The epidemiology of Budd–Chiari syndrome in France

IntroductionEpidemiological data is lacking on primary Budd–Chiari syndrome (BCS) in France.MethodsTwo approaches were used:(1) A nationwide survey in specialized liver units for French adults.(2) A query of the French database of discharge diagnoses screening to identify incident cases in adults. BCS associated with cancer, alcoholic/viral cirrhosis, or occurring after liver transplantation were classified as secondary.ResultsApproach (1) 178 primary BCS were identified (prevalence 4.04 per million inhabitants (pmi)), of which 30 were incident (incidence 0.68 pmi). Mean age was 40 ± 14 yrs. Risk factors included myeloproliferative neoplasms (MPN) (48%), oral contraceptives (35%) and factor V Leiden (16%). None were identified in 21% of patients, ≥2 risk factors in 25%. BMI was higher in the group without any risk factor (25.7 kg/m² vs 23.7 kg/m², p < 0.001).Approach (2) 110 incident primary BCS were admitted to French hospitals (incidence 2.17 pmi). MPN was less common (30%) and inflammatory local factors predominated (39%).ConclusionThe entity of primary BCS as recorded in French liver units is 3 times less common than the entity recorded as nonmalignant hepatic vein obstruction in the hospital discharge database. The former entity is mostly related to MPN whereas the latter with abdominal inflammatory diseases.     

Milestones in the discovery of Budd‐Chiari syndrome

In 1845, George Budd published a brief report regarding three patients who developed an obstruction of the hepatic veins. The condition has never been reported before, and was related to sepsis and alcoholism. Fifty‐three years later, Hans Chiari postulated that syphilis was causing the obstruction of the hepatic veins, and enriched the debate with clinical and pathological correlations. Following the hypothesis on the ‘phlebitis obliterans’, several authors proposed other pathophysiological explanations including congenital causes, chronic trauma and exogenous toxins. RG Parker, in 1959, first recognized the relationship between obstruction of hepatic veins and thrombophilic conditions such as polycythaemia vera, pregnancy and hormonal therapy. Based on that, anticoagulant treatment was attempted, but with unsatisfactory outcome. We need to wait until the mid 1980s to see a widespread adoption of anticoagulants, with a consequent improvement of patients' survival. The fear of haemorrhagic events in patients with liver disease discouraged this therapeutic approach, and other surgical interventions (mainly port‐systemic shunts) were conceived, but with high morbidity and mortality. The first liver transplantation in 1976 and the first trans‐jugular intra‐hepatic porto‐systemic shunt in 1993 represented two major cornerstones in the management of Budd‐Chiari syndrome (BCS). Such progresses allowed modifying the treatment of BCS until the modern concept of stepwise therapy. The present review thoroughly reviews the major landmarks in the discovery, treatment and clinical management of patients with BCS.     

Long‐term outcome of a covered vs. uncovered transjugular intrahepatic portosystemic shunt in Budd–Chiari syndrome

Background: The clinical outcome of a covered vs. uncovered transjugular intrahepatic portosystemic shunt (TIPS) for patients with Budd–Chiari syndrome (BCS) is as yet largely unknown. Objectives: To compare patency rates of bare and polytetrafluoroethylene (PTFE)‐covered stents, and to investigate clinical outcome using four prognostic indices [Child–Pugh score, Rotterdam BCS index, modified Clichy score and Model for End‐Stage Liver Disease (MELD)]. Methods: Consecutive patients with BCS who had undergone TIPS between January 1994 and March 2006 were evaluated in a retrospective review in a single centre. Results: Twenty‐three TIPS procedures were performed on 16 patients. The primary patency rate at 2 years was 12% using bare and 56% using covered stents (P=0.09). We found marked clinical improvement at 3 months post‐TIPS as determined by a drop in median Child–Pugh score (10–7, P=0.04), Rotterdam BCS index (1.90–0.83, P=0.02) and modified Clichy score (7.77–2.94, P=0.003), but not in MELD (18.91–17.42, P=0.9). Survival at 1 and 3 years post‐TIPS was 80% (95% CI: 59–100%) and 72% (95% CI: 48–96%). Four patients (25%) died and one required liver transplantation. Conclusions: A transjugular intrahepatic portosystemic shunt using PTFE‐covered stents shows better patency rates than bare stents in BCS. Moreover, TIPS leads to an improvement in important prognostic indicators for the survival of patients with BCS.     

