Portal vein thrombosis with protein C-S deficiency in a non-cirrhotic patient

Deshpande, Sheetal; Patil, Smita; Pendse, Manish; Kashyap, Prashant; Dave, Dhaval

doi:10.18203/2320-6012.ijrms20163818

Cited by 4 publications

(4 citation statements)

References 6 publications

(8 reference statements)

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“…In patients with venous thromboembolism, the prevalence of deficiency of protein-C and protein-S was 2-5%. [4][5][6][7] Here, we report a case of a women who is in her twenties, already had a history of deep vein thrombosis, and yet again diagnosed with chronic abdominal venous thrombosis including portal vein, bilateral iliofemoral vein and inferior venacava.…”

Section: Discussionmentioning

confidence: 95%

Recurrent abdominal and lower limb venous thrombosis due to inherent protein-C and protein-S deficiency

Polavarapu¹,

Narayanasamy²,

Subramanian³

2019

Int J Res Med Sci

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Protein-C and protein-S deficiency is associated with a hypercoagulable state which usually presents with recurrent venous thrombosis as a common complication. But extensive thrombosis involving all the major abdominal and lower limb veins is quite rare. Here, we report a case of a 27-year-old woman presented with engorged veins all over abdomen and chest since 20 days. Clinical examination revealed signs suggestive of portal venous hypertension with moderate splenomegaly. Protein-C and protein-S levels were found to be low. Portal venous doppler ultrasound and CT-venogram revealed chronic thrombosis of portal vein, inferior vena cava, bilateral iliac and femoral veins with extensive collaterals formation and partial thrombosis in collaterals as well. The patient was successfully managed with anti-coagulating agent (acenocoumarol) and has been maintained on regular follow-up to avoid reoccurrence of thrombosis.

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Section: Discussionmentioning

confidence: 95%

Recurrent abdominal and lower limb venous thrombosis due to inherent protein-C and protein-S deficiency

Polavarapu¹,

Narayanasamy²,

Subramanian³

2019

Int J Res Med Sci

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“…In the literature, Deshpande et al, reported protein C-S deficiency in a non-cirrhotic 24-year-old male who debuted with acute superior mesenteric and PVT in which protein S activity was 38% (65%-140%); the patient was successfully treated with anticoagulation and no surgery intervention was needed (9) . Rodríguez-Leal et al also identified protein C-S deficiency in a 63 non-cirrhotic patient who developed PVT and was treated successfully with enoxaparine and warfarin (10) .…”

Section: Discussionmentioning

confidence: 99%

Laparoscopic splenectomy and proximal splenorenal shunt for portal hypertension due to portal vein thrombosis in a patient with protein S deficiency

Gómez

Pozo²,

Chávez

et al. 2021

Rev Gastroenterol Peru

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Portal vein thrombosis (PVT) is a rare condition in the general population that develops serious complications if left untreated for long time. We present a case of a 29-year-old woman who developed PVT due to protein S deficiency versus neonatal funiculitis. Over time, the patient developed upper gastrointestinal bleeding due to esophageal varices and hypersplenism with splenic sequestration that caused minor bleeding episodes. Laparoscopic splenectomy and proximal splenorenal shunt with distal pancreatectomy due to aneurysmal dilatations of the splenic artery were successfully performed to avoid mayor progression of portal hypertension. Patient was discharged with indefinite anticoagulation and after surgery platelets raised 3 3 ectomy and proximal splenorenal shunt for portal hypertension due to portal vein ombosis is an adequate surgery procedure which should be applied in these medical cases.

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“…Thrombosis of portal vein can reduce blood supply to the liver and cause portal hypertension [8]. In children, the most common etiology of portal vein thrombosis is intra-abdominal infections and history of umbilical vein catheterization in the neonatal period; other less common causes include inherited or acquired thrombophilic states such as mutation of prothrombin or factor V Leiden; deficiency of protein C, protein S, or antithrombin III; or antiphospholipid syndrome [3].…”

Section: Discussionmentioning

confidence: 99%

“…Anticoagulation therapy is necessary for patients with inherited coagulation disorders. In complicated cases, shunt surgery or Transjugular Intrahepatic Portosystemic Shunt (TIPS) procedure is used [8]. In Mexico, Majluf-cruz et al studied 36 patients with portal hypertension and non-cirrhotic portal vein thrombosis.…”

Section: Discussionmentioning

confidence: 99%

Tense Ascites as a Presentation of Protein S Deficiency in a 9-Year-Old Boy

Asgarshirazi

Ghafarypour

Aghighi

et al. 2019

crcp

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Ascites is not a usual finding in prehepatic portal hypertension, including portal vein thrombosis, but when portal vein thrombosis is acute and massive, ascites can be a presenting feature. We report a 9-year-old boy with tense ascites and portal and superior mesenteric vein thrombosis. A 9-year-old boy was evaluated for tense ascites which led to umbilical hernia since one month before admission. He did not have any clinical or laboratory stigmata of parenchymal liver disease. Imaging studies showed superior mesenteric and portal vein thrombosis. In laboratory tests for pre-thrombotic states, he suffered from significant protein S deficiency. Thrombophilic states like protein S deficiency predispose patient to vascular thrombosis. This vascular thrombosis can be present with signs and symptoms related to their territories. Prevention of thrombosis and rethrombosis with anticoagulant therapy is recommended.

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Portal vein thrombosis with protein C-S deficiency in a non-cirrhotic patient

Cited by 4 publications

References 6 publications

Recurrent abdominal and lower limb venous thrombosis due to inherent protein-C and protein-S deficiency

Recurrent abdominal and lower limb venous thrombosis due to inherent protein-C and protein-S deficiency

Laparoscopic splenectomy and proximal splenorenal shunt for portal hypertension due to portal vein thrombosis in a patient with protein S deficiency

Tense Ascites as a Presentation of Protein S Deficiency in a 9-Year-Old Boy

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