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PubMed2005

Early postoperative hepatic sonography as a predictor of vascular and biliary complications in adult orthotopic liver transplant patients.

OBJECTIVE: Our objective was to quantitatively assess the value of early posttransplantation hepatic artery resistive indexes in predicting vascular and nonvascular complications in adult orthotopic liver transplant (OLT) patients. MATERIALS AND METHODS: Between 1999 and 2001, 110 consecutive adults received grafts. Doppler sonographic graft evaluations measured main, right, and left resistive indexes within 24 to 48 hr after surgery (normal resistive index cutoff, 0.6). Clinical, operative, procedural, and radiologic reports were reviewed for vascular and biliary complications. Frequency, Student's t test, logistic, and regression statistical analyses were performed. RESULTS: even patients (6.4%) had vascular complications, including two (1.8%) hepatic artery and two (1.8%) hepatic vein stenoses, one (0.9%) hepatic vein thrombosis, two (1.8%) portal vein thromboses, and one (0.9%) thrombosis and two (1.8%) stenoses of the inferior vena cava (IVC). In 19 patients (17.3%), biliary complications included anastomotic strictures and leaks 1 week to 18 months after transplantation. In 11 patients (10%), sonographically large hematomas required surgical evacuation. In grafts with vascular complications or large hematomas, the mean early posttransplant main, right, and left indexes were significantly lower (< or = 0.6) than without these complications (p < 0.01). In grafts with and without biliary complications, mean early posttransplant main, right, and left indexes did not differ significantly. CONCLUSION: In adult OLT patients, low early posttransplant hepatic artery resistive indexes were sensitive (100%) and specific (80%) predictors for vascular complications (e.g., hepatic artery, portal vein, hepatic vein, and IVC) but not for biliary complications. All patients with indexes less than 0.6 within 24-48 hr after surgery should be monitored closely for vascular complications.

Adolescent↗
PubMed2005

Orthotopic liver transplantation for Byler's disease.

In this study we analyzed the features of 12 patients who underwent liver transplantation for progressive familial intrahepatic cholestasis (Byler's disease [BD]) in view of the technical features of the OLTx, incidence and type of complications, need for retransplantation, as well as patient and graft survivals. BD was the indication in 12 patients of median age 1.32 years and median weight 10 kg. Median follow-up was 670 days. Major surgical complications requiring reintervention occurred in three patients. No thrombosis of the hepatic artery was observed. Infections with positive blood cultures were diagnosed in four patients. One patient had a biliary anastomotic stenosis successfully treated by percutaneous techniques. Four patients had episodes of acute rejection treated with steroids. Two patients were retransplanted, both of whom died in the early postoperative period due to hepatic vein thrombosis and venoenteric fistula. The actuarial patient and graft survival was 83% at 1 year and 83% at 5 years. Split-liver grafts represent an excellent organ supply for these patients, achieving good results with no mortality on the waiting list.

Adolescent↗
PubMed1988

[Budd-Chiari syndrome and Behçet's disease. A case treated by mesenterico-atrial prosthesis].

A 36 year-old North African man, with Behçet's syndrome complicated by an inferior vena caval thrombosis, developed a chronic Budd-Chiari syndrome associated with bleeding esophageal varices. He was treated by an emergency mesoatrial shunt. Results at 2 years were good. Analysis of this case and the 13 other similar cases with associated Budd-Chiari syndrome and Behçet's syndrome found in the literature showed that hepatic veins thrombosis: a) is often due to inferior vena caval thrombosis or membranous obstruction; b) has a high spontaneous mortality rate by acute liver failure; c) remains a potential indication for porto-systemic shunt, as are other causes of Budd-Chiari syndrome.

Adult↗
PubMed1988

Drug-induced vascular and sinusoidal lesions of the liver.

Drugs can induce various vascular and sinusoidal lesions of the liver, in particular hepatic vein thrombosis, veno-occlusive disease, sinusoidal dilatation, peliosis hepatis, hepatoportal sclerosis and angiosarcoma. None of these lesions is specific of drug-induced injury and all of them can be determined by causes other than drugs. However, for one of these lesions, veno-occlusive disease, chemotherapy (and/or irradiation) represents the main aetiology.

Budd-Chiari Syndrome↗
PubMed1998

Sex hormonal preparations and the liver.