Population‐based assessment of chronic myeloid leukemia in Sweden: striking increase in survival and prevalence

The clinical outcome for patients with chronic myeloid leukemia (CML) has improved dramatically following the introduction of tyrosine kinase inhibitors. An improved survival, combined with a constant incidence, is expected to increase the prevalence of CML. However, data on the prevalence of CML remain scarce. We examined the overall and relative (age and gender matched) survival and assessed the past, present, and projected future prevalence of CML in Sweden. Data on all patients diagnosed with CML between 1970 and 2012 were retrieved from the Swedish Cancer Register and the Swedish Cause of Death Register. The 5‐year overall survival increased from 0.18 to 0.82, during the observed time period. Between 2006 and 2012, the 5‐year relative survival was close to normal for 40‐year‐old, but considerably lower for 80‐year‐old CML patients. The observed prevalence tripled from 1985 to 2012, from 3.9 to 11.9 per 100 000 inhabitants. Assuming no further improvements in relative survival, the prevalence is projected to further increase by 2060 to 22.0 per 100 000 inhabitants (2587 persons in Sweden). The projected dramatic increase in CML prevalence has major medical and health economic implications and needs to be considered in planning how to organize future care of CML patients.     

Budd–Chiari syndrome: Focus on surgical treatment

Budd–Chiari syndrome (BCS) is caused by an obstruction in the hepatic venous outflow tract at various levels from small hepatic veins to the inferior vena cava (IVC) due to thrombosis or fibrous sequelae. This rare disease mainly affects young adults. Risk factors have been identified and patients often have multiple risk factors. Myeloproliferative diseases of atypical presentation account for nearly 50% of patients in Europe and North America countries. Multistep management is required for such patients. Interventional revascularization and transjugular intrahepatic portosystemic shunt procedure are indicated after initial anticoagulation therapy, whereas IVC plasty using a patch graft is indicated for obstruction of the IVC. Liver transplantation (LT) is usually indicated as a treatment for liver failure despite various treatments. The outcomes of LT are good, with a 5‐year survival after LT of nearly 70%.     

A serology‐based approach combined with clinical examination of 125 Ro/SSA‐positive patients to define incidence and prevalence of subacute cutaneous lupus erythematosus

Objective

To estimate the incidence and prevalence of Ro/SSA‐positive subacute cutaneous lupus erythematosus (SCLE) in Stockholm County, Sweden (1.8 million inhabitants) and to investigate the frequency of photosensitivity and other clinical manifestations associated with Ro/SSA autoantibodies.

Methods

Ro/SSA‐positive patients in Stockholm were identified via registry‐based searches. All patients who tested positive for the presence of Ro/SSA autoantibodies during 1996–2002 (n = 1,323; 85% women) were identified. A questionnaire was sent to all patients still living in Stockholm in 2003 (n = 1,048). Patients who reported having skin symptoms and photosensitivity (n = 125) underwent a clinical examination.

Results

Of the 741 (71%) of 1,048 Ro/SSA‐positive patients who responded to the questionnaire, 400 (54%) reported having photosensitivity, and of these patients, 125 agreed to be clinically examined. A diagnosis of LE was confirmed in 59 of the 125 patients (SCLE in 20, systemic LE [SLE] in 33, and chronic CLE in 6). Eighty‐six patients reported experiencing symptoms consistent with polymorphous light eruption (PLE). Comorbidities such as cardiovascular disease, autoimmune disease, and other skin diseases were common. The incidence of Ro/SSA‐positive SCLE during the study period was estimated to be 0.7 cases per 100,000 persons per year and the prevalence was ∼6.2–14 in 100,000 persons.

Conclusion

The incidence of Ro/SSA‐positive SCLE in Stockholm County, Sweden is estimated to be 0.7 per 100,000 persons per year as compared with an incidence of SLE in Sweden of 4.8 per 100,000 persons per year. The prevalence is estimated to be 6.2–14 in 100,000 persons. Self‐reported photosensitivity commonly corresponds to a history of PLE in Ro/SSA‐positive patients, even when the clinical profile of SCLE is absent. Photoprotection should therefore be included in the treatment recommendations for these patients.

