To determine the causes and outcome of all patients with acute liver failure (ALF) in Sweden 1994-2003 and study the diagnostic accuracy of King's College Hospital (KCH) criteria and the model for end-stage liver disease (MELD) score with transplant-free deaths as a positive outcome.
Adult patients in Sweden with international normalized ratio (INR) of >or=1.5 due to severe liver injury with and without encephalopathy at admission between 1994-2003 were included.
A total of 279 patients were identified. The most common cause of ALF were acetaminophen toxicity in 42% and other drugs in 15%. In 31 cases (11%) no definite etiology could be established. The KCH criteria had a positive-predictive value (PPV) of 67%, negative-predictive value (NPV) of 84% in the acetaminophen group. Positive-predictive value and negative-predictive value of KCH criteria in the nonacetaminophen group were 54% and 63% respectively. MELD score>30 had a positive-predictive value of 21%, negative-predictive value of 94% in the acetaminophen group. The corresponding figures for the nonacetaminophen group were 64% and 76% respectively.
Acetaminophen toxicity was the most common cause in unselected patients with ALF in Sweden. KCH criteria had a high NPV in the acetaminophen group, and in combination with MELD score
Reliable epidemiological data for portal vein thrombosis are lacking.
To investigate the incidence, prevalence and survival rates for patients with portal vein thrombosis.
Retrospective multicentre study of all patients registered with the diagnosis of portal vein thrombosis between 1995 and 2004.
A total of 173 patients (median age 57 years, 93 men) with portal vein thrombosis were identified and followed up for a median of 2.5 years (range 0-9.7). The mean age-standardized incidence and prevalence rates were 0.7 per 100,000 per year and 3.7 per 100,000 inhabitants, respectively. Liver disease was present in 70 patients (40%), malignancy in 27%, thrombophilic factors in 22% and myeloproliferative disorders in 11%. Two or more risk factors were identified in 80 patients (46%). At diagnosis, 65% were put on anticoagulant therapy. Thrombolysis, TIPS, surgical shunting and liver transplantation were performed in 6, 3, 2 and 8 patients, respectively. The overall survival at 1 year and 5 years was 69% and 54%. In the absence of malignancy and cirrhosis, the survival was 92% and 76%, respectively.
The incidence and prevalence rates of portal vein thrombosis were 0.7 per 100,000 inhabitants per year and 3.7 per 100,000 inhabitants, respectively. Concurrent prothrombotic risk factors are common. The prognosis is variable and highly dependent on underlying disease.
OBJECTIVES. To study whether primary liver cancer (PLC) could be associated with acute intermittent porphyria (AIP) carriership and whether the activity of erythrocyte porphobilinogen deaminase (PBGD) could be used as a tumour marker for PLC. DESIGN. Prospective study. SETTING. Medical and surgical wards in two general hospitals in G?teborg, Sweden. SUBJECTS. All patients with a strong suspicion of PLC (n = 109) who came to the authors' attention. MAIN OUTCOME MEASURES. Measurement of PBGD activity in erythrocytes. Comparison of the PBGD activity in groups with various final diagnoses-hepatocellular carcinoma (n = 58), cholangiocellular carcinoma (n = 2), malignancy other than PLC (n = 18), benign liver disorders (n = 11)--and according to presence of cirrhosis. RESULTS. None of the patients had a clinical or family history of AIP. Four cases with low PBGD activity, suggesting AIP gene carriership, were found, which is more than expected. However, the cases were evenly distributed amongst the groups. The mean activity of PBGD was higher in cirrhotic patients, irrespective of the presence of PLC, than in others. CONCLUSIONS. (i) Acute intermittent porphyria gene carriership might be associated with an increased risk not only for PLC but also for secondary malignancies and benign tumours in the liver. (ii) High activity of PBGD is not unusual in liver cirrhosis and the reason for this needs to be elucidated, but it seems to be of no clinical value as a tumour marker for PLC.
OBJECTIVE: To assess the incidence and prognosis of fibrolamellar hepatic carcinoma in a defined population. DESIGN: Retrospective study of histological slides. SETTING: University hospital, Sweden. SUBJECTS: The 532 patients (out of a total of 711 who were treated at the university hospital during a 22 year period 1 January 1958-31 December 1979) whose primary hepatocellular carcinoma was confirmed on review of the histological slides. MAIN OUTCOME MEASURES: Incidence and prognosis of fibrolamellar hepatic carcinoma. RESULTS: Two patients (women aged 22 and 46) were found to have fibrolamellar tumours and in both they were advanced and the patients died 2 weeks and 9 months, respectively, after exploratory laparotomy. If these are taken as a proportion of the 18 patients who were under the age of 50 at the time of diagnosis then the incidence of the fibrolamellar type of hepatocellular carcinoma is 11%. Since then (in 1993) we have come across one further case, a woman of 39 who was well 22 months after operation though she had metastatic disease. CONCLUSIONS: The fibrolamellar type of hepatocellular carcinoma is rare, and all three of our cases were young women (under the age of 50). It seems to have a slightly better prognosis than other types of primary hepatic tumours.