The long-term use of oral contraceptives (OCs) may be associated with an increased, though quite small, risk of certain types of liver disease: acute intrahepatic canalicular idiosyncratic cholestasis, benign hepatic tumors (hepatic adenoma, focal nodular hyperplasia, hemangiomas), hepatocellular carcinoma, peliosis hepatis, hepatic vein thrombosis, and portal vein thrombosis. Estrogens have lithogenic properties, as shown by a rise in biliary cholesterol secretion and cholesterol saturation index, yet no substantial increase in the risk of gallstones among estrogen users has been found. Hormone replacement therapy (HRT), given after oophorectomy or menopause, is not associated with clinically significant liver injury. Generally speaking, synthetic sex hormones should not be used in patients with acute and chronic liver disease. A trial of a low-dose estrogen can be instituted under close monitoring for adverse reactions and HRT preparations are not contraindicated in patients with chronic liver disease. Moreover, OCs and HRT can be prescribed quite safely following successful liver transplantation. The incidence of hepatic abnormalities in patients taking androgen hormones is very high. Liver adenomas, cholestasis, peliosis, nodular regenerative hyperplasia and, particularly, hepatocellular carcinoma may complicate long-term use of C17-substituted testosterone and anabolic steroids.

Acute Disease↗
PubMed1991

Imaging primary and metastatic cancer of the liver.

Either CT or MRI can be used as a primary screening test for liver masses in a patient with a known or suspected malignancy. A number of variations in techniques are available for both CT and MRI, and combinations of these techniques are often required to increase the detectability rate for hepatic lesions. Whether CT or MRI is chosen as an initial screening technique depends upon the particular patient and the institution. After a liver lesion has been identified, attempts should be made to obtain a specific diagnosis. Certain liver masses may have a specific CT appearance, especially when they are calcified. With some benign lesions a specific diagnosis is possible using imaging techniques, and in these instances CT and a supplemental radionuclide study may be of complimentary value. These include cavernous hemangioma, focal fatty liver, and focal nodular hyperplasia. Another group of lesions have a CT or an MRI appearance that is suggestive for a specific diagnosis, but may require confirmation with a biopsy or other tests. These include hepatoma, which may present as a mass with portal vein thrombosis, hepatic adenoma, which may appear as a mass with central hemorrhage, focal nodular hyperplasia may occur as a mass with a central stellate scar (on CT), or a cavernous hemangioma, which fulfills specific CT or MRI criteria. A final group of lesions consists of masses without a characteristic or suggestive CT or MRI appearance. These lesions will require biopsy for final diagnosis.

Humans↗
PubMed2006

Current role of portosystemic shunt surgery in the management of hepatic venous outflow obstruction.

BACKGROUND: Hepatic venous outflow obstruction (HVOO) is a rare disorder that occurs predominantly due to a hypercoagulable state. The syndrome may result from hepatic vein obstruction, inferior vena cava obstruction or a combination of both and manifests with post-sinusoidal portal hypertension. The presentation may be fulminant with poor prognosis or as either acute, subacute or chronic forms with relatively better prognosis. Portosystemic shunt surgery (PSS) has thus far been the mainstay of treatment for HVOO. However, over the last decade, transjugular intrahepatic portosystemic shunt (TIPS) and liver transplantation have emerged as viable options. This review aims to evaluate the available treatment options and the current relevance of PSS for the management of HVOO. METHODS: A literature review on investigations and treatment was performed using Medline and additional library searches. RESULTS: Portosystemic shunts form the mainstay of treatment for patients with subacute presentation (preserved liver function) with medically intractable ascites or recurrent variceal hemorrhage. Excellent results with 5-year survival of more than 90% have been reported from specialized centers. The main limitation for PSS is the reported perioperative mortality of 10-20% and a declining technical expertise for such surgery. Liver transplantation with disease-specific 5-year survival between 50 and 95% is presently the treatment of choice for patients with fulminant presentation, end-stage liver disease (ESLD), unshuntable anatomy or decompensation after PSS. TIPS may be preferable for sick patients with acute presentation with isolated hepatic vein thrombosis or as a temporizing measure for those with ESLD awaiting transplantation. The drawback of TIPS is late shunt dysfunction that occurs in more than 50% of patients at 1 year. CONCLUSIONS: Due to rarity of the disorder there is a lack of trials comparing the different treatment modalities. Hence, the current treatment recommendations are based on retrospective studies. In a select group of HVOO patients (subacute presentation with preserved liver function), PSS remains the treatment of choice with excellent long-term results.