     

Favourable medium term outcome following hepatic vein recanalisation and/or transjugular intrahepatic portosystemic shunt for Budd Chiari syndrome

BACKGROUND: We report our experience with management of patients with Budd Chiari syndrome over the past two decades. In 1996 we described a novel approach involving recanalisation of hepatic veins by combined percutaneous and transvenous approaches. This was incorporated into an algorithm published in 1999 in which our preferred treatment for all cases of Budd Chiari syndrome with short segment occlusion or stenosis of the hepatic veins involves recanalisation of the hepatic veins by transvenous or combined percutaneous-transvenous approaches. In symptomatic Budd Chiari syndrome where recanalisation is not possible, we perform transjugular intrahepatic portosystemic shunts (TIPS) because TIPS decompresses the portal circulation directly in an adjustable way. In this series of patients with Budd Chiari syndrome treated with radiological interventions alone, we assess their medium term outcome using two independent objective prognostic indices. METHODS: We retrospectively studied 61 patients with non-malignant Budd Chiari syndrome treated by radiological intervention alone in our centre. RESULTS: Actuarial survival for the entire cohort at one year and five years was 94% and 87%, respectively. Survival of our patients with mild disease (according to the Murad classification) was 100% at one year and at five years, with intermediate disease severity 94% at one year and 86% at five years, and with severe disease 85% at one year and 77% at five years. CONCLUSION: Management of Budd Chiari syndrome by interventional radiology resulted in excellent medium term survival for patients in all categories of disease severity.     

Prevalence and incidence of primary biliary cirrhosis are increasing in Finland

OBJECTIVE: To examine the epidemiology of primary biliary cirrhosis (PBC) in Finland and to evaluate whether the possible increase in prevalence was attributable to the increasing incidence, better survival, or both. MATERIAL AND METHODS: The Hospital Discharge Register, pathology registers, and death certificates for the years 1988 99 were scrutinized, and the patients identified were followed-up for survival until 31 October 2004. The study area covered four university hospital districts: a total of 25 hospitals. The diagnosis of PBC was regarded as definite (or probable) if three (or two) of the following criteria were fulfilled: positive antimitochondrial antibodies, constantly elevated alkaline phosphatase, and compatible liver histology. RESULTS: In the total population of the study areas, the age-standardized prevalence of PBC increased during the study period from 103 (95% CI: 97-110) to 180 (172-189) per million inhabitants. Incidence increased from 12 (10-14) to 17 (15-20) per million inhabitants per year. The annual average increase in prevalence was 5.1% (4.2-5.9%, p <0.0001) and in incidence 3.5% (0.9%-6.0%, p =0.008). In gender-specific analyses among women, the prevalence of PBC increased from 161 (151-171) to 292 (277-207) per million during the study period and the incidence from 20 (16-24) to 27 (23-32) per million per year. The death rate was 4% per year and half the deaths were from liver-related causes. Survival after diagnosis during the study period lengthened. CONCLUSIONS: The prevalence of PBC increased in Finland during 1988-99, owing to both the increased incidence and the prolonged survival.     

Japanese case of Budd-Chiari syndrome due to hepatic vein thrombosis successfully treated with liver transplantation

A 22‐year‐old Japanese woman was found to have severe esophageal varices and then suffered from hepatic encephalopathy. She was diagnosed with Budd‐Chiari syndrome (BCS) due to hepatic vein (HV) thrombosis accompanied by portal vein thrombosis without inferior vena cava (IVC) obstruction. Latent myeloproliferative neoplasm (MPN) lacking the JAK2‐V617F mutation was considered to be the underlying disease. Liver transplantation was strikingly effective for treating the clinical symptoms attributable to portal hypertension. Although thrombosis of the internal jugular vein occurred due to thrombocythemia, which manifested after transplantation despite anticoagulation therapy with warfarin, the thrombus immediately disappeared with the addition of aspirin. Neither thrombosis nor BCS has recurred in more than 4 years since the amelioration of the last thrombotic event, and post‐transplant immunosuppression with tacrolimus has not accelerated the progression of MPN. In Japan, IVC obstruction, which was a predominant type of BCS, is suggested to have decreased in incidence with recent improvements in hygiene. The precise diagnosis of BCS and causative underlying diseases should be made with attention to the current trend of the disease spectrum, which fluctuates with environmental sanitation levels. Because the stepwise strategy, including liver transplantation, has been proven effective for patients with pure HV obstruction in Western countries, this strategy should also be validated for utilization in Japan and in developing countries where HV obstruction potentially predominates.     

