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Background and Study Aims: The Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction. A step-wise management was recently proposed. The aim of this study is to reassess our treatment approach and long-term outcome., Patients and Methods: The data of 37 Budd-Chiari patients, seen in our unit, were critically analyzed and compared with the ENVIE (European Network For Vascular Disorders of the Liver) data., Results: Most patients had multiple prothrombotic conditions (41%), of which an underlying myeloproliferative neoplasm was the most frequent (59%). The JAK2V617F mutation was associated with more complete occlusion of all hepatic veins (JAK2 mutation +: 70% vs JAK2 mutation -: 23% and a higher severity score. The step-wise treatment algorithm used in our unit, in function of the severity of the liver impairment and the number and the extension of hepatic veins occluded, resulted in the following treatments: only anticoagulation (n = 7.21%), recanalization procedure (n = 4.21%), portosystemic shunts (n = 9.26%) and liver transplantation (n = 14.44%). This resulted in a 10 year survival rate of 90%. Treatment of the underlying hemostatic disorder offered a low recurrence rate. None of the 21 patients with a myeloproliferative neoplasm died in relation to the hematologic disorder., Conclusions: An individualized treatment regimen consisting of anticoagulation and interventional radiology and/or transplantation when necessary and strict follow-up of the underlying hematologic disorder, provided an excellent long-term survival, which confirm the data of the ENVIE study., (© Acta Gastro-Enterologica Belgica.)

Chronic pancreatitis (CP) is an inflammatory disorder characterized by inflammation and fibrosis, resulting in a progressive and irreversible destruction of exocrine and endocrine pancreatic tissue. Clinicians should attempt to classify patients into one of the six etiologic groups according to the TIGARO classification system. MRI/MRCP, if possible with secretin enhancement, is considered the imaging modality of choice for the diagnosis of early-stage disease.In CP, pain is the most disabling symptom, with a significant impact on quality of life. Pain should be assessed using the Izbicki score and preferably treated using the "pain ladder" approach. In painful CP, endoscopic therapy (ET) can be considered as early as possible. This procedure can be combined with extracorporeal shock-wave lithotripsy (ESWL) in the presence of large (> 4 mm), obstructive stone(s) in the pancreatic head, and with ductal stenting in the presence of a single main pancreatic duct (MPD) stricture in the pancreatic head with a markedly dilated MPD. Pancreatic stenting should be pursued for at least 12 months in patients with persistent pain relief. On-demand stent exchange should be the preferred strategy. The simultaneous placement of multiple, side-by-side, pancreatic stents can be recommended in patients with MPD strictures persisting after 12 months of single plastic stenting. We recommend surgery in the following cases: a) technical failure of ET ; b) early (6 to 8 weeks) clinical failure ; c) definitive biliary drainage at a later time point; d) pancreatic ductal drainage when repetitive ET is considered unsuitable for young patients; e) resection of an inflammatory pancreatic head when pancreatic cancer cannot be ruled out; f) duodenal obstruction. Duodenopancreatectomy or oncological distal pancreatectomy should be considered for patients with suspected malignancy. Pediatricians should be aware of and systematically search for CP in the differential diagnosis of chronic abdominal pain. As malnutrition is highly prevalent in CP patients, patients at nutritional risk should be identified in order to allow for dietary counseling and nutritional intervention using oral supplements. Patients should follow a healthy balanced diet taken in small meals and snacks, with normal fat content. Enzyme replacement therapy is beneficial to symptomatic patients, but also in cases of subclinical insufficiency. Regular follow-up should be considered in CP patients, primarily to detect subclinical maldigestion and the development of pancreatogenic diabetes. Screening for pancreatic cancer is not recommended in CP patients, except in those with the hereditary form.

Non-alcoholic fatty liver disease (NAFLD) is the most prevalent liver disorder in the Western world. It comprises a disease spectrum ranging from simple steatosis to non-alcoholic steatohepatitis (NASH), which may progress to fibrosis, cirrhosis and its complications like hepatocellular carcinoma and liver failure. In addition, evidence is accumulating that NAFLD is an independent risk factor for cardiovascular diseases. Progress has been made in unraveling the pathogenesis, which paved the way for several clinical trials for the treatment of NAFLD. Life style intervention consisting of increased physical activity and dietary modifications, remain the cornerstone of the treatment. Some pharmacological agents show promising results, although on the basis of recent clinical trials no firm conclusions can be drawn. Suggestions for treatment in some particular groups of patients can be made. Further research is required to face the burden of NAFLD, which is already present in epidemic proportions.

