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We report a 25-year-old man who presented with congenital absence of the portal vein, or Abernethy malformation, a rare congenital disorder in which the mesenteric and splenic venous drainages bypass the liver and directly drain into the inferior vena cava through an extrahepatic portosystemic shunt. Magnetic resonance imaging, which showed multiple nodular lesions in both liver lobes that were associated with an absence of intrahepatic portal venous branches, strongly suggested the diagnosis of the Abernethy malformation. Carbon dioxide wedged venography and transvenous liver biopsy, which were performed in the same session by a right jugular approach, confirmed these findings. This technique can be considered a valuable alternative diagnostic tool to catheter arteriography and percutaneous transhepatic liver biopsy.

Primary lymphoma of the liver is a very rare malignancy. Most often, these lesions consist of diffuse large B-cell non-Hodgkin's lymphoma that occurs mostly in immunodeficient patients. To prove the primary nature of a hepatic lesion, a systemic lymphoproliferative disease should be ruled out. Secondary liver involvement during Hodgkin's and non-Hodgkin's lymphoma is frequent. In advanced cases the incidence varies from 25% to 50%. The present case describes the MRI features of a primary lymphoma of the liver presenting as a solitary nodule. The primary lymphoma presents as a T2-hyperintense homogeneous nodule, with a signal intensity comparable with the signal intensity of the spleen. Signal intensity is comparable on in and out of phase imaging. The nodule is slightly T1-hypointense and doesn't show any arterial contrast uptake. In the late venous phase a slight increase in signal intensity is noted. Two and a half minutes after the administration of contrast agent, the lesion is iso-attenuating with the liver parenchyma. This case is rare because of the concomitant presence of heterozygous sickle cell anaemia and the presence of Gamna-Gandy bodies in the splenic parenchyma. It remains uncertain whether the presence of the Gamna-Gandy bodies is associated with the liver lymphoma or with the underlying sickle cell anaemia, or with a combination of both.

Background The application of available predictive scoring systems for the detection of common bile duct (CBD) stones has not reduced the number of patients who undergo unnecessary endoscopic retrograde cholangiopancreatography. The aim of this study was to create a predictive model for CBD stones and to assess the value of magnetic resonance cholangiopancreatography (MRCP) in prediction. Methods In 1998, 366 patients with gallstone disease (118 males, 248 females; mean age 57 (range 8–84) years) underwent cholecystectomy. Statistical analysis was performed on patient data obtained at the time of first presentation. Results CBD stones were demonstrated in 43 (12 per cent) of 366 patients. The predictive model for common duct stones included ultrasonography showing CBD stones or bile duct dilatation, age greater than 60 years, fever, serum alkaline phosphatase level above 670 units/l and serum amylase level above 95 units/l. In patients with a predicted probability greater than 5 per cent, CBD stones were present in 11 per cent, compared with 1 per cent in patients with a probability of 5 per cent or less. MRCP had an observed sensitivity of 95 per cent, specificity of 100 per cent, positive predictive value of 100 per cent and negative predictive value of 98 per cent. Conclusion In patients with a predicted probability for CBD stones of more than 5 per cent, MRCP is recommended in order to confirm the presence or absence of stones and as guidance in further management.

BACKGROUND The differential diagnosis of hepatic fibrin-ring granulomas includes infective agents (Coxiella burneti, CMV, EBV,....), hypersensitivity to medication (allopurinol) and malignancy. METHODS During a period of 6 months, four patients presented at our university hospital with a similar clinical picture of fever and abnormal liver tests, and fibrin-ring granulomas on liver biopsy. Clinical course, laboratory and imaging findings, and histopathological features were compared. RESULTS Clinical manifestations, and laboratory and imaging findings were similar. Histopathological assessment of the hepatic fibrin-ring granulomas appeared not to be helpful in identifying the causative agent. Other histopathological features (e.g. sinusoidal rows of lymphocytes, eosinophilic polymorphonuclear infiltrate) were suggestive for the causative agent, yet conclusive identification was obtained by either serology (Q fever, CMV, EBV), or by exclusion with concomitant stop of medication (allopurinol). CONCLUSIONS In the differential diagnosis of hepatic fibrin-ring granulomas, serologic titers remain the determining factor, since an infective agent is the most common cause. When hepatic fibrin-ring granulomas are present, other histopathological features may be helpful in making the differential diagnosis.

