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PubMed2001

Factor V Leiden related Budd-Chiari syndrome.

BACKGROUND: The role of factor V Leiden as a cause of Budd-Chiari syndrome has only recently been described. AIMS: To assess the specific features of factor V Leiden related Budd-Chiari syndrome. PATIENTS: Sixty three consecutive patients with hepatic vein or terminal inferior vena cava thrombosis. METHODS: Standardised chart review. RESULTS: Factor V Leiden was found in 20 patients (31% (95% CI 20-43)). In the subgroup of patients with, compared with the subgroup without, factor V Leiden, a combination of prothrombotic states was more common (70% (95% CI 50-90) v 14% (95% CI 3-24)); inferior vena cava thrombosis was more frequent (40% (95% CI 19-61) v 7% (95% CI 0-14)); and distribution of initial alanine aminotransferase values was bimodal (almost normal or extremely increased) versus unimodal (p=0.003). Factor V Leiden accounted for four of five cases of massive ischaemic necrosis (transaminases >50-fold the upper limit of normal values) (p=0.014), and also for all three cases developing during pregnancy. Patients with and without factor V Leiden did not differ with regard to mortality, portosytemic shunting, or listing for liver transplantation. Hepatocellular carcinoma developed in two patients; both had factor V Leiden and indolent obstruction of the inferior vena cava. CONCLUSIONS: In patients with Budd-Chiari syndrome, factor V Leiden (a) is common; (b) precipitates thrombosis mostly when combined with another risk factor; (c) is associated with one of two contrasting clinical pictures: indolent thrombosis-particularly of the inferior vena cava-or massive ischaemic necrosis; and (d) is a major cofactor of Budd-Chiari syndrome developing during pregnancy.

Adult↗
PubMed2003

Serious consequences of a sore throat.

Lemierre's syndrome, caused by Fusobacterium necrophorum, is a potentially fatal sequelae of a sore throat characterised by septicaemia, internal jugular vein thrombophlebitis and metastatic abscesses. The Chief Medical Officer reported in February 2001 that the incidence is increasing. Two cases seen in one year, with different presentations, are reported. The first patient presented with sepsis, jaundice, hepatic abscesses and portal vein/superior mesenteric vein thrombosis, whilst the second presented with sepsis, sore throat and internal jugular vein thrombophlebitis. Both patients were treated with antibiotics and anticoagulants with a favourable outcome.

Adult↗
PubMedDate not supplied

[Liver transplant in childhood: our experience].

From december 1984 to december of 1991, 12 children underwent on orthotopic liver transplantation (OLT): 6 had extrahepatic biliary atresia (EHBA), 2 had Byler disease, 2 hepatocellular carcinoma (HCC), 1 Alagille Syndrome and 1 had a hyperacute Wilson disease. The children, transplanted for the most part a broad, return for observation 3 months after OLT. A patient with hyperacute Wilson's Disease had 2 emergency OLTs and died of sepsis (due to Aspergillus); another with EHBA, operated for hepatoportoenterostomy, without result, died after OLT because of a ruptured aortic aneurysm. The other 10 are living with a variable follow-up between 8 months and 7 years. The post-operatory complications were present in 4 cases: in the same patient (15 months old) a hepatic artery thrombosis and then a portal vein thrombosis were observed; 3 patients had to have their biliary-digestive anastomosis redone. 7 of 10 patients had acute rejection. During the first month after OLT infection episodes were mostly due to bacteria (G-), Candida and Pneumocystis carinii (blood and intraabdominal sepsis). In the second period (1-3 months) there were viral infections, in particular CMV. An emergency transplanted patient, incompletely vaccinated, developed HBV infection. During long term follow-up (after the 3rd months from OLT) the children usually have mild infections of the respiratory and urinary tracts. After 1 year, they have a mean annual growth velocity that is between the 50th and 90th percentile. They showed a good rehabilitation. Their hospitalizations rate was reduced if compared with the period before OLT. Health, motor function and general behavior improved significantly.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2004

[Pediatric liver transplantation--experience at Schneider Children's Medical Center].