Prevalence of the JAK2V617F mutation in Chinese patients with Budd-Chiari syndrome and portal vein thrombosis: a prospective study

Background and Aim: Whether routine screening for the JAK2V617F mutation should be performed in Chinese patients with Budd‐Chiari syndrome (BCS) and portal vein thrombosis (PVT) is unclear. Therefore, we aimed to evaluate the prevalence of the JAK2V617F mutation in such patients and to explore the risk factors associated with the mutation. Methods: All consecutive patients with BCS and PVT diagnosed between September 2009 and May 2011 were prospectively enrolled in the observational study and underwent the JAK2V617F mutation detection. Results: Prevalence of the JAK2V617F mutation was 4.3% (4/92) in patients with primary BCS, 26.6% (17/64) in non‐malignant and non‐cirrhotic patients with PVT, and 1.4% (1/71) in cirrhotic patients with PVT. All BCS patients with the JAK2V617F mutation had both platelet count (PLT) of above 100 × 10⁹/L (range, 107–188 × 10⁹/L) and splenomegaly. In non‐malignant and non‐cirrhotic patients with PVT, higher PLT and older ages were the independent predictors of the JAK2V617F mutation. Further, the difference in PLT between the patients with and without the mutation displayed greater significance in the subgroup of patients with splenomegaly (P < 0.0001), but the statistical significance disappeared in the subgroup of patients with splenectomy (P = 0.1312). Conclusions: The low prevalence of the JAK2V617F mutation in patients with BCS suggests that myeloproliferative neoplasm should be an uncommon etiological factor of BCS in China. Routine screening for the JAK2V617F mutation might be recommended in non‐malignant and non‐cirrhotic patients with PVT, but not in cirrhotic patients with PVT. The coexistence of higher PLT and splenomegaly might be closely associated with the JAK2V617F mutation.     

Incidence and prevalence of idiopathic inflammatory myopathies in South Australia: a 30‐year epidemiologic study of histology‐proven cases

Aim: To describe the epidemiology of biopsy‐proven idiopathic inflammatory myopathies (IIM) in South Australia (SA). Methods: Cases of IIM were ascertained by review of all muscle biopsy reports from the Neuropathology Laboratory, Hanson Institute (wherein all adult muscle biopsies in SA are reported) from 1980 to 2009. Clinical correlation of these patients by review of medical records was undertaken. SA population denominator numbers were obtained from the Australian Bureau of Statistics. Results: Three hundred and fifty‐two biopsy‐proven cases of IIM were identified between 1980 and 2009. The overall annual incidence of IIM appeared to be rising with a mean incidence of eight cases per million population (95% CI: 7.2–8.9). This corresponded with an increasing annual incidence of inclusion body myositis (IBM) (prevalence of 50.5 cases per million population in 2009, 95% CI: 40.2–62.7). A female preponderance was noted in both dermatomyositis (DM) (F : M = 2.75 : 1.00) and polymyositis (PM) (F : M = 1.55 : 1.00) but gender distribution was almost equal in IBM (F : M = 1.1 : 1.0). Mean age at diagnosis for IBM (67.5 years) was higher than for DM (55.1 years) and PM (59.0 years). A higher proportion of DM patients reported living in urban dwellings and DM patients tended to be predominantly professionals. Conclusions: In SA there is an increasing incidence of IBM and the prevalence is one of the highest reported to date. This may reflect an increase in the number of biopsies performed, improved histological techniques or a genuine increase in incidence.     