The antimycotic synthetic azole compounds are known to lead to toxic liver injury. The occurrence of acute hepatitis is best known for ketoconazole. With itraconazole, hepatotoxic reactions have only very rarely been reported, and histologic data are lacking. We report on three patients who developed acute liver damage during therapy with itraconazole, and in whom liver biopsy specimens were obtained.Three patients with apparent itraconazole-induced liver injury were studied. Clinical, laboratory, serologic, and histologic data of all three cases were analyzed.All three patients developed a biochemical-histologic pattern of cholestatic liver injury with damage to the interlobular bile ducts. Beginning ductopenia was present in two, suggesting that itraconazole might be responsible for the occurrence of prolonged drug-induced cholangiopathy. Jaundice was the presenting symptom in all three. It was not accompanied by clinical hallmarks of hypersensitivity, which is suggestive for metabolic rather than for immunoallergic idiosyncrasy.Itraconazole-induced liver injury presents with a cholestatic pattern of injury with damage to the interlobular bile ducts, possibly leading to ductopenia. We suggest that itraconazole should be added to the list of drugs that may be responsible for a drug-induced vanishing bile duct syndrome. Further histologic documentation in other cases is necessary to strengthen our current findings.

We report a patient who presented with recurrent right upper quadrant colics after a previous cholecystectomy. Radiological examination revealed the presence of a polypoid mass in the ileum caused by an anatomo-pathologically proven inflammatory fibroid polyp (IFP) in the ileum. Clinical and pathological aspects related to this rare benign lesion are discussed.

Introduction It is known that anticitrullinated peptide antibody (ACPA)–positive rheumatoid arthritis (RA) has a preclinical phase. Whether this phase is also present in ACPA-negative RA is unknown. To determine this, we studied ACPA-negative arthralgia patients who were considered prone to progress to RA for local subclinical inflammation observed on hand and foot magnetic resonance imaging (MRI) scans. Methods We studied a total of 64 ACPA-negative patients without clinically detectable arthritis and with arthralgia of the small joints within the previous 1 year. Because of the character of the patients’ symptoms, the rheumatologists considered these patients to be prone to progress to RA. For comparisons, we evaluated 19 healthy, symptom-free controls and 20 ACPA-negative RA patients, who were identified according to the 1987 American Rheumatism Association criteria. All participants underwent MRI of unilateral wrist, metacarpophalangeal and metatarsophalangeal joints. Synovitis and bone marrow oedema (BME) were scored according to the OMERACT rheumatoid arthritis magnetic resonance imaging scoring system, and the scores were summed to yield the ‘MRI inflammation score’. Scores were compared between groups. Among the ACPA-negative arthralgia patients, MRI inflammation scores were related to C-reactive protein (CRP) levels and the tenderness of scanned joints. Results MRI inflammation scores increased progressively among the groups of controls and ACPA-negative arthralgia and RA patients (median scores = 0, 1 and 10, respectively; P

A method for mutation detection in the alpha-1 antitrypsin gene (protease inhibitor 1; PI) has been developed using denaturing gradient gel electrophoresis of PCR amplified gene fragments. Using this experimental approach, all common phenotypes and mutations could be detected. Denaturing gradient gel electrophoresis (DGGE) was compared with standard isoelectric focusing (IEF) in 20 potential α1-antitrypsin deficient patients and their relatives. The genotype determined by DGGE was found to be more reliable in some cases than IEF, which is essential for a proper diagnosis of alpha-1 antitrypsin malfunctioning. Hum Mutat 18:243–250, 2001. © 2001 Wiley-Liss, Inc.

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%.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.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.Cytological changes were described as 'benign', 'columnar cell intraepithelial neoplasia', 'inconclusive' by the presence of atypical cells, or 'malignant'.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.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.