Endotipsitis or primary infection of a TIPS-stent, is an uncommon but possible life- threatening condition by its potential evolution to sepsis and death. Diagnosis should be suspected in patients with a TIPS-stent presenting with stent-dysfunction associated with fever or relapsing episodes of bacteremia/sepsis without any other alternative focus. A certain diagnosis is made by post-factum histopathological and/or microbiological examination of the TIPS-stent which is only possible after liver transplantation or at autopsy, whereas it can be highly suspected in case of repetitive positive blood-cultures without any other focus in a patient with a TIPS-stent. The microorganisms responsible for endotipsitis are most frequently of Gram-negative enteric origin. The regimen and duration of the treatment should be individualized and depends on multiple factors like the antibiotic sensitivity of the organism and the patients condition. In case of a fungal infection, longer treatment is recommended.

Introduction: Non-alcoholic Fatty Liver Disease (NAFLD) is increasingly recognised as a source of liver related morbidity and mortality. Hard data on epidemiology and natural history are scarce., Aim: To study demographic and metabolic characteristics of the NAFLD patients seen by Belgian hepatologists., Methods: Belgian hepatologists filled in a questionnaire for every newly diagnosed NAFLD patient between January 1st and December 31st 2004. Liver biopsy was advised if ALT > 1.5 x ULN and if 3/5 of the criteria for the metabolic syndrome (MS) (ATPI-II) were present, but was not mandatory. Biopsy was scored using the Brunt classification., Results: 230 patients were prospectively included in 9 centres; 54% were males; mean age was 49.4 +/- 13.9 y; mean BMI was 30.6 +/- 4.6 kg/m2. The MS was present in 53%. In 16% formerly undiagnosed diabetes was discovered. 51% had a liver biopsy: 25% met the criteria, 26% did not. Grading did not differ between patients with or without MS. Staging was significantly more severe in patients with MS (2.43 +/- 1.25 vs. 1.73 +/- 1.18, p < 0.001). A subgroup of patients with GGT > 5 x ULN were significantly older (55.9 vs. 47.64 y, p = 0.02), more frequently diabetic (53% vs. 23%, p = 0.01) and had more advanced fibrosis (3.42 vs. 1.08, p = 0.008). ALT levels were variable., Conclusions: The MS is highly prevalent in Belgian NAFLD patients and is associated with more severe disease. Mild to moderate fibrosis is frequent, and the proposed criteria for liver biopsy are not accurate in selecting these patients. Patients with elevated GGT constitute a subgroup with more advanced disease.

In a review of the literature concerning autoimmune pancreatitis we had special interest for the concept of IgG4-related pathology as a systemic disease with several clinical manifestations. In general, IgG4-positivity can not only be found in the pancreas, but also at the level of the kidneys, extrahepatic biliary ducts, gallbladder, lungs, salivary glands, lacrimal glands, retroperitoneal tissue, ureters, prostate, meninges and lymph nodes. IgG4 seems to be a central key player in the pathophysiology of this disease.

Background and Study Aims: Endoscopic insertion of a biliary stent is standard practice in the palliative treatment of malignant biliary obstructions. Experience with the new ePTFE-covered Viabil stent is mainly limited to the percutaneous approach. We report our experience with its endoscopic application in patients with distal malignant biliary obstructions., Patients and Methods: Eleven patients with an inoperable tumour, without apparent metastatic disease, and with an ECOG score of 0 to 1, were included. All patients received an ePTFE-covered Viabil stent of 10 mm diameter, with transmural side-holes. Primary endpoints were stent patency and patient survival., Results: Overall median patient survival was 220 days; 10 patients died free of jaundice from non-stent related causes. Due to malfunction of the prototype stents at insertion, the introduction of 2 Viabils was required in 3 patients to acquire complete bile duct drainage. Thus, a total of 14 stents was needed in 11 patients. Stent dysfunction occurred in 3/11 patients. It always resulted from massive stone impaction needing stone removal with additional stenting in two out of 3 patients. Stent patency was 80% at 3 and 6 months, and 63% at 9 and 12 months. Lifetime palliation was 73%., Conclusions: Although the biliary Viabil device has been developed to minimize bacterial adherence and sludge formation, stent dysfunctions in this series always resulted from stone impaction. Moreover, malfunction of the prototype stents needed the insertion of a second stent in 3 patients. Overall life time palliation was 73%. Further experience with newer versions of the device as well as comparative studies versus other metallic stents are needed.