Prevalence and incidence of primary biliary cirrhosis are increasing in Finland

Objective. To examine the epidemiology of primary biliary cirrhosis (PBC) in Finland and to evaluate whether the possible increase in prevalence was attributable to the increasing incidence, better survival, or both. Material and methods. The Hospital Discharge Register, pathology registers, and death certificates for the years 1988–99 were scrutinized, and the patients identified were followed-up for survival until 31 October 2004. The study area covered four university hospital districts: a total of 25 hospitals. The diagnosis of PBC was regarded as definite (or probable) if three (or two) of the following criteria were fulfilled: positive antimitochondrial antibodies, constantly elevated alkaline phosphatase, and compatible liver histology. Results. In the total population of the study areas, the age-standardized prevalence of PBC increased during the study period from 103 (95% CI: 97–110) to 180 (172–189) per million inhabitants. Incidence increased from 12 (10–14) to 17 (15–20) per million inhabitants per year. The annual average increase in prevalence was 5.1% (4.2–5.9%, p<0.0001) and in incidence 3.5% (0.9%–6.0%, p=0.008). In gender-specific analyses among women, the prevalence of PBC increased from 161 (151–171) to 292 (277–207) per million during the study period and the incidence from 20 (16–24) to 27 (23–32) per million per year. The death rate was 4% per year and half the deaths were from liver-related causes. Survival after diagnosis during the study period lengthened. Conclusions. The prevalence of PBC increased in Finland during 1988–99, owing to both the increased incidence and the prolonged survival.     

A serology-based approach combined with clinical examination of 125 Ro/SSA-positive patients to define incidence and prevalence of subacute cutaneous lupus erythematosus

OBJECTIVE: To estimate the incidence and prevalence of Ro/SSA-positive subacute cutaneous lupus erythematosus (SCLE) in Stockholm County, Sweden (1.8 million inhabitants) and to investigate the frequency of photosensitivity and other clinical manifestations associated with Ro/SSA autoantibodies. METHODS: Ro/SSA-positive patients in Stockholm were identified via registry-based searches. All patients who tested positive for the presence of Ro/SSA autoantibodies during 1996-2002 (n = 1,323; 85% women) were identified. A questionnaire was sent to all patients still living in Stockholm in 2003 (n = 1,048). Patients who reported having skin symptoms and photosensitivity (n = 125) underwent a clinical examination. RESULTS: Of the 741 (71%) of 1,048 Ro/SSA-positive patients who responded to the questionnaire, 400 (54%) reported having photosensitivity, and of these patients, 125 agreed to be clinically examined. A diagnosis of LE was confirmed in 59 of the 125 patients (SCLE in 20, systemic LE [SLE] in 33, and chronic CLE in 6). Eighty-six patients reported experiencing symptoms consistent with polymorphous light eruption (PLE). Comorbidities such as cardiovascular disease, autoimmune disease, and other skin diseases were common. The incidence of Ro/SSA-positive SCLE during the study period was estimated to be 0.7 cases per 100,000 persons per year and the prevalence was approximately 6.2-14 in 100,000 persons. CONCLUSION: The incidence of Ro/SSA-positive SCLE in Stockholm County, Sweden is estimated to be 0.7 per 100,000 persons per year as compared with an incidence of SLE in Sweden of 4.8 per 100,000 persons per year. The prevalence is estimated to be 6.2-14 in 100,000 persons. Self-reported photosensitivity commonly corresponds to a history of PLE in Ro/SSA-positive patients, even when the clinical profile of SCLE is absent. Photoprotection should therefore be included in the treatment recommendations for these patients.     

Prevalence of Wegener's granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg-Strauss syndrome within a defined population in southern Sweden

OBJECTIVES: To estimate the point prevalence (p.p.) of Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS) within a defined population in southern Sweden. METHOD: A cross-sectional p.p. study using multiple sources for case identification. The study area, a healthcare district around the city of Lund in southern Sweden (Mellersta Sk?nes sjukv?rdsdistrikt), had, on 31 December 2002, a total population of 287 479 inhabitants. All the identified cases were verified by medical record review. The patients were classified according to an algorithm based on the American College of Rheumatology classification criteria 1990 and the Chapel Hill Consensus Conference definitions 1994. RESULTS: Eighty-six patients (49% female) with a median age of 64.8 yrs (range 15-90.5) fulfilled the study criteria. There were 46 patients with WG; 27 with MPA; nine with PAN; and four with CSS. The p.p. per million inhabitants was estimated on 1 January 2003 to be 160 (95% confidence interval 114-206) for WG, 94 (58-129) for MPA, 31 (11-52) for PAN and 14 (0.3-27) for CSS. Capture-recapture analysis estimated the completeness of the case finding to 96%. CONCLUSIONS: The prevalence of WG, MPA, PAN and CSS in our district is the highest figure reported so far. Explanations for this finding may include high incidence, extensive ANCA-testing, good survival as well as sensitive search methods for case identification.     