Viral markers of chronic hepatitis were tested for in 95 frozen serum samples from 299 patients from Malmö, Sweden, with hepatocellular carcinoma (HCC), diagnosed between 1977 and 1994. Hepatitis B analysis included anti-HBc, HBsAg and, if anti-HBc positive, HBV DNA. Hepatitis C infection analysis included anti-HCV screening, RIBA, HCV RNA and HCV genotyping. HCV genotyping was also carried out in 9 HCV-viraemic HCC-patients from Gothenburg. HCV genotype distribution in HCC cases was compared with Swedish HCV-infected blood donors. Among the 95 patients from Malmö, 28 (29%) had anti-HBc, but only 5 (5%) were chronic HBV carriers, compared with 16 (17%) with chronic hepatitis C (p = 0.021). HCV-related HCC was more common among immigrants (8/16 vs. 8/79; p
Genetic susceptibility to PBC can, at least in part, be ascribed to the major histocompatibility complex. The relevance of immunogenetic markers for the clinical presentation and course, however, is unclear. Thus, the aim of this study was to investigate the contribution of HLA class II genes to susceptibility, clinical presentation and course of disease in PBC patients. HLA genotyping for HLA-DRB1, -DQB1 and -DPB1 was carried out in a total of 99 Swedish PBC patients and 158 controls. Clinical parameters including epidemiologic variables, signs and symptoms of PBC-related liver disease and histologic data were collected and analyzed in 92 patients at study entry and at follow-up five years later. Significant clinical heterogeneity was seen among PBC patients upon study entry. Although a significant disease association was seen for HLA DRB1*08 and DQB1*0402, immunogenetic markers identified neither a particular subset of patients nor an association with the clinical course of the disease. HLA-DRB1*08 and DQB1*0402 provide the strongest immunogenetic influence in PBC. However, this association is not restricted to any particular, clinically defined subgroup of patients and it is not predictive for the course of the disease.
The prevalence of primary liver cancer (PLC) varies throughout the world. It has been attributed to variations in incidence of the predominant histological type, hepatocellular carcinoma (HCC). The incidence of PLC types other than HCC such as cholangiocellular carcinoma (CCC) is far less known, especially in low-incidence areas. The aetiology of HCC and other PLC types is obscure, with the exception of the association between HCC and cirrhosis as well as chronic viral hepatitis. The present retrospective incidence and aetiology study concerns a well-defined population from a period with a high autopsy frequency. Preserved biopsy specimens were re-evaluated histopathologically and patient records were studied. Among 590 histologically verified cases of PLC, HCC constituted 90%, CCC 8% and a mixed form of these types 1%. At the end of the study period the annual age-standardised incidence rate of HCC was 3.6 cases per 100,000 inhabitants. Other PLC types were hepatoblastoma (n = 3), fibrolamellar carcinoma (n = 2), angiosarcoma (n = 1) and infantile haemangioendothelioma (n = 1), each constituting less than 1% of the PLC cases. Comparing HCC with CCC we found that cirrhosis (70%) and alcoholism (21%) was significantly more frequent in HCC, and cholelithiasis was significantly more common (60%) in patients with CCC. In the majority of the PLC cases with liver cirrhosis this disorder was unknown before diagnosis of the tumour.
The objectives of this research were to study the distribution of in- and out-hospital deaths and causes of death in male alcoholics and in particular to analyze obscure cases. In a population-based sample of 1123 men treated in one detoxification unit during 1986-1989, 97 patients with alcohol dependence (DSM-III-R) died
The incidence of primary carcinoma of the liver in Sweden has been reported to increase. In order to study the role of chronic hepatitis B virus (HBV) infection for liver cancer development 40 cases with hepatocellular carcinoma (HCC) were examined for the presence of HBV surface antigen and HBV core antigen in the cancer and in the surrounding non-neoplastic liver tissue. It was not possible to demonstrate a single case with tissue HBV antigen, indicating that HBV plays a minor role in the etiology of HCC in Sweden and thus does not seem to be responsible for the increasing incidence of this cancer.