BACKGROUND: Introduction of segmental graft transplantation from living donors and split livers from cadaver donors has led to major advances in liver transplantation (LTx) in children. AIM: To report our initial experience with pediatric LTx performed at our center. METHODS: Data collection on all children undergoing LTx between the years 1996-2003 including the analysis of the graft and patient survivals and reports of complications. RESULTS: Forty LTx were performed in 38 children at the mean age of 6.2 years, including two retransplants. There were 15 whole liver allografts and 25 segmental grafts including: 12 living donor grafts, 5 splits and 8 reduced grafts from cadaver donors. At 40 months mean follow-up period, patient and graft survival were 81% and 72.5%, respectively. There was post-transplant mortality in seven cases--5 children died during the first month and two children passed away after 6 months (recurrent disease) and 14 months (metastatic tumor). Vascular complications included: one early and one late portal vein thrombosis (5%) and six cases of hepatic artery thrombosis (15%). In the latest group, 3 grafts were salvaged by thrombectomy and another 3 children underwent re-transplantation. There were two bile leaks (5%) and 6 bile duct strictures (15%). The bile-duct strictures were successfully corrected by surgery in one child and by transhepatic dilatation in another 4 children. One child remained with intrahepatic strictures in one of the two hepatic segments. CONCLUSIONS: The use of segmental liver allografts enables the performance of pediatric liver transplantation in Israel. Gathered experience and enhanced skills will ensure improved results over time.

Adolescent↗
PubMed1994

Portal vein graft in living related hepatic transplantation.

In living related hepatic transplantation, sclerotic small portal vein and thrombosis can impede standard end to end anastomosis of the graft portal vein and the recipient portal vein. We have described herein the successful use of the donor ovarian vein as the portal vein graft. It offers a safe and simple modality that can avoid unnecessary wound to the living donor.

Adolescent↗
PubMed1992

Hematological causes of venous thrombosis in young people: high incidence of myeloproliferative disorder as underlying disease in patients with splanchnic venous thrombosis.

Thrombotic events occur frequently in myeloproliferative disorders, namely polycythaemia vera and essential thrombocythaemia. Standard diagnostic criteria are designed quite stringent, so that a number of patients could be underdiagnosed. Spontaneous erythroid colonies formation from bone marrow or peripheral blood in the absence of exogenous erythropoietin is considered a reliable index of myeloproliferative disorder even at early stages. Endogenous erythroid colonies (EECs) formation was assessed in 43 patients having recently suffered from venous thrombosis prior to 45 years and without a previous diagnosis of hematological disease favouring thrombosis. A screening for coagulative abnormalities associated with thrombophilia was also carried out: in 5 patients (11.6%) a plasmatic thrombogenic defect was found (quantitative deficiency of antithrombin III, 1 case, protein C, 2 cases, protein S, 1 case, and plasminogen, 1 case). In 10 patients (2 males and 8 females) (23.2%) EECs assay was positive, allowing diagnosis of myeloproliferative disease even though 7 of them did not fulfill standard diagnostic criteria. In the other 3 patients who met the criteria for diagnosis of overt myeloproliferative disease the thrombotic event was the inaugural manifestation. In all these EECs-positive patients thrombosis involved mesenteric and portal veins (n = 4), hepatic veins (n = 3), portal vein (n = 2), mesenteric vein (n = 1). One of them was simultaneously affected from congenital protein C deficiency. Thus latent or atypical forms of myeloproliferative disease as well as the overt stages were the most frequent recognized cause of splanchnic venous thrombosis, accounting for 55% of the cases of our series. On the contrary no EECs-positive subject was found among the 25 patients with other sites of thrombosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2006

[Effectiveness of radiation therapy to the venous thrombosis between the inferior vena cava and right ventricle].

In the management of hepatocellular carcinoma (HCC), a tumor thrombus occurrence between the hepatic vein and right ventricle is life threatening. We studied the effectiveness of radiation therapy to the venous thrombosis between the inferior vena cava and right ventricle. CASE 1: A 66-year-old man who suffered from no hepatic viral infection had hepatectomy of the huge HCC (over 20 cm) and recurrence at the post dperated liver and lung. After transarterial embolization, he suffered from dispnea and was found with tumor thrombus from the left hepatic vein to right atrium. Radiation therapy to the tumor thrombus was done and dispnea disappeared. He died by pneumonia at 5 months after the radiation. CASE 2: A 74-year-old woman who had hepatecomy and RFA for multiple HCC. For the recurrence of HCC, TAE and RFA were performed. After the tumor thrombus in the inferior vena cava, mammarian cancer was found and radiation therapy was performed. She died after 4 months from lung edema, but no growth of tumor thrombus was found. CASE 3: A 79-year-old man who had TAE, hepatectomy, RFA and MCT for multiple hepatoma. After these treatments, tumor thrombus at the right ventricle was found. Although he suffered from portal tumor thrombosis, lung metastases, bone metastases and colon cancer after the radiation therapy, he is still alive at the 19 month of treatments. Radiation therapy is safe and effective for venous tumor thrombosis of HCC.