A patient is reported who developed acute pancreatitis complicated by pseudocyst formation immediately after the insertion of a self-expanding metallic biliary stent via the percutaneous route. The distal end of the stent was positioned into the duodenum. Endoscopists should be aware of this potential complication of metallic biliary stents.

We report a case of retroperitoneal hemorrhage due to multiple, small pseudoaneurysms complicating a chronic alcoholic pancreatitis. Cross-sectional imaging with CT and US could not clearly depict these vascular lesions. Selective arteriography of the superior mesenteric and gastroduodenal arteries clearly showed the small pseudoaneurysms and definitive treatment was performed by transcatheter embolization using coils. Eight months after successful embolization, the patient is asymptomatic without any recurrent bleeding.

PSC has characteristics of an (auto)immune-mediated disease: however, few studies have evaluated corticosteroid therapy for this disorder.We performed an 8-wk double-blind randomized pilot study to assess the effects of additional treatment with 9 mg budesonide (n = 6) versus 3 mg budesonide (n = 6) versus 10 mg prednisone (n = 6) in patients who had been treated with UDCA (mean dose, 12 mg/kg/day) for at least 5 months without achieving biochemical remission. Pruritus and fatigue were evaluated using visual analog scales. Serum liver biochemistry was measured every 4 wk. At entry and at the end of the trial, adrenocorticotrophic hormone (ACTH) and dehydroepiandrosterone (DHEA) were measured to assess effects on the pituitary-adrenal axis. Duodenal bile was collected for assessment of biliary corticosteroid activity.Pruritus decreased significantly more in the prednisone group compared to both the 3-mg and the 9-mg budesonide groups (p0.05). Alkaline phosphatase (mean: -23.4%; p = 0.03) and IgG (mean: -16.2%; p = 0.04) decreased in the prednisone group, whereas bilirubin, gamma-glutamyl transferase, aspartate aminotransferase, and alanine aminotransferase did not change significantly. No significant clinical or liver biochemical changes were observed in the 3-mg and 9-mg budesonide groups. Significantly larger drops in serum ACTH were found in the 10-mg prednisone group (-40.7%; p = 0.04) and 9-mg budesonide group (-36.6%; p = 0.02) compared to the 3-mg budesonide group (+ 19.0%). No significant differences in percentage change in baseline values for DHEA between the three treatment arms were found. Mononuclear cell proliferation assays did not demonstrate corticosteroid activity in bile. Autoimmune hepatitis was observed in one case (9 mg budesonide) when corticosteroids were tapered off.The results of this pilot study suggest only minor beneficial short-term effects of prednisone but not budesonide on symptoms and serum liver tests in UDCA-treated PSC patients.

A case of hepatic arterial haemorrhage after percutaneous biliary drainage catheter placement is presented. Temporary control of the haemorrhage was obtained by placement of a larger drainage catheter which tamponaded the bleeding artery. Hepatic angiography followed by transcatheter embolotherapy provided a definitive control of bleeding.

Bowel rest during treatment of acute pancreatitis deprives the gut of nutrients and affects its structure and function. Enteral feeding is usually performed late in the course of acute pancreatitis and therefore cannot prevent intestinal barrier dysfunction and possible bacterial translocation. To assess the effect of early enteral nutrition we performed a prospective study on 21 patients (11 males/10 females) presenting with severe acute pancreatitis (13 biliary, 6 alcoholic, and 2 miscellaneous). Severity was established by a mean Ranson score of 3.57. All but one patient could be fed through a double-lumen nasogastrojejunal tube within 60 h after admission. No significant complication of the technique was observed. We conclude that early jejunal feeding can be used safely in severe acute pancreatitis. Further comparative studies are necessary to demonstrate any superiority to total parenteral nutrition.

