Budd-Chiari Syndrome Related to Factor v Leiden Mutation

Delarive, Joakim; Gonvers, Jean‐Jacques

doi:10.1111/j.1572-0241.1998.184_b.x

Cited by 7 publications

(2 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Factor V should be routinely investigated in patients with BCS, as factor V Leiden mutation is probably the cause of the procoagulable state that is responsible for many cases of "idiopathic" Budd-Chiari syndrome. 7 Moreover, factor V Leiden mutation is likely to be present in association with other prothrombotic states. 8 So there is a need to investigate other prothrombotic disorders when factor V Leiden mutation is recognized in a patient with BCS.…”

Section: Budd-chiari Syndrome With Underlying Homozygous Factor V Leimentioning

confidence: 99%

Budd-Chiari syndrome with underlying homozygous factor V Leiden and heterozygous methylenetetrahydrofolate reductase mutations

et al. 2005

View full text Add to dashboard Cite

The concurrence of either acquired or inherited thrombogenic risk factors has been observed in 26% of patients with BCS. 3 In our patient, BCS was a result of the combined effect of a hereditary clotting disorder (factor V Leiden mutation) and two acquired disorders, i.e., the myeloproliferative disease and the hypercoagulable stale due to the effect of fertility drugs. The fertility drugs used for the induction of superovulation in IVF may have promoted venous thrombosis by increasing the serum estradiol concentration in this patient with underlying thrombophilia. Although increased serum estradiol concentrations persist for only a few days before oocyte collection in stimulated IVF treatment cycles, the concentrations are five times the normal level. 4 At present, many women conceive by IVF, 5 and the number is going to increase in the near future. 2 The risk of the fertility drugs used in IVF to induce BCS in latent thrombophilia states requires further research. Testing for thrombogenic risk factors may be necessary before patients proceed to assisted conception procedures.

show abstract

Section: Budd-chiari Syndrome With Underlying Homozygous Factor V Leimentioning

confidence: 99%

Budd-Chiari syndrome with underlying homozygous factor V Leiden and heterozygous methylenetetrahydrofolate reductase mutations

et al. 2005

View full text Add to dashboard Cite

show abstract

“…Latent myeloproliferative disorders account for many cases previously thought to be idiopathic [5,7]. The factor V Leiden mutation is the most frequent cause of hereditary thrombophilia, and may be the second most common cause of thrombotic occlusion [8][9][10][11][12]. Paroxysmal nocturnal hemoglobinuria (PNH) and deficiencies of…”

Section: Introductionmentioning

confidence: 99%

Budd-Chiari syndrome

Faust

1999

Curr Treat Options Gastro

View full text Add to dashboard Cite

Many options are available to diagnose and treat patients with the Budd-Chiari syndrome who present with either thrombotic or non-thrombotic occlusion of the major hepatic veins and or vena cava. The goal of therapy is to alleviate venous obstruction and to preserve hepatic function. Low-sodium diets, diuretics, and therapeutic paracentesis are generally ineffective, except for the rare patient who presents with volume overload and incomplete hepatic venous occlusion. Anticoagulants and thrombolytics may be appropriate for selected patients with acute thrombotic venous obstruction. Percutaneous transluminal angioplasty (PTA) of hepatic venous stenoses or caval webs with or without placement of intraluminal stents yield excellent short-term results, but additional studies are warranted to assess long-term efficacy. Transjugular intrahepatic portosystemic shunts (TIPS) may be effective for patients with subacute or chronic disease and ascites refractory to sodium restriction and diuretics. Intrahepatic stents may also serve as a bridge to transplantation for selected patients presenting with fulminant hepatic failure consequent to hepatic venous occlusion. Additional studies will be necessary to assess the role of TIPS in the armamentarium of therapies for patients with the Budd-Chiari syndrome. Decompressive shunts, reconstruction of the vena cava and hepatic venous ostia, transatrial membranotomy, and dorsocranial resection of the liver with hepatoatrial anastomosis are appropriate options for patients with acute or subacute disease who are not candidates for, or fail less invasive therapies. The majority of patients benefit with improvement in liver function tests, ascites, and liver histology; however, hepatic function may deteriorate in patients with marginal reserve. Liver transplantation is reserved for patients with Budd-Chiari syndrome who present with fulminant hepatic failure or end-stage liver disease with portal hypertensive complications. Transplantation is also appropriate for patients who deteriorate after failed attempts at surgical shunting.

show abstract

Myeloid Neoplasms with an Erythroid Cell Lineage

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

View full text Add to dashboard Cite

Budd-Chiari Syndrome Related to Factor v Leiden Mutation

Cited by 7 publications

References 11 publications

Budd-Chiari syndrome with underlying homozygous factor V Leiden and heterozygous methylenetetrahydrofolate reductase mutations

Budd-Chiari syndrome with underlying homozygous factor V Leiden and heterozygous methylenetetrahydrofolate reductase mutations

Budd-Chiari syndrome

Myeloid Neoplasms with an Erythroid Cell Lineage

Contact Info

Product

Resources

About