Budd-Chiari Syndrome↗
PubMed1983

Budd-Chiari syndrome associated with oral contraceptive steroids. Review of treatment of 47 cases.

Oral contraceptive steroids (OCS) have been implicated as the cause of a number of instances of hepatic vein thrombosis (Budd-Chiari syndrome). Survival appears to be related to early diagnosis and treatment, but there does not appear to be a consensus as to the most appropriate management of these patients. Portosystemic shunting has frequently been advocated, although the results have been quite variable. Some patients appear to do well with conservative measures only. In the effort to obtain a clearer understanding of the effects of different medical and surgical therapies in this disorder, we analyzed the treatment of 47 cases associated with OCS, 29 of which were found in the literature and 18 additional cases identified through a questionnaire survey mailed to members of the AASLD. Surgery had been performed in 27 of these 47 patients (57%); 17 patients had been treated medically (36%); two individuals had received unspecified treatment, and one patient died before any treatment could be initiated. In the surgical group 13 patients underwent portosystemic shunt surgery with six surviving up to 5 years. Two patients survived more than 15 months following orthotopic liver transplant and one patient is well after partial hepatectomy. Of 10 patients who underwent exploratory laparotomy (three with the intent to perform a shunt), seven died postoperatively. Mean survival for the surgically treated group was 19.4 months (range 10 days to 7 years). Of those patients treated medically with combinations of diuretics, anticoagulants, antiplatelet agents, fibrinolytic agents, and peritoneovenous shunts to control ascites, 11 (65%) have survived from 3 months to 6 years (mean survival 29.0 months). We conclude that a satisfactory response may accompany either medical or surgical management of patients with Budd-Chiari syndrome associated with OCS. Patients with severe occlusive disease may benefit most from surgical decompression of the hepatic veins. However, for those with mild to moderate disease, the proper role for operative intervention remains to be defined.

Adult↗
PubMedDate not supplied

A single-center experience of late retransplantation of the liver.

Liver retransplantation is considered to carry a higher risk than primary transplantation. However, there are an increasing number of retransplant candidates, especially owing to late graft failure. The aim of this study was to analyze a single-center experience in late liver retransplantation. The overall rate of primary retransplantation was 11.4% (28 re-OLT out of 245 primary OLT); the 14 (52%) who underwent retransplantation at more than 3 months after the first transplant were analyzed by a medical record review. Causes of primary graft failure leading to retransplantation were chronic hepatic artery thombosis in five cases (36%); recurrent HCV cirrhosis in four cases (29%); chronic rejection in two cases (14%); veno-occlusive disease; hepatic vein thrombosis or idiopathic graft failure in one case each (7%). UNOS status at re-OLT was always 2A, all patients were hospitalized; three were intensive care unit bound. ICU and total hospital stay had been 7 +/- 5 and 28 +/- 16 days, respectively. One- and 2-year patient and graft survivals were 84% and 62% and 67% and 67%, respectively. Death occurred in four patients. Two out of the three recovered in ICU at the time of retransplantation, at a median interval of 15 +/- 9 days after retransplantation. The survival rate after late retransplantation is improving, and this option should be considered to be a efficient way to save lives, especially by defining the optimal timing for retransplantation.

Adult↗
PubMed2005

Possible association between Budd-Chiari Syndrome and gemtuzumab ozogamicin treatment in a patient with refractory acute myelogenous leukemia.

Gemtuzumab ozogamicin (GO; CMA-676; Mylotarg) is a chemotherapeutic agent approved for the treatment of CD33-positive acute myelogenous leukemia in patients of age 60 years or older after first relapse. Hepatic veno-occlusive disease has been reported to develop as a late complication of gemtuzumab ozogamicin treatment. A patient who developed Budd-Chiari Syndrome with hepatic vein thrombosis following treatment with GO is presented. This complication has not been previously reported, and it deserves to be considered as a possible adverse effect of gemtuzumab ozogamicin.

Aged↗
PubMed1992

Fibrinolytic and anticoagulant activities of highly sulfated fucoidan.