The objective of this study was to assess the usefulness of dynamic single-shot MR cholangiopancreatography in the evaluation of the morphology and contractility of the normal Vaterian sphincter complex and to assess whether i.v. injection of glucagon can improve visualization.Sixty patients without signs of Vaterian sphincter complex dysfunction were studied. A fast single-shot MR imaging sequence was used to obtain 20 consecutive images of the Vaterian sphincter complex during successive episodes of breathholding. In patients of group A (n = 30), 10 images were obtained before and 10 after i.v. administration of a sphincter-relaxing agent (glucagon). In the patients of group B (n = 30), no glucagon was administered. The degree of visualization of the Vaterian sphincter complex was assessed.Overall, the morphology and contractility of the Vaterian sphincter complex was adequately assessed in 57 patients (95%). However, the number of repetitions required to obtain this result varied greatly (mean, seven; range, two to 18). Glucagon had no apparent effect on the visibility of the most distal portion of the common bile duct.Nonvisualization of the most distal portion of the common bile duct on MR cholangiopancreatography studies is a normal variant that can simulate disease. Obtaining serial breath-hold images using a single-shot technique is helpful to avoid diagnostic errors.

A 43-year-old man presented with abdominal discomfort caused by relapsing pancreatitis. Radiological examination revealed a multilocular cystic mass in the tail of the pancreas, which was resected. Gross examination showed a multilocular cystic lesion measuring 2.5 cm in diameter and containing clear fluid. Microscopically, a mucinous cystadenoma with mesenchymal stroma was diagnosed. The lesion showed two different components: a cyst lined by a columnar, mucin-secreting epithelium and a moderate cellular stroma composed of spindle cells. The stromal element appeared similar to primitive mesenchyme. Immunohistochemical staining confirmed this origin through vimentin expression and showed moderate to strong nuclear staining with oestrogen and progesterone receptor antibodies. Cystadenomas are rare tumours of the pancreas, but mesenchymal stroma is uncommon in such tumours; it is more frequently described in the liver and the bile ducts, and primarily in women.

Shortage of liver grafts is the only limiting factor for application of liver transplantation and causes an increasing mortality on the waiting list. Very old donors (>70 to 80 years old) are rarely referred to transplant centers because of the assumption that these livers will not work properly. Alternatively, transplant teams may be reluctant to use these very old livers due to the risk of poor posttransplant outcome. We reviewed our experience with seven liver transplantations using very old donor livers. We found that the results in terms of graft function and patient survival are adequate. Interestingly, the majority of these donors originated from a single referring donor unit (of more than 20 units who belong to our donor network) that systematically refers all brain-dead donors to the transplant center, independent of the age of the potential donor. This implies that many of these donors are left undetected in other units. In conclusion, very old donors should be referred to transplant centers since results of transplantation with these grafts are favorable.

The severity of rheumatoid arthritis (RA) is highly variable between patients and currently known risk factors explain only part of this variance.1 Much research is dedicated to identify additional new risk factors. Such factors may shed light on the processes underlying progression of RA and many risk factors together may enable risk stratification and individualised treatment of RA. With interest we read the study by Moller et al ,2 showing that patients with RA with anaemia have more severe radiological progression. Although anaemia in RA is generally considered to be a consequence of chronic inflammation, this recent study based on patients with RA included in the Swiss SCQM-database- observed that the association between anaemia and joint damage was independent of the association between disease activity (measured with the Disease Activity Score including the 28-swollen joint count and erythrocyte sedimentation rate (DAS28ESR) and clinical Disease Activity Index (cDAI)) and joint damage. This led to the presumptions that anaemia in RA captures disease processes that are unmeasured …

universal sustained virological response (SVR), it is eagerly hoped that morbidity and mortality from hepatitis C virus (HCV) will soon disappear. At present, cirrhosis, the predominant risk factor for hepatocellular carcinoma (HCC), is increasing among patients with HCV as they become older and the duration of infection longer. We estimate the impact of DAAs with high SVR rates on the incidence of HCC in the population. Methods: Since the extent to which the risk of HCC decreases in patients with established cirrhosis achieving SVR remains poorly defined, we conducted a simulation experiment in which a cohort of 50 year-old subjects (n = 1,000) with compensated HCV cirrhosis (MELD=6) is followed for 20 years. In a viremic subject, the MELD score, HCC and mortality from end stage liver disease (ESLD) would increase progressively. Once SVR is achieved, MELD would stop increasing and the proportion at risk of HCC decrease over time. Results: The Table summarizes 20-year outcomes in the 50 year-old cohort based on SVR eliminating HCC risk in 3%/year.

