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Results for “HEPATIC VEIN THROMBOSIS”

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At least 217 records · Page 12Linked to original sources

PubMed1994

Antiphospholipid syndrome as the second cause of non-tumorous Budd-Chiari syndrome.

In a prospective study of 22 patients with non-tumorous Budd-Chiari syndrome, four were found to have the antiphospholipid syndrome with no other cause of hepatic vein thrombosis. All four patients were young women. The antiphospholipid syndrome was secondary to systemic lupus in one case, to a "lupus-like disease" in another, and apparently primary in the remaining two cases. Two patients died. The other two are in good health on chronic oral anticoagulation. In our experience, the antiphospholipid syndrome is a frequent cause, after myeloproliferative disorders, of non-tumorous Budd-Chiari syndrome. In such patients, long-term anticoagulation may prevent recurrence or extension of thrombosis.

Adolescent↗

PubMed2002

Domino as a bridge to definitive liver transplantation in a neonate.

An interim liver transplant was used to extend survival in a neonate. This was accomplished by the initial transplant of a left-lateral segment of a metabolically abnormal liver obtained from a 7-yr-old patient with primary oxalosis. This bridging strategy was required because our neonatal patient was dying of fulminant hepatic failure caused by hepatic vein thrombosis and a small liver or liver segment could not be found. Although problems with hyperoxaluria were encountered in the neonate post-transplant, the interim liver transplant enabled the baby to survive and grow until the age of 4 months. At that time, a definitive transplant was performed using the left-lateral segment of his mother's liver. This case represents the first reported use of a pediatric domino transplant where a metabolically abnormal liver was used to allow sufficient growth to permit a definitive liver transplantation.

Child↗

PubMed1980

A distinctive perivenular hepatic lesion associated with heart failure.

Hepatic morphologic changes in seven patients in heart failure who were initially thought to have hepatitis are reported. In each instance, percutaneous liver biopsy or post mortem tissue examination disclosed a striking lesion involving all perivenular areas, with replacement of hepatocytes by erythrocytes. Four patients were in pure left-heart failure, while three were in biventricular failure. Upon treatment of the heart failure, the conditions of all patients improved clinically, and liver-function tests returned to normal or near normal. In two patients who later had refractory heart failure and died, there was no evidence of hepatic-vein thrombosis or occlusion post-mortem. A hypothesis is offered for the pathogenesis of this lesion, and its clinico-pathologic differential is discussed.

Biopsy↗

PubMed1987

Polycythaemia vera in young people: an analysis of 58 cases diagnosed before 40 years.

Over 20 years, 58 cases of PV in young people (46 meeting the full PVSG criteria, 12 with elevated red cell volume and leucocytosis or thrombocytosis, without splenomegaly) were studied and have been followed for periods of 3-24 years. These cases represent approximately 5% of the cases of PV referred to the Department of Nuclear Medicine of St Louis Hospital during this period. They differ from older patients in the initial clinical severity, the short interval between the first symptoms and the diagnosis, frequent presentation with a life-threatening complication (two cases of hepatic vein thrombosis, six thrombotic or haemorrhagic events, six splenectomies, two abortions) and a very enlarged spleen in half the cases. However, after the initial complications, the overall survival is very long (exceeding 70%, even when including the initial complications, at 15 years). The vascular accidents occur exclusively in the phlebotomized patients, the main risk factor being the poor stability of the haematocrit. Only one acute leukaemia was observed among the 14 cases treated by radioactive phosphorus and/or alkylating chemotherapy. The most frequent late complication was evolution towards myelofibrosis. This spent phase seemed to occur earlier in patients treated by phlebotomy. On the basis of this data, we would advise the following therapeutic strategy: phlebotomies, as soon as the diagnosis is established, and a systematic long-term treatment by hydroxyurea with the hope of reducing the number of vascular complications and of delaying the evolution towards the spent phase and the myelofibrosis.

Adult↗

PubMed2004

Acute Budd-Chiari syndrome with liver failure: the experience of a policy of initial interventional radiological treatment using transjugular intrahepatic portosystemic shunt.

BACKGROUND AND AIMS: The optimal management of acute Budd-Chiari syndrome (BCS) with liver failure is controversial. Options include anticoagulation, portal systemic shunting or liver transplantation. In recent years, transjugular intrahepatic portosystemic shunts (TIPS) have been tried in place of shunt surgery. We report our experience with a policy of initial interventional radiological treatment (TIPS) in this patient group. METHODS: A consecutive, non-selected series of five patients with acute BCS with liver failure presenting to our liver unit over a 36-month period from October 2000 were treated with a policy of initial attempt at transjugular shunt. RESULTS: Stents were successfully inserted in four patients. In one patient the hepatic vein remnant could not be cannulated. One of the four stented patients had a hepatic venous web. Rapid recurrent portal and hepatic vein thrombosis occurred in one patient despite anticoagulation and trans-shunt thrombectomy. This patient and the patient with failed stenting underwent successful liver transplantation. One successfully stented patient died unexpectedly at home 2 weeks after TIPS insertion. Four of five patients are alive (80%) at a mean follow-up time of 30 months. CONCLUSION: Initial TIPS, with liver transplantation for treatment failures, may be a reasonable, cost-effective and surgery-sparing treatment for acute Budd-Chiari presenting with liver failure.