A series of fucoidan [sulfated poly(L-fucopyranose)] derivatives were prepared by chemical sulfation and desulfation, and they were tested for their abilities to stimulate tissue plasminogen activator (t-PA)-catalyzed plasminogen activation, clot lysis, and the inhibition of fibrin polymer formation. The magnitude of their activities was dependent upon the degree of sulfation. A striking feature of the sulfated fucoidan was that, unlike heparin, it stimulated t-PA-induced plasma clot lysis by protecting plasmin activity from alpha 2-plasmin inhibitor and decreased the rate of fibrin polymer formation. The inhibition of hyaluronic acid-mediated enhancement of fibrin clot formation was also observed with the fucoidan derivative. We also showed that highly sulfated fucoidan prevents significantly endotoxin-induced hepatic vein thrombosis in the hyperlipemic rat model. The present results are the first to describe the fibrinolytic and anticoagulant activities of fucoidan, and thus may provide useful clues for the development of an ideal thrombolytic agent.

Animals↗
PubMed1995

Cholangitis associated with paroxysmal nocturnal hemoglobinuria: another instance of ischemic cholangiopathy?

Ischemia is increasingly recognized as a cause of cholangiopathy. The aim of this study was to report a case of association of paroxysmal nocturnal hemoglobinuria with abrupt-onset cholangiopathy. Anomalies resembling sclerosing cholangitis were documented in a patient suffering from recurrent biliary pain. None of the conditions that have been associated with primary sclerosing cholangitis or other forms of cholangiopathy was present, but shortly thereafter, paroxysmal nocturnal hemoglobinuria occurred. Hepatic vein thrombosis later complicated the course of the disease. Because the fortuitous coincidence of these uncommon conditions is unlikely, this case indicates that paroxysmal nocturnal hemoglobinuria is a cause of ischemic cholangiopathy. Other thrombogenic conditions may also be implicated in some instances of apparently idiopathic cholangiopathy.

Adult↗
PubMed1993

Preparation of oversulfated fucoidan fragments and evaluation of their antithrombotic activities.

Oversulfated fucoidan fragments (20-40 and 40-60 kDa) were prepared, and their fibrinolytic and anticoagulant activities were compared with those of oversulfated fucoidan (100-130 kDa) reported previously [Soeda et al., Biochem. Pharmacol. 43, 1853-1858, 1992]. The results of these experiments indicated that the in vitro abilities of oversulfated fucoidan to stimulate tissue plasminogen activator (t-PA)-catalyzed plasminogen activation and to potentiate thrombin inhibition by antithrombin III or heparin cofactor II decreased with a decrease in its molecular size. However, the preventive effects of both fucoidan fragments on endotoxin-induced hepatic vein thrombosis in hyperlipemic rats were almost the same as that of oversulfated fucoidan (100-130 kDa). We also found that, unlike heparin treatment, the concentrations of serum and vascular endothelium t-PA in rats treated with oversulfated fucoidan or its fragments (1 mg each/kg/week) were maintained at normal levels. The 20-40 and 40-60 kDa fragments had an ability to decrease the elevated levels of serum cholesterol in hyperlipemic rats, whereas the 100-130 kDa fucoidan derivative did not. These results suggest that oversulfated fucoidan and its fragments have another function(s), besides the regulation of blood coagulation and fibrinolysis, and are of therapeutic benefit for the prevention of thrombus formation in hyperlipemia.

Animals↗
PubMed2006

Interruption or congenital stenosis of the inferior vena cava: prevalence, imaging, and clinical findings.

OBJECTIVE: To present the prevalence, clinical, and imaging findings of interruption or congenital stenotic lesions of the inferior vena cava (IVC), associated malformations, and their clinical relevance. MATERIALS AND METHODS: Between March 2004 and March 2006, 7972 patients who had undergone consecutive routine abdominal multidetector row computed tomography were analyzed for interruption or stenotic lesion of the IVC. RESULTS: Prevalence of interruption (n=8) or congenital stenosis (n=4) of the IVC occurred in 12 (0.15%) of 7972 patients. Four patients with interruption and four patients with congenital stenosis of the IVC were symptomatic with DVT (n=4), leg swelling (n=4), leg pain (n=2), lower extremity varices (n=2), hepatic vein thrombosis (n=1), and hematochezia (n=1). All four of the asymptomatic patients were from the interruption group, and these patients had interrupted IVC with well-developed azygos/hemiazygos continuation. Eight symptomatic patients did not have a well-developed azygos/hemiazygos continuation, and drainage of lower extremity was mainly from collateral veins. Additional findings in eight symptomatic patients were abdominal venous collaterals (n=8), venous aneurysm (n=2), lower extremity varices (n=2), varicocele (n=2), and pelvic varices (n=1). CONCLUSION: Interruption or stenosis of the IVC are rare on routine abdominal CT examinations and may cause different clinical findings depending on the variant drainage patterns or collaterals. Interrupted IVC is commonly asymptomatic if associated with well-developed azygos/hemiazygos continuation, whereas commonly symptomatic if well-developed azygos/hemiazygos continuation is not present.