OBJECTIVE: To describe the use of percutaneous cholangioscopy in older patients with complex diagnostic and therapeutic bile duct disorders. DESIGN: Case series. SETTING: Tertiary care center. PATIENTS: Fourteen patients with a mean age of 74 (SD ± 9) years (range, 60–91 years) underwent percutaneous cholangioscopy. Eleven of these patients presented with endoscopically irretrievable bile duct stones. These 11 patients represent 4.1% of a group of 342 patients (age ≥ 60 years) with a mean age of 76 (SD ± 9) years who were treated endoscopically because of common bile duct stones between January 1993 and January 1996. Three patients presented with obstructive jaundice resulting from a bile duct stricture. In these three patients, brushing cytology of the strictures had proved to be negative. INTERVENTION: After creation and dilatation of a percutaneous transhepatic tract, cholangioscopy was carried out with a flexible cholangioscope. All procedures were performed under mild sedation and analgesia. Stone disintegration was obtained by electrohydraulic lithotripsy, applied through the working channel of the cholangioscope. RESULTS: Complete stone disintegration and removal was obtained after one to three cholangioscopic sessions in all 11 patients with stones. A histological diagnosis of malignancy was obtained in the three patients with biliary strictures. CONCLUSIONS: Percutaneous cholangioscopy is a well tolerated and promising technique in our diagnostic and therapeutic strategy in older patients with complex biliary disorders not responsive to peroral endoscopic diagnosis or treatment. J Am Geriatr Soc 44:1384–1387, 1996.

Normal pancreatic ageing is characterized by functional and morphological changes of the pancreatic parenchyma and of the ductal system, which, however, do not interfere with normal exocrine pancreatic function. It can be speculated that 'pancreatic lithiasis in the aged' as well as the 'senile idiopathic chronic pancreatitis', two conditions of chronic pancreatitis in the elderly, may represent more extreme forms of these normal age-related changes in pancreatic structure and function. In elderly people, acute and chronic pancreatitis are only rarely related to alcohol abuse, in contrast to the situation in a younger patient population. The presence of gallstones represents the most frequent cause of acute pancreatitis in the elderly. In most aged patients with acute biliary pancreatitis, endoscopic sphincterotomy is the treatment of choice, even when bile duct stones cannot clearly be demonstrated at ERCP. Endoscopic sphincterotomy has been shown to reduce morbidity as well as mortality rates in acute biliary pancreatitis. This technique can even be considered as treatment of choice in elderly patients with an increased operative risk. An elective laparoscopic cholecystectomy should be performed in elderly patients with an acceptable operative risk.

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.

Octreotide has been proposed for the treatment of variceal bleeding. The effects on portal pressure, however, have been variable in published studies. As bleeding is more directly related to pressure in the varices, this study investigated the effect on variceal pressure of octreotide and terlipressin, a vasoactive drug with a well established effect. Variceal pressure was measured during four to eight minutes by a continuous non-invasive endoscopic registration method. Thirty patients in whom a stable variceal pressure recording had been obtained during at least one minute, were randomised to receive either 2 mg terlipressin, 50 micrograms octreotide or an identical volume of saline, as a single intravenous injection given over 60 seconds. For the final analysis three patients had to be excluded because of lack of a satisfactory recording. There were no significant clinical differences between the three groups of patients. Placebo administration did not induce significant changes, but a mean decrease in variceal pressure of -27% was noted with terlipressin, starting from two minutes onwards. Variceal pressure changes after injection of octreotide were variable and the mean change in pressure did not reach statistical significance. Seven of 10 patients showed a temporary increase in variceal pressure. In conclusion, terlipressin induces a significant and progressive decrease in variceal pressure but inconsistent variations of variceal pressure changes were seen after octreotide administration. This is probably related to its effect on central venous pressure. This study also shows that continuous variceal pressure recording with the non-invasive endoscopic registration technique detects in an accurate way the effect of vasoactive drugs on variceal pressure, because placebo injection did not produce significant changes.