Aged↗
PubMed1997

The role of granulomatous phlebitis and thrombosis in the pathogenesis of cirrhosis and portal hypertension in sarcoidosis.

Sarcoidosis often involves the liver with mild elevation of serum enzymes and granulomas histologically. Rarely, chronic cholestasis, portal hypertension, cirrhosis, or nodular hyperplasia may be found. The pathogenesis of the portal hypertension and of the cirrhosis are not understood, in part because large samples of tissue have seldom been described. We describe the clinical and anatomic findings of four patients with sarcoid liver disease in whom the whole livers were available for examination. One patient had cirrhosis, one had diffuse nodular hyperplasia, and two had small regions of parenchymal fibrosis. The first two of these had a history of variceal bleeding and healed portal vein thrombosis. One had chronic cholestasis without cirrhosis. We suggest that the cirrhosis and focal fibrosis were caused by ischemia secondary to primary granulomatous phlebitis of portal and hepatic veins. The portal hypertension in two patients was likely secondary to portal vein thrombosis, because cirrhosis was absent at the onset of variceal bleeding.

Adult↗
PubMed2004

Propensity for hemorrhage and thrombosis in chronic myeloproliferative disorders.

Polycythemia vera (PV) and essential thrombocythemia (ET) are two myeloproliferative disorders (MPDs) with frequent thrombotic and hemorrhagic complications. Thrombosis is often the cause of mortality in PV and ET; hemorrhage occurs more commonly in idiopathic myelofibrosis patients, but is rarely fatal. Thromboses may occur in arteries or veins. Splanchnic, portal, hepatic, and splenic vein thromboses are not uncommon and thrombosis is also thought to cause placental vascular insufficiency and fetal wastage during pregnancies in MPD patients. These complications may result because of altered interactions between platelets, white blood cells, or endothelial cells, due to either altered receptor expression, receptor-ligand interactions, or signaling events. Age, leukocytosis, increased hematocrit, and a history of thrombotic events are risk factors for thrombosis. In determining a link between clonality and thrombosis using X-chromosome inactivation patterns in patients with ET, those who were polyclonal were less likely to experience thromboses. The search for hypercoagulability in these patients led to identification of changes in the expression patterns of coagulation proteins from the coagulation cascade. Mutations in factor V Leiden were examined and the incidence of mutations did not vary between normal and MPD patients. However, mutations in factor V Leiden were found to be risk factors for venous thrombotic events. Similarly, presence of a prothrombin gene mutation showed a higher risk for venous thromboembolic events. Proteolyzed thrombospondin appeared to contribute to hypercoagulability, and acquired von Willebrand factor disorder gave rise to hemorrhagic complications. These findings provide several potential reasons for thrombotic and hemorrhagic complications in MPD patients. Therefore, the best therapy for these patients is reduction of their platelet counts to less than 450,000/microL and close regulation of their hematocrits. The role of leukocytosis in bleeding or hemorrhage in this population remains to be elucidated.

Abortion, Spontaneous↗
PubMed1993

Diagnosis of portal vein thrombosis in patients with hepatocellular carcinoma: efficacy of color Doppler sonography compared with angiography.

OBJECTIVE: The aim of this study was to determine the efficacy of color Doppler sonography in the diagnosis of portal vein thrombosis in patients with primary hepatocellular carcinoma. The findings on angiography were used as the gold standard for diagnosis. SUBJECTS AND METHODS: We compared the findings on color Doppler sonograms and hepatic angiograms in 18 patients with hepatocellular carcinoma and portal vein thrombosis and in 22 patients with hepatocellular carcinoma without portal vein thrombosis. In most patients, tumor-related thrombus of the portal vein was confirmed by autopsy or surgery. The sonographic criteria for diagnosing portal vein thrombosis included nonvisualization of portal vein flow, pulsatile flow in the thrombus, arterioportal shunts, and cavernous transformation of the portal vein. RESULTS: Nonvisualization of portal vein flow was the predominant color Doppler finding in all patients with portal vein thrombosis. Other flow abnormalities overlapped the finding of nonvisualization of portal vein flow in 10 of 18 patients with portal vein thrombosis. The sensitivity of color Doppler sonography was 100% for nonvisualization of portal vein flow, 89% for detection of pulsatile flow in the thrombus, 60% for detection of arterioportal shunts, and 100% for detection of cavernous transformation. Among these findings, pulsatile flow in the thrombus was diagnostic for pathologically proved neoplastic thrombi in the main portal vein; sensitivity and specificity were 89% and 100%, respectively, and accuracy was 96%. No portal vein that appeared normal on color Doppler sonograms was thrombosed on arteriograms. CONCLUSION: We conclude that color Doppler sonography is a useful means of imaging the vascular extension of a tumor-based thrombus and an accurate means of screening for portal vein thrombosis.