Background and Study Aims: Large international clinical trials conducted in the past 5 years rapidly improved the treatment of chronic hepatitis C; however, it is unclear whether the advances seen in clinical trials are being paralleled by similar improvements in routine clinical practice. PegIntrust is a Belgian community-based trial evaluating the sustained virological response., Patients and Methods: Observational study of 219 patients receiving pegylated interferon alfa-2b (1.5 microg/kg/wk) and weight-based ribavirin (800-1200 mg/day) for 48 weeks. Primary study end point was sustained virological response (SVR), defined as undetectable HCV RNA 6 months after the completion of treatment., Results: In total, 108 patients (49.3 %) had undetectable HCV RNA at the end of therapy, 91 (41.6%) attaining SVR. Of the 111 patients without an end-of-treatment response, 28 were non-responders, and 21 had virological breakthrough. In total, 134 patients attained early virological response (EVR); 88 (65.7%) of those patients attained SVR. In contrast, 82 (96.5 %) of the 85 patients who did not attain EVR also did not attain SVR. Age, fibrosis score and baseline viral load were identified as important predictors of treatment outcome. The most frequently reported serious adverse events resulting in treatment discontinuation were anemia (n = 10), fatigue/asthenia/malaise (n = 6) and fever (n = 3)., Conclusion: Our data indicate that treatment of chronic hepatitis C with PEG-IFN alfa-2b plus weight-based ribavirin results in favourable treatment outcomes in a Belgian cohort of patients treated in community-based clinical practice.

The insertion of a biliary endoprosthesis has become standard therapy in the palliative treatment of a malignant biliary stenosis. For plastic stents, stent occlusion results from clogging caused by the adherence of proteins, bacteria, and sludge to the inner stent wall, resulting in a median stent patency of about 4 to 5 months. No major gain in stent patency can be obtained by the omission of side holes, nor by changes in stent material. Putting the stent inside the bile duct, in a suprapapillary position, does not lead to a longer stent patency. The prophylactic administration of antibiotic agents such as ciprofloxacin or norfloxacin, that are active against the gram-negative enterobacteriaceae leading to stent clogging, could have potential advantages but still needs further study. The insertion of a straight 10 French gauge polyethylene Amsterdam-type of prosthesis in a normal transpapillary position, and without the administration of any prophylactic antibiotic treatment, can still be regarded as state-of-the-art therapy with a plastic stent. This mainly holds true for those patients with a low life expectancy of only a few months, such as it is often the case in patients in a poor clinical condition, with liver metastasis, or with a large primary tumor. Patients with a longer life expectancy can be treated with a self-expandable metallic stent. At present, there is no major indication that coated metallic stents will perform any better than the uncoated ones.

Background: Previous reports on endoscopic retrograde brush cytology (ERBC) of bile ducts and of the main pancreatic duct have reached widely varying sensitivity levels of 33 up to 85%., Aims: To report our experience with ERBC in a series of biliary strictures (n = 98) and pancreatic duct strictures (n = 8). For the purpose of our study, that was mainly directed to the value of the cytologic examination as such, only those specimens that were considered satisfactory for cytological interpretation were studied., Patients: From October 1988 until August 1994, 154 cytologic brushings were performed at ERCP in 132 patients. In 132 brushings obtained from 115 patients (86%), cell yield was satisfactory for cytologic interpretation. Nine patients lacked adequate follow-up. Hence, 123 brushings from 106 patients were included in this study. A final diagnosis of malignancy was obtained in 62 patients., Methods: Cytological changes were described as 'benign', 'columnar cell intraepithelial neoplasia', 'inconclusive' by the presence of atypical cells, or 'malignant'., Results: For a positive diagnosis of the malignant nature of a stenosis, ERBC had an overall sensitivity of 63% with a specificity of 96%. One false positive result was obtained in a patient with a biliary infection by Fasciola Hepatica. Sensitivity was highest in malignant ampullary strictures (91%). Sensitivity was 60% for cholangiocarcinomas, and 65% for pancreatic cancer. The finding of 'columnar cell intra-epithelial neoplasia' in the ampullary region led to a Whipple resection and diagnosis of invasive carcinoma in one patient. Atypical cells were found in 4 brush specimens: in three of these 4 cases, a malignant lesion proved to be present., Conclusions: Brush cytology is a simple technique with a high specificity and should be performed in biliary and pancreatic duct strictures of unknown etiology. Categorizing the smears according to cytomorphology may improve diagnostic accuracy.

An overview is given of diagnostic determinations to be carried out on ascites fluid, and on the medical therapy as based on our knowledge of the pathophysiology. Paracentesis has become more frequently used. It is slightly more effective than therapy with high doses of diuretics and carries less side effects. Longterm studies are needed to investigate whether albumin can be safely substituted by dextran-70 or haemaccel.

N-acetylcysteine is a good and reasonable specific antidotum in case of paracetamol intoxication. It is very active when administered within eight hours after intoxication. It can be used as well as in intravenous as peroral administration. There are few side effects.