In the treatment of patients with primary biliary cirrhosis (PBC), methotrexate (MTX) and ursodeoxycholic acid (UDCA) have both been associated with clinical, biochemical, and histologic improvement. Studies with methotrexate have only been performed in uncontrolled conditions. We conducted a prospective controlled study on the combined treatment with methotrexate and ursodeoxycholic acid, comparing the clinical, biochemical, and histologic evolution in six untreated patients with that in eight patients treated with MTX 15 mg/week in association with UDCA 500 mg/day. All patients had noncirrhotic PBC and were followed up for two years. A significant decrease of alkaline phosphatase, glutamic pyruvic transaminase, and gamma-glutamyltranspeptidase was found in the methotrexate/ursodeoxycholic acid treated-group, as compared to the control group. The clinical and histologic evolution, however, was not significantly different in the two groups. Methotrexate toxicity consisted of interstitial pneumonitis in one, of a transient rise of transaminases at three months in five, and of a significant decrease of blood platelets and white blood cells after two years of treatment. In controlled conditions, a two-year treatment with methotrexate and ursodeoxycholic acid does not produce a significant clinical or histologic benefit. Based on this experience, and taking into account the possible risks associated with this therapy, the empiric use of methotrexate cannot be recommended in patients with non-cirrhotic PBC.

We evaluated whether elderly patients with pulmonary tuberculosis and without apparent preexisting liver disease are at an increased risk to develop hepatotoxicity from an isoniazid-rifampin regimen and require regular liver function tests in comparison with younger patients. We analyzed the data of 131 patients treated in the period 1980-1985 of whom 64 (49%) were at least 60 yr of age. Subsequent increases of transaminases (measured weekly for as long as 4 wk after the start of treatment and later on when symptoms suggestive of hepatotoxicity occurred) above baseline values were found more frequently in the elderly (38 versus 18%, p0.05) and were also more pronounced in them (p0.01). The ratio of the highest transaminase value over the baseline value was called the transaminase index (TI). A TI of at least 5 was found in 22% of elderly and 8% of younger patients (p = NS), but ratios as high as 10 were mostly asymptomatic and always normalized progressively without treatment adjustment. Only symptomatic patients with a TIor = 10 (five elderly and three younger) required temporary or definitive treatment adjustment. We conclude that repeated liver function test evaluations are generally unnecessary, except for symptomatic elderly and younger patients alike, in order to detect those with a TIor = 10, thus requiring drug adjustment.

A long-term study and follow-up of an uniform treatment strategy for esophageal varices was evaluated to plan future strategies more effectively.Morbidity and mortality in 101 patients with liver cirrhosis followed up for 5 years in a single center after the first detection of oesophageal varices were studied.Definitive hemostasis of the first variceal bleeding episode could not be obtained in 31.5%, despite emergency endoscopic sclerotherapy in combination with vasopressin and/or balloon tamponade. Mortality related to this bleeding was 26% in this cohort of patients. Overall mortality from variceal bleeding during the 5 year period was 38%. Death occurred predominantly in patients with advanced liver disease, since the Child-Pugh score of patients who bled and survived was 8 +/- 2 as compared to 11 +/- 2 (p0.01) in those who bled and died. If fundic varices were the origin of the hemorrhage, the mortality reached 78% within 6 months. Eighty-five percent of the fatalities were related to the liver disease. Besides variceal bleeding, infection was the predominant trigger leading to liver failure; occurring in 25%. The cause of non-liver related death was malignancy of esophagus, lung and pancreas. Only 13% of the patients with liver failure had undergone an orthotopic liver transplantation.Since variceal hemorrhage still carries a high mortality rate, primary prophylaxis for patients at high risk is indicated. The systematic, concomitant use of vasoactive drugs to sclerotherapy and the early performance of TIPS in case of failure of sclerotherapy have to be evaluated. Finally patients with an advanced liver disease and increased risk factors for variceal bleeding should be considered earlier for liver transplantation.

A case of successful wire coil embolization of a bleeding gastroduodenal pseudoaneurysm secondary to necrotizing pancreatitis with pseudocyst formation is reported. The embolization technique using multiple coils is discussed and compared with other techniques previously described.

Abnormal liver tests, right upper quadrant pain and hepatomegaly occurring in an obese or in a diabetic patient may point to the presence of fat or of glycogen accumulation in the liver parenchymal cells. Marked hepatomegaly due to cytoplasmic glycogen deposition is mainly found in poorly controlled insulin-dependent diabetic patients. If accompanied by cushingoid features, growth retardation and by delayed puberty, a diagnosis of Mauriac syndrome can be made. Hyperglycaemia, insulin administration and increased concentrations of the counterregulatory hormone cortisol may all play a role in the glycogen deposition by their concerted actions on the glycogen phosphorylase and synthase enzymes, promoting the accumulation of glycogen. Hypercortisolism may be responsible for growth retardation and delayed puberty in Mauriac patients. Regression of hepatomegaly and of the associated clinical characteristics may be obtained by a better metabolic control due to the administration of long-acting insulin and the change from single to twice daily injections. Fatty liver is rare in insulin-dependent diabetic patients and is indicative of a poor diabetic control. This process is quickly reversible by adequate insulin treatment. Steatosis is frequently found in maturity-onset diabetics and in obese patients. The pathogenetic mechanisms leading to the accumulation of triglycerides and of fatty acids in the hepatocytes can easily be understood from the normal cycling of fatty acids between the adiopose tissue and the liver. Histologic features of nonalcoholic steatohepatitis can also be found in obese and in diabetic patients. Steatohepatitis may rarely evolve into cirrhosis. In general, there is no correlation between the degree of the biochemical alterations and the severity of the histological findings.(ABSTRACT TRUNCATED AT 250 WORDS)

A patient with primary biliary cirrhosis (PBC) developed marked hypoprothrombinemia with decreased concentrations of the vitamin K-dependent coagulation factors VII, IX, and X during treatment with rifampicin. The coagulation abnormalities were easily corrected by administration of vitamin K. Different mechanisms may be involved, such as a decreased production of menaquinones by intestinal bacteria, a warfarin-like effect by inhibition of the vitamin K epoxide reductase, or an increased oxidative degradation of vitamin K as a result of hepatic microsomal enzyme stimulation. Whatever the mechanism involved, the appearance of this complication in a patient with PBC probably points to the importance of a pre-existing poor vitamin K status. Patients with PBC, treated with rifampicin, should have a regular monitoring of their vitamin K status. Adequate vitamin substitution should be administered, if necessary.

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.

Plasma gonadotropins were measured in 126 postmenopausal women (age range 69-90 years) admitted to a geriatric ward. After clinical examination, patients were classified as 'acutely or severely ill' or as 'not ill'. Plasma gonadotropins were compared between both groups. Logistic regression was used to select clinical and/or biochemical parameters which differentiated patients with abnormal, low gonadotropins from patients with high gonadotropin concentrations. Plasma gonadotropins were significantly lower in the 'acutely or severely ill' than in the 'healthy' patient group. By logistic regression, blood sedimentation rate, total protein concentration, and serum thyrotropin concentration were significantly correlated with low gonadotropins. Linear regression analysis showed a significant linear relationship between plasma gonadotropins and age (at least for ages over 80 years), blood sedimentation rate (at values more than 65 mm) and total protein concentration. Marked suppression of plasma gonadotropins was frequently found to occur in acutely ill postmenopausal women, in relation to biochemical parameters of acute illness such as blood sedimentation rate, total protein concentration, and serum thyrotropin. In addition, ageing per se seems characterized by a progressive attenuation of the high postmenopausal gonadotropin levels.

We reviewed the radiological documents and protocols of 196 cases of bile duct tumors examined over a period of 12 years: 20 of them (10.2%) presented with a polypoid endoluminal growth. The aