Evaluation of risk factors in the development of hepatocellular carcinoma in autoimmune hepatitis: Implications for follow-up and screening.

UNLABELLED: Hepatocellular carcinoma (HCC) has traditionally been considered a rare complication of cirrhosis secondary to autoimmune hepatitis (AIH), yet the true incidence remains unknown due to a lack of published data. Consequently, some professional guidelines do not mandate routine surveillance for HCC in this condition. Our aims were to evaluate the rate at which HCC develops among a large, prospectively obtained cohort of patients with AIH at a single center. Demographic, clinical, and laboratory indices associated with the development of HCC were also identified. HCC was discovered in 15 of 243 patients with AIH, all of whom had type 1 AIH equating to 1090 cases per 100,000 patient follow-up years. HCC occurred in the same proportion of females as males, 6.1% versus 6.4%, P = 0.95. HCC occurred more frequently in patients who had cirrhosis at presentation, 9.3% versus 3.4%, P = 0.048, or who had a variceal bleed as the index presentation of AIH, 20% versus 5.3%, P = 0.003. The median duration from time of confirmed cirrhosis to a diagnosis of HCC was 102.5 months, range 12-195 months. Median survival in patients whose HCC was diagnosed on surveillance was 19 months (range 6-36 months) compared with 2 months (range 0-14 months) for patients presenting symptomatically (P = 0.042). CONCLUSION: Cirrhosis in AIH is the sine qua non for HCC development, which subsequently occurs at a rate of 1.1% per year and affects men and women in equal proportions.

Hepatic dysfunction in sickle cell disease: a new system of classification based on global assessment.

BACKGROUND & AIMS: Hepatic dysfunction in adults with sickle cell disease varies in character and severity from self-limited cholestasis to life-threatening acute liver failure and cirrhosis. Because previous attempts to describe patterns of liver disease have not reflected clinical experience, we aimed to characterize the presentation, clinicopathologic findings, and natural history of such patients. METHODS: We reviewed the clinical, laboratory, radiographic, and histologic features with the natural history of 38 patients (mean age, 33 years) with Hb SS, SC, or S-beta thalassemia referred to a tertiary liver center for assessment. RESULTS: Distinct disease patterns were identified that comprised massive hepatocellular necrosis (5%), acute severe sequestration and cholestasis in the context of sepsis (18%), cirrhosis (18%), chronic, fluctuating sequestration without cholestasis (21%), mechanical biliary obstruction (8%), siderosis without cirrhosis (8%), generalized cholangiopathy (8%), venous outflow obstruction (3%), and miscellaneous (11%). Of the 20 who required emergency admission, 8 did not survive their index admission, and 3 patients died during follow-up admissions (4 months-4 years later). There were 3 instances of hemorrhage related to liver biopsy. One patient underwent transplantation but died. Hematologic and biochemical markers did not discriminate well between survivors and nonsurvivors. The incidence of a second hepatic pathology (ie, viral hepatitis, autoimmune disease, transfusional siderosis) was 37% and was associated with the finding of more advanced histologic fibrosis. CONCLUSIONS: Patterns of hepatic dysfunction in sickle cell disease are diverse and demand clear characterization for each individual; however, groups with a poor prognosis can be identified after collation of clinical, laboratory, and radiologic data. Findings at biopsy (which is associated with higher risk of bleeding in this group) might be anticipated by noninvasive test results.

Chronic mesenteric venous thrombosis: evaluation and determinants of survival during long-term follow-up.

BACKGROUND & AIMS: The natural history of chronic portomesenteric (PM) and portosplenomesenteric (PSM) venous thrombosis is defined poorly. Therapeutic options are limited, and are directed at the prevention of variceal bleeding and the control of abdominal pain related to gastrointestinal hyperemia. METHODS: Patients with extensive PM and PSM thrombosis were reviewed retrospectively to evaluate the efficacy of medical therapy and to determine which clinical variables had prognostic significance regarding long-term survival. RESULTS: Sixty patients, with a median age at diagnosis of 44 years (range, 18-68 y), were assessed. The median follow-up period was 3.5 years (range, 0.2-32.0 y). The overall survival rate was 73.3%, with 1- and 5-year survival rates of 81.6%, and 78.3%, respectively. One- and 5-year survival rates, excluding patients who died from malignancy-related causes, were 85.7% and 82.1%, respectively. Factors associated with improved survival included treatment with beta-blockers (P = .02; odds ratio [OR], .09; 95% confidence interval [CI], 0.01-0.70) and anticoagulation (P = .005; OR, 0.01; 95% CI, <0.01 to 0.26). Eighteen patients in total were anticoagulated, including 8 patients who had variceal bleeding, all of whom underwent endoscopic band ligation of esophageal varices before anticoagulation. By using Cox regression analysis, variables associated with reduced survival were the presence of ascites (P = .001; OR, 42.6; 95% CI, 5.03-360), and hyperbilirubinemia (P = .01; OR, 13.8; 95% CI, 1.9-100) at presentation. Six patients died of variceal hemorrhage. CONCLUSIONS: Patients with chronic PM and PSM venous thrombosis without underlying malignancy have an acceptable long-term survival. Treatment with beta-blockers and anticoagulation appears to improve outcome.