Adolescent↗
PubMed2005

Combined liver and kidney transplantation in a multicenter transplantation program in Chile.

INTRODUCTION: Combined liver and kidney transplantation (CLKT) is an exceptional therapeutic procedure limited to a few diseases with advanced compromise of these organs. Hyperoxaluria type I and polycystic disease are the most frequent indications. The aim of this article was to report our indications and results of CLKT in a multicenter transplantation program in Chile. MATERIAL AND METHODS: Our Excel database was reviewed to select patients who were treated with CLKT between 1993 and July 2004. RESULTS: Among 242 liver transplantations (LT) and 48 kidney transplantations (KT), 7 were CLKT, representing 2.8% of LT and 14.5% of KT. Four patients were women and 3 were male of average age 46.8 years. One patient was a child. Most frequent indications were chronic renal failure associated with terminal liver disease and polycystic disease. One patient needed liver retransplantation due to hepatic vein thrombosis. One patient had a biliary fistula and another had a urinary fistula, treated conservatively. Acute liver rejection took place in 3 cases, 1 of which required antibodies. Two patients died, 1 due to aspergillosis and the other due to vascular complications in the transplanted liver. Actuarial survival rates were 71.4% at 1 and 5 years. Chronic renal failure is not a contraindication to LT. CONCLUSION: CLKT is an acceptable option for these patients.

Child↗
PubMed1986

Magnetic resonance imaging diagnosis of Budd-Chiari syndrome.

Noninvasive imaging modalities may suggest the diagnosis of Budd-Chiari syndrome but they are rarely diagnostic. Inferior vena cavography, hepatic venography, and liver biopsy, alone or in combination, are usually necessary for definitive diagnosis. Because of its excellent depiction of blood vessels as regions of absent signal, magnetic resonance imaging has the potential to make a noninvasive diagnosis of hepatic vein thrombosis. A case illustrating the usefulness of magnetic resonance imaging in the diagnosis of Budd-Chiari syndrome is presented.

Adult↗
PubMed2000

Flow cytometric analysis of autonomous growth of erythroid precursors in liquid culture detects occult polycythemia vera in the Budd-Chiari syndrome.

BACKGROUND/AIMS: Hepatic vein thrombosis (Budd-Chiari syndrome) is associated with various hypercoagulable states, such as polycythemia vera (PV), presence of the lupus anticoagulant, paroxysmal nocturnal hemoglobinuria (PNH) and deficiencies of antithrombin III, protein C and protein S. In recent years, it has become evident that patients with the Budd-Chiari syndrome may have more than one risk factor that may cause a state of hypercoagulability. The aim of the current study was to assess the prevalence of occult PV in patients with Budd-Chiari syndrome using a novel method for the detection of spontaneous erythroid growth. METHODS: Twenty-two patients with Budd-Chiari syndrome were evaluated. As controls, we studied normal donors and four patients with liver cirrhosis and five patients with right-side heart failure, two conditions that in part mimic Budd-Chiari syndrome. The presence of PV was determined by flow cytometric analysis of autonomous growth of erythroid precursors. Patients were considered as having occult PV if they had spontaneous erythroid cell growth in the absence of erythropoietin and with no features of overt PV. RESULTS: Cells from ten patients with Budd-Chiari syndrome demonstrated spontaneous erythroid cell growth; eight patients (32%) were found to have occult PV and two patients had overt PV. None of the controls had spontaneous erythroid growth. Of the eight Budd-Chiari patients with occult PV, six had one or more additional recognized hypercoagulable states. Seven patients (32%) had protein C deficiency, six patients (27%) had activated protein C resistance, five (23%) had anti-cardiolipin antibodies, five (23%) had antithrombin III deficiency, and four patients (18%) had protein S deficiency. Three patients (14%) were homozygous to methyltetra hydrofolate reductase and ten (45.5%) were heterozygous. One patient had PNH. Overall, in 12 patients there were two or more combined risk factors. CONCLUSIONS: Using a flow cytometric analysis of autonomous growth of erythroid precursors we found a clear correlation between Budd-Chiari syndrome and occult PV.

Adult↗