Acute Disease↗

PubMed1995

Identification of latent myeloproliferative disease in patients with Budd-Chiari syndrome using X-chromosome inactivation patterns and in vitro erythroid colony formation.

Some patients with an early or latent myeloproliferative disorder (MPD) present with Budd-Chiari syndrome (BCS, hepatic vein thrombosis). Cell culture analysis of erythroid progenitors (BFU-E) can be used to discriminate primary from secondary MPD and examination of X-chromosome inactivation (in females) can be used to demonstrate clonality in neoplastic tissues. The present study used these techniques to examine whether a group of 7 female patients who presented with BCS had evidence to support a diagnosis of MPD. Unilateral X-inactivation and therefore clonality can be studied in females heterozygous for X-linked restriction fragment length polymorphisms (RFLP) by differences in methylation between active and inactive chromosomes. Probes for two polymorphic loci, phosphoglycerate kinase (PGK, at Xq13.3 [BstX1 RFLP]) and M27 beta (an anonymous locus DXS255 at Xp11.22 [Pst1 RFLP]) were used to study methylation patterns. All 7 patients were heterozygous using M27 beta and 2/7 were also heterozygous using the PGK probe. Polyclonal patterns of X-inactivation in granulocytes were demonstrated in 3/7, a skewed/monoclonal pattern in 1/7 and aberrant patterns in 3/7 using M27 beta. Two patients who had aberrant patterns of X inactivation with M27 beta demonstrated a skewed/monoclonal pattern with PGK. The results of BFU-E growth patterns and clonality were entirely concordant in 5/6 patients. Thus X-chromosome inactivation patterns, in conjunction with erythroid colony studies, can be used to assist in the diagnosis of an underlying MPD in BCS.

Biomarkers↗

PubMed2006

Elevated serum erythropoietin levels in patients with Budd-Chiari syndrome secondary to polycythemia vera: clinical implications for the role of JAK2 mutation analysis.

PURPOSE: It is widely accepted that an increased serum endogenous erythropoietin (Epo) level in a patient presenting with an elevated red cell mass makes a diagnosis of clonal polycythemia vera (PV) extremely unlikely. However, until the recent description of the constitutively activating V617F point mutation of the Janus 2 tyrosine kinase (JAK2)--a high-frequency molecular marker that is extremely specific for clonal chronic myeloproliferative disorders--distinction of PV from secondary erythrocytosis or other conditions has often been difficult. The purpose of this study was to use JAK2 V617F analysis to re-evaluate the validity of elevated Epo levels as a PV-exclusion criterion in patients with hepatic vein thrombosis [Budd-Chiari syndrome (BCS)]. PATIENTS AND METHODS: We reviewed clinical data from 30 patients at our institution who presented with erythrocytosis and BCS. We isolated myeloid cells from fresh or archival bone marrow samples from four patients with BCS and an elevated serum Epo level, and analyzed them for the presence of the JAK2 V617F mutation. RESULTS: All four samples were positive for JAK2 V617F, confirming the presence of a clonal hematopoietic disorder consistent with PV. CONCLUSION: In the presence of BCS, elevated serum Epo levels do not exclude the diagnosis of PV.

Adult↗

PubMed2002

Coexistence of homozygous factor V Leiden mutation and antiphospholipid antibodies in two patients presented with Budd-Chiari syndrome.

Two female patients are reported, who presented with Budd-Chiari syndrome (hepatic vein thrombosis), and were found to have both, antiphospholipid antibodies and homozygous factor V Leiden mutation. Both patients also had recurrent fetal losses, as well as splenic and portal vein thrombosis. The coexistence of homozygous factor V Leiden mutation and antiphospholipid antibodies in patients with Budd-Chiari syndrome is extremely rare. The interaction of antiphospholipid antibodies and factor V Leiden mutation in the pathogenesis of antiphospholipid syndrome and their contribution to Budd-Chiari syndrome are discussed.

Abortion, Habitual↗

PubMed1975

[Study of 187 portacaval anastomoses. Statistical analysis of prognostic factors. II. Comparative study of the results of anastomoses with and without interruption of the portal trunk. Discussion].

The authors report a comparative retrospective study of 124 end-to-side portal anastomoses and 63 side-to-side anastomoses. The influence of a certain number of clinical and pathological factors on mortality and immediate and long-term survival, were statistically verified. The quality of the functional result result was analysed in each group of operated patients. Comparison of the results of trunkular anastomoses with or without interruption of the portal vein, showed both as regards, post-operative and late mortality, that the functional results were definitely in favor of end-to-side anastomosis. Thus side-to-side anastomoses gave 40,4 p. 100 of good results and 59,6 average or poor results, as against 60 p. 100 gave rise to portal systemic encephalopathy in 17 p. 100 of cases as against 35 p. 100 in the case of the side-to-side anastomosis. Only the sub-group of operated patients with hepatic vein thrombosis seemed to benefit from side-to-side anastomosis.

Brain Diseases↗

PubMed1954