Interventional radiology in liver transplantation.

Radiology is a key specialty within a liver transplant program. Interventional techniques not only contribute to graft and recipient survival but also allow appropriate patient selection and ensure that recipients with severe liver decompensation, hepatocellular carcinoma or portal hypertension are transplanted with the best chance of prolonged survival. Equally inappropriate selection for these techniques may adversely affect survival. Liver transplantation is a dynamic field of innovative surgical techniques with a requirement for interventional radiology to parallel these developments. This paper reviews the current practice within a major European center for adult and pediatric transplantation.

One liver, three recipients: segment IV from split-liver procedures as a source of hepatocytes for cell transplantation.

Hepatocyte transplantation is emerging as a possible treatment for patients with acute liver failure and liver-based metabolic disorders. With the limited availability of donor tissue, it is important to find new sources of liver tissue for isolation of high-quality hepatocytes. Segment IV with or without the caudate lobe was removed during three split-liver procedures. Hepatocytes were isolated from the tissues using a collagenase perfusion technique under strict sterile conditions. The mean number of hepatocytes that were isolated was 5.14 x 10(8) cells with a mean cell viability of 89%. Two of the hepatocyte preparations were used for cell transplantation in a 1-day-old boy with an antenatal diagnosis of a severe urea cycle defect caused by ornithine transcarbamylase deficiency. The six recipients of split-liver grafts demonstrated no complications related to the removal of segment IV. Segment IV with or without the caudate lobe obtained from split-liver procedures is potentially a good source of high-quality hepatocytes for cell transplantation.

Microbubble ultrasound contrast in the assessment of hepatic artery patency following liver transplantation: role in reducing frequency of hepatic artery arteriography.

We prospectively evaluated the role of microbubble ultrasound contrast for detection of hepatic artery thrombosis following liver transplantation. The hepatic artery of adult liver transplant recipients with suspected thrombosis on surveillance Doppler ultrasound (US) were re-examined by a second observer. In patients with no hepatic spectral Doppler signal the microbubble contrast agent Levovist was used. The presence or absence of flow following microbubble contrast was evaluated against arteriography or repeated Doppler US findings. A total of 794 surveillance Doppler US examinations were performed in 231 patients. Hepatic artery flow was demonstrated in 759 of 794 (95.6%) examinations. Microbubble ultrasound contrast was administered in 31 patients (35 studies) with suspected hepatic artery thrombosis. Following microbubble US contrast the hepatic artery could not be demonstrated in 13 of 35 (37.1%) studies (12 patients). Eight patients had arteriography: there was hepatic artery thrombosis in 7 patients and 1 patient had a patent, highly attenuated artery. Detection of a patent hepatic artery increased from 759 of 794 (95.6%) to 781 of 794 (98.4%) with the addition of microbubble contrast. Upon independent reading of the data, the degree of operator confidence in the assessment of the hepatic artery patency prior to microbubble contrast was 4.7 (CI 1.92-7.5) but rose to 8.45 (CI 7.06-9.84) following microbubble contrast ( p<0.0001). In 22 of 35 (62.9%) of studies arteriography could potentially have been avoided. Ultrasound microbubble contrast media may reduce the need for invasive arteriography in the assessment of suspected hepatic artery thrombosis.

Adult-to-adult living donor liver transplant: UK experience.

BACKGROUND: Adult-to-adult living donor liver transplantation (ALDLT) is being adopted widely in the USA and mainland Europe, fueled by the increasing waiting lists for cadaver organs. The present report describes the first UK experience with the procedure in patients from overseas who have the lowest priority for cadaver organ allocation. METHODS: The 16 patients seen over the period November 1998 to March 2002 had end-stage cirrhosis from chronic hepatitis C virus (HCV) or hepatitis B virus (HBV) infection (13 cases), with single instances of cryptogenic cirrhosis, secondary biliary cirrhosis and alcoholic liver disease. Grafts were left lobe in the first two recipients and right lobe in the subsequent 14 recipients, donated by nine sons/daughters and seven brothers/sisters. RESULTS: Twelve of the 16 recipients did well. The four recipients who died had recurrent sepsis; two of these died following hepatic arterial occlusion, and in three major surgical factors were present before transplantation. Serial computed tomography (CT) measurements in the survivors showed regeneration of the grafted lobe with final volumes reaching in each case the calculated standard liver volume for body size. In the donors, liver function tests had returned to normal by day 7-14, with rapid regeneration of the remaining lobe, although the final size attained that estimated before donation in only four donors. CONCLUSIONS: ALDLT, although requiring considerable facilities and organization, can give good results for both recipient and donor. As with cadaver grafts, outcome in the recipient if the larger right lobe is used is dependent on surgical risk factors and the severity of clinical decompensation before transplantation. Measures to ensure the safety of the donors remain the main concern.