Prevalence and incidence of Type 2 diabetes and its complications 1996–2003—estimates from a Swedish population‐based study

Aims To determine the prevalence and incidence of Type 2 diabetes and its complications in Uppsala county, Sweden between 1996 and 2003. Methods Retrospective population‐based study of patients with Type 2 diabetes identified in computerized medical records at 26 county primary care centres. Prevalence and incidence of Type 2 diabetes were estimated in the population aged 30–39, 40–49, 50–59, 60–69, 70–79 and ≥ 80 years. Mortality, prevalence and incidence of complications in patients with Type 2 diabetes were determined through linkage to national inpatient, uraemia and cause‐of‐death registers. Results Crude prevalence of Type 2 diabetes increased from 2.2 to 3.5% between 1996 and 2003. In the population aged ≥ 30 years, the age‐ and sex‐adjusted period increase was 53%[odds ratio (OR) 1.53, 95% confidence interval (CI) 1.47–1.58]. Crude population incidence was approximately stable after 1997 (3.7 cases/1000 residents in 1997 compared with 3.8/1000 in 2003). Age‐ and sex‐adjusted mortality rates in Type 2 diabetic patients decreased by 4% per year (OR 0.96, 95% CI 0.94–0.97). Prevalence rates of cardiovascular disease in Type 2 diabetic patients were essentially stable, affecting 13.8% of females and 18.0% of males in 2003. No trend was detected for prevalence of renal failure or incidence of acute myocardial infarction, stroke and amputation. Conclusions Prevalence of Type 2 diabetes increased in Uppsala county between 1996 and 2003 as a consequence of approximately stable incidence since 1997 and declining mortality. Rates of diabetes‐related complications, notably cardiovascular disease, continued to impose a substantial burden.     

Early changes of the portal tract on microcomputed tomography images in a newly‐developed rat model for Budd–Chiari syndrome

Background and Aim: The effect of increased sinusoidal pressure on the portal tract in Budd–Chiari syndrome (BCS) is as yet not elucidated. Our aim was to investigate portal changes in a newly‐developed rat model for BCS. Methods: We created an outflow obstruction in Sprague–Dawley rats (n = 6) by diameter reduction of the inferior vena cava. Left and right liver lobes with portal vein contrast were scanned using microcomputed tomography, and volumes of the portal tree and liver parenchyma were computed by the ANALYZE software program. Results: Portal branching density was significantly lower in BCS than the shams, and decreased over time (P < 0.01). There was a significant drop in volume of both parenchyma and the portal tree in the left but not right lobes. At 6 weeks post‐surgery, the perfusion index (i.e. ratio between both volumes) became equal to (left) or even higher than (right) the shams, suggesting a new equilibrium with preserved portal perfusion. Histological findings were consistent with those observed in humans. Conclusion: As early as day 2, a significant loss of peripheral portal branches was seen, which progressed over time. Inter‐lobar differences in vascular abnormalities suggest compensatory mechanisms. Despite a decrease in both liver and portal vein volume, relative portal perfusion appeared spared.     

Incidence, prevalence and clinical course of primary biliary cirrhosis in a Spanish community

BACKGROUND AND AIMS: Primary biliary cirrhosis (PBC) is characterized by the autoimmune inflammatory response of small intrahepatic bile ducts. Prevalence in Spain is estimated as 61.9 cases per million inhabitants, whereas Northern Europe rates over 200 cases/million. Our objective was to determine the incidence and prevalence of PBC in our health area. MATERIAL AND METHODS: PBC was defined by the presence of abnormal liver tests (dissociated cholestasis) with positive antimitochondrial antibodies and/or compatible liver histology. Medical records from patients diagnosed between 1990 and 2002 were reviewed retrospectively. The following data were collected: diagnostic data, demographic and analytic data, liver histology and stage and treatment and disease outcome. RESULTS: In a population of 389 758 inhabitants, 87 patients were diagnosed with PBC. Mean age at diagnosis was 63.9+/-12.6 years. Eighty-four (96.6%) were women. Mean annual incidence was 17.2 per 10 inhabitants and the prevalence at the end of study was 195 per 10. Biopsy was performed in 71 (81.6%) patients, 61 of whom (86%) did not have fibrosis. Time of follow-up was 63.6+/-43.2 (2.28-153.9) months. CONCLUSION: Incidence and prevalence in our reference area are higher than in some Spanish areas, as per the results previously published; however, they are comparable with those obtained in Northern Europe and the US.     

