-588 ◽

Cited By ~ 46

Author(s):

Ravit Arav-Boger ◽

Shimon Reif ◽

Yoram Bujanover

Keyword(s):

Portal Vein ◽

Portal Vein Thrombosis ◽

Cytomegalovirus Infection ◽

Protein C ◽

Protein S ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency

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Acquisition of Protein S Deficiency by Liver Transplantation

The American Journal of Gastroenterology ◽

10.14309/00000434-200609001-00676 ◽

2006 ◽

Vol 101 ◽

pp. S278

Author(s):

Erina N. Foster ◽

George W. Meyer ◽

Norah Terrault

Keyword(s):

Liver Transplantation ◽

Protein S ◽

Protein S Deficiency ◽

S Deficiency

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PROTEIN S DEFICIENCY WITH PORTAL VEIN THROMBOSIS : A CASE REPORT

Journal of Evidence Based Medicine and Healthcare ◽

10.18410/jebmh/2014/206 ◽

2014 ◽

Vol 1 (11) ◽

pp. 1383-1386

Author(s):

Kekathi Vidyasagar ◽

Saifullah Quraishi S M ◽

Ravi Theja ◽

Chandrashekar S

Keyword(s):

Case Report ◽

Portal Vein ◽

Portal Vein Thrombosis ◽

Protein S ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency

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Successful medical management of acute mesenteric ischemia due to superior mesenteric and portal vein thrombosis in a 27-year-old man with protein S deficiency: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-017-1463-4 ◽

2017 ◽

Vol 11 (1) ◽

Cited By ~ 1

Author(s):

N. P. Osti ◽

D. N. Sah ◽

R. S. Bhandari

Keyword(s):

Case Report ◽

Portal Vein ◽

Portal Vein Thrombosis ◽

Medical Management ◽

Mesenteric Ischemia ◽

Acute Mesenteric Ischemia ◽

Protein S ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency

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A Rare Case of Portal Vein Thrombosis due to Protein S deficiency in a patient with Decompensated Cryptogenic CLD with Portal Hypertension, Completely Recanalized by Single Oral Anticoagulant-Rivaroxaban

10.36811/ijho.2020.110007 ◽

2020 ◽

pp. 29-37

Author(s):

Richmond Ronald Gomes

Keyword(s):

Portal Hypertension ◽

Liver Disease ◽

Portal Vein ◽

Portal Vein Thrombosis ◽

Oral Anticoagulant ◽

Protein S ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency ◽

Hepatic Portal

Venous thromboembolic diseases are a group of heterogeneous diseases with different clinical forms and prognosis. Abdominal venous thrombosis may present either as Budd-Chiari syndrome (BCS) caused by hepatic vein or proximal inferior vena cava (IVC) obstruction or as an extra hepatic portal obstruction (EHPVO) caused by Portal vein thrombosis or mesenteric vein thrombosis. Portal vein thrombosis (PVT) is a rare form of venous thrombosis that affects the hepatic portal vein flow, which can lead to portal hypertension. Treatment of PVT includes anticoagulants, thrombolysis, and insertion of shunts, bypass surgery, and liver transplantation. Single anticoagulation therapy can be associated with a reduction in new thrombotic episodes. Here we experienced a 23 year old young lady with history of recent intrauterine death (IUD) diagnosed as PVT provoked by protein S deficiency with newly diagnosed decompensated cryptogenic chronic liver disease with portal hypertension. PVT was completely recanalized with single oral anticoagulant therapy rivaroxaban as initial low molecular weight heparin, enoxaparin administration caused reversible pancytopenia and there is a concern for bleeding and regular monitoring of INR with warfarin in this patient. Keywords: Portal vein thrombosis; Chronic liver disease; Protein S deficiency; Oral anticoagulant; Portal hypertension; Thrombolysis

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Portal and mesenteric vein thrombosis after portal vein embolization in a patient with protein S deficiency

Journal of Hepato-Biliary-Pancreatic Surgery ◽

10.1007/s00534-004-0905-8 ◽

2004 ◽

Vol 11 (5) ◽

pp. 338-341 ◽

Cited By ~ 7

Author(s):

Masayuki Ohkubo ◽

Masato Nagino ◽

Junichi Kamiya ◽

Toshiyuki Arai ◽

Hideki Nishio ◽

...