Angiography↗
PubMed2006

[Hyperbaric oxygen therapy in rats submitted to hepatic veins ligation: mortality valuation and histological study of liver and spleen].

PURPOSE: To evaluate the effects of hyperbaric oxygen therapy in rats submitted to instantaneously hepatic vein obstruction. METHODS: 30 Holtzman adult male rats were utilised, distributed into two groups: 1) hepatic vein obstruction; 2) hepatic vein obstruction associated with hyperbaric oxygen therapy. General anaesthesia was utilized by a solution composed of ketamine chloride (40 mg/ml) and meperidine chloride (10 mg/ml) in a dose of 50/mg/weight, applied into the right gluteus muscle. The animals belonged to group 2 were submitted to hyperbaric oxygen therapy, 8 hours after the operations, in a 2,5 atmosphere, which lasts 120 minutes per day, in consecutive 20 days. The statistical analysis was made in relation to mortality and histological study of livers and spleens utilizing the Fisher test, and the results were considered statistically significant when p < 0.05. RESULTS: Occurred seven (46.67%) deaths between animals belonged to group 1 and no deaths in the animals belonged to group 2. The histological studies made in the livers and spleens of the animals belonged to group 1 showed many alterations in the following percentages: thrombosis of hepatic, portal and center-lobular veins in five (33.3%), very extensive necrosis of liver cells in seven (46.7%), and light in eight (53.3%), Kupffer cells developed and hypertrophied in 14 (93.3%), high congestion of the spleen purple in six (40.0%) and moderate and severe hemossiderinosis spleen in 14 (93.3%). The analysis of this parameters in the group 2 only showed light necrosis of liver cells, Kupffer cells light developed and hypertrophied, moderated congestion of the spleen purple and light hemossiderinosis spleen. All these parameters analysed showed significantly difference (p < 0.05) between these two groups. CONCLUSIONS: It could be concluded that the hyperbaric oxygen therapy applied in rats, with instantaneously hepatic vein obstruction decreased their post-surgical mortality and their early deleterious effects in the liver and spleen.

Animals↗
PubMed1998

Noncirrhotic portal hypertension and nodular regenerative hyperplasia of the liver in dogs with mucopolysaccharidosis type I.

Nodular regenerative hyperplasia (NRH) is an uncommon hepatic lesion often associated with noncirrhotic portal hypertension (PHT). We have noted that NRH and PHT are frequent occurrences in a colony of dogs with the genetic storage disease, mucopolysaccharidosis I (MPS-I). This observation provides the opportunity to study the histology and pathogenesis of NRH and noncirrhotic PHT in a new animal model. Thirteen of 32 dogs (41%) with MPS-I developed multiple portocaval shunts between 4 and 48 months of age that were grossly visible at necropsy. Seven of the 13 developed marked ascites, whereas all those without shunts and littermates (n = 24) heterozygous for the mutated alpha-L-iduronidase allele (carriers unaffected by the storage disease) did not. The large and medium-sized portal veins were widely patent without thrombosis or vascular malformations. Hepatic parenchymal fibrosis was absent or mild and did not correlate with shunt formation. All 32 livers had varying degrees of diffuse periportal hepatocellular hyperplasia with multifocal atrophy and compression of centrolobular cords (NRH) most prominent in dogs with shunts. Many small portal veins were reduced in diameter or absent, especially in animals with shunts. Noncirrhotic PHT and NRH appear to be related to the obliteration of small portal veins in these dogs, but the pathogenesis of this vascular change remains unknown.

Animals↗
PubMed1988

[Venous thrombosis in Behçet's disease].

Venous lesions in Behçet's disease (BD) were defined by Adamantiades and represent one of the most suggestive signs of the disease. They are occasionally the first sign of the disease and are frequently the basis for the diagnosis in a case of recurrent thrombosis in a young subject, the preferential context of BD. Involvement of superficial vessels is virtually constant. Venous vasculitis is responsible for non-specific hypersensitivity and erythema nodosa, which constitute some of the major diagnostic criteria. Ocular periphlebitis is one of the elements responsible for posterior uveitis. The originality of the venous involvement is due to the involvement of deep territories. Any vein may be affected, but the remarkable features are the size of the thrombosed vessels: superior and inferior vena cava, iliofemoral veins and the unusual site of the involvement: supra-hepatic veins, cerebral vessels, etc. Inferior vena cava thrombosis may be associated with aneurysms of the pulmonary arteries in the context of Hughes-Stovin syndrome. Cerebral phlebitis, which can now be identified more easily by means of digital angiography, is responsible for a typical picture: headaches, bilateral papilloedema and raised CSF pressure. The classical pictures of optic chiasmatic arachnoiditis and so-called benign intracranial hypertension actually correspond to unrecognised phlebitis. They may also be associated with other neurological lesions. In one half of cases, phlebitis cutaneous manifestations. However, they may precede the diagnostic signs or may occur very late in the course of the disease. They are recurrent and affect a number of different territories.(ABSTRACT TRUNCATED AT 250 WORDS)

Anti-Inflammatory Agents↗
PubMed2000

Recent portal or mesenteric venous thrombosis: increased recognition and frequent recanalization on anticoagulant therapy.

Characteristics and outcomes of recent portal or mesenteric venous thrombosis are ill-known. We intended to compare these features with those of patients with portal cavernoma, and also to assess the incidence of recanalization of recent thrombosis on anticoagulation therapy. All patients seen between 1983 and 1999 were enrolled into this retrospective study if recent portal or mesenteric venous thrombosis or portal cavernoma had been documented, and if cancer of the liver, pancreas, or bile duct, intrahepatic block including cirrhosis, and obstruction of the hepatic veins had been ruled out. The proportion of recent thrombosis was 7% in patients seen before 1990 and 56% after 1994 (P <.05). Patients with recent thrombosis (n = 33) or cavernoma (n = 108) did not differ with regard to age, sex ratio, or prevalence of prothrombotic states and of previous thrombotic events. In patients with recent thrombosis, septic pylephlebitis was more common and the incidence of gastrointestinal bleeding was lower (2.4 vs. 12.7/100 patient-years). Recanalization occurred in 25 of 27 patients given anticoagulation and 0 of 2 patients not given anticoagulation. The probability of recanalization was related to the extent of thrombosis (P =.003). In conclusion, mesenteric or portal venous thrombosis is increasingly recognized at an early stage. The features differentiating recent thrombosis and cavernoma are related to silent onset precluding early recognition and therapy in the latter. Frequent association with prothrombotic states and frequent recanalization on anticoagulation support the recommendation of early anticoagulation therapy in all patients with recent portal vein thrombosis.

Adult↗
PubMed1965

THROMBOPHLEBITIS.

Explore the source record for details and available documents.

Anticoagulants↗
PubMedDate not supplied

Surgical complications and long-term outcome in pediatric liver transplantation.

BACKGROUND/AIMS: Liver transplantation has been widely accepted for the treatment of children with end-stage liver disease over the last 10 years particularly with the advent of reduced-size liver transplant technique. This study reviewed the perioperative and long-term results in the pediatric program of the Queensland Liver Transplant Service, Brisbane, Australia. METHODOLOGY: Retrospective analysis was performed in 153 children who received 176 liver grafts between 1985 and 1995, including 109 (62%) reduced-size and 67 (38%) whole liver grafts. Median follow-up period was 5.3 years. RESULTS: One-, 5-, and 10-year patient and graft survival rates were 82% and 74%, 75% and 63%, and 70% and 60%, respectively. Normal physical and intellectual development was observed in 98% of survivors. There were no significant differences in patient or graft survival rates between transplants using reduced-size and whole liver grafts. Portal vein thrombosis was the most common vascular complication, occurring in 8%. Hepatic artery thrombosis occurred in 7%, including 11% of children less than 1 year old and 8% of those under 10 kg. Biliary complication was found in 16% and posttransplant gastrointestinal perforation in 19%. CONCLUSIONS: Liver transplantation has the potential to cure and allow development in children with end-stage liver disease.

Adolescent↗