Diabetes,healthcare cost and loss of productivity in Sweden 1987 and 2005—a register‐based approach

Aim The aim of this study was to estimate healthcare cost and productivity losses as a result of diabetes and diabetes‐related chronic complications in Sweden in 1987 and 2005. Research design and methods Published estimates on relative risks and Swedish age‐specific diabetes‐prevalence rates were used to calculate the proportions of diabetes‐related chronic complications that are attributable to diabetes. These attributable risks were applied to cost estimates for diabetes‐related chronic complications based on data from Swedish population registers. Results The estimated total costs for Sweden in 1987 and 2005 were EUR439m and EUR920m, respectively. The increase of 110% was as a result of a 69% increase in the estimated prevalence from 150 000 (1.8% of the population) to 254 000 (2.8%) and of an increase in the estimated annual cost per person diagnosed with diabetes by 24%. Healthcare accounted for 45% of the estimated cost in 1987 and for 37% in 2005. The estimated diabetes‐related healthcare cost accounted for approximately 1.0% of total healthcare cost in Sweden in 1987 and for 1.4% in 2005. Diabetes per se accounted for 57% of the healthcare cost in 1987 and for 50% in 2005. The most important chronic complication was cardiovascular disease. Conclusions The cost of diabetes is substantial and increasing even in a fairly low‐prevalence country such as Sweden. Measures to curb the increase in prevalence and to improve individual control of his or her diabetes seem to be the most important challenges.     

因子V Leiden突变在布加综合征与门静脉血栓形成中的意义

布加综合征与门静脉血栓是2种罕见的肝脏血管病,可伴发肝功能衰竭及门静脉高压症等严重并发症。研究证据表明因子V Leiden(FVL)突变是西方布加综合征与门静脉血栓患者的重要危险因素,但在中国布加综合征患者中比较罕见。着重回顾FVL突变在布加综合征与门静脉血栓形成中的意义,提出尚须前瞻性研究进一步评估FVL突变在中国门静脉血栓患者中的患病率,以判断FVL突变筛查的必要性。     

Haemodynamic response to abdominal decompression in acute Budd–Chiari syndrome

Background: Intra‐abdominal hypertension (IAH) and abdominal compartment syndrome commonly occur in patients with liver disease. Aims: We compared haemodynamic variables pre‐ and post‐abdominal decompression in patients with acute Budd–Chiari syndrome (BCS) and patients with chronic liver disease (CLD), ascites and IAH. Methods: Patients with IAH admitted to the Liver ICU, King's College Hospital were studied. Transpulmonary thermodilution cardiac output (CO) monitoring was performed with the PiCCO^® system. Results: Ten patients with decompensated BCS (median age 39 years, 20–52) and eight patients with CLD (59 years, 33–65) and tense ascites requiring paracentesis were studied. Intra‐abdominal pressure (IAP) was raised in both groups pre‐intervention (BSC 23 mmHg, 17–40; CLD 26, 20–40). Intrathoracic blood volume (ITBVI) was persistently low in the BCS group (632 ml/m², 453–924) despite volume resuscitation. Post‐intervention, reduction in IAP was noted in both groups (BCS P<0.001, CLD P<0.0001). The ITBVI increased (P=0.001) in the BCS group only. An increase in cardiac index (CI) and stroke volume index (SVI) was noted in both groups (BCS: CI P=0.003, SVI: P=0.007; CLD: CI P=0.005, SVI P=0.02). The central venous pressure did not change in either group and did not correlate with markers of flow (CI, SVI) or IAP. Both groups demonstrated an inverse relationship between IAP, CI and SVI. Conclusion: Patients with BCS and IAH have evidence of central hypovolaemia. In addition to raised IAP, hepatic venous obstruction and caudate lobe hypertrophy limit venous return in patients with BCS. Reduction in IAP and re‐establishment of caval flow restores preload with improvement in CO.