Keyword(s):

Portal Vein ◽

Portal Vein Embolization ◽

Protein S ◽

Protein S Deficiency ◽

Mesenteric Vein Thrombosis ◽

Mesenteric Vein ◽

Vein Thrombosis ◽

S Deficiency

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Hereditary Protein S Deficiency and Venous Thrombo-Embolism

Thrombosis and Haemostasis ◽

10.1055/s-0038-1661292 ◽

1985 ◽

Vol 53 (02) ◽

pp. 273-277 ◽

Cited By ~ 59

Author(s):

A W Broekmans ◽

R M Bertina ◽

J Reinalda-Poot ◽

L Engesser ◽

H P Muller ◽

...

Keyword(s):

Total Protein ◽

Oral Anticoagulant ◽

Anticoagulant Activity ◽

Coagulation Factor ◽

Protein S ◽

Protein S Deficiency ◽

Anticoagulant Treatment ◽

Oral Anticoagulant Treatment ◽

S Deficiency ◽

The Mean

SummaryProtein S, a vitamin K-dependent coagulation factor, is involved in the regulation of the anticoagulant activity of activated protein C.Using an immunoradiometric assay for total protein S in plasma we identified 14 patients (7 male and 7 female) in three unrelated Dutch families as fulfilling the criteria for an isolated protein S deficiency. In 9 patients who were not receiving oral anticoagulant treatment the mean total protein S antigen concentration was 0.50 ± 0.08 U/ml (± S.D.) and the calculated free protein S concentration was 0.15 ± 0.01 U/ml (± S.D.). In the five patients who were on oral anticoagulant treatment the mean total protein S antigen was 0.23 ± 0.05 U/ml (± S.D.).Seven of the 14 patients had a history of venous thromboembolism occurring at a mean age of 25 years and often without an apparent cause. Protein S deficiency is inherited as an autosomal dominant trait.

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Role of protein S deficiency in children with venous thromboembolism

Thrombosis and Haemostasis ◽

10.1160/th14-06-0533 ◽

2015 ◽

Vol 113 (02) ◽

pp. 426-433 ◽

Cited By ~ 12

Author(s):

Gili Kenet ◽

Karin Kurnik ◽

Martine Alhenc Gelas ◽

Ulrich Finckh ◽

Ralf Junker ◽

...

Keyword(s):

Risk Factor ◽

Venous Thromboembolism ◽

Purpura Fulminans ◽

Protein S ◽

High Risk Population ◽

Cerebral Veins ◽

Protein S Deficiency ◽

Family Status ◽

Paediatric Patients ◽

S Deficiency

SummaryVenous thromboembolism [TE] is a multifactorial disease, and protein S deficiency [PSD] constitutes a major risk factor. In the present study the prevalence of PSD and the clinical presentation at TE onset in a cohort of children is reported. In 367 unselected paediatric patients with TE (age 0.1–18 years) recruited between July 1996 and December 2013, a comprehensive thrombophilia screening was performed along with recording of anamnestic data. Thirty of 367 paediatric patients (8.2 %) derived from 27 families had PSD. Mean age at first TE onset was 14.5 years (range 0.1 to 18). Thrombotic locations were cerebral veins (n=8), calf vein TE (n=3) deep veins (DVT) of the leg (n=12), DVT & pulmonary embolism (n=5) and intra-cardiac veins (n=1) or purpura fulminans (n=1). PSD co-occurred with the factor 5 mutation at rs6025 or the homozygous factor 2 susceptibility variant at rs1799963 in one case each. The Heerlen polymorphism detected in five children presented with milder PSD. In 18 patients (60 %) a concomitant risk factor for TE was identified. A second TE event within primarily healthy siblings occurred in three of 27 PSD families (11.0 %). In this cohort of children with symptomatic TE, the prevalence of PSD adjusted for family status was 7.4 %. Given its clinical implication for patients and family members, thrombophilia testing should be performed and the benefit of medical or educational interventions should be evaluated in this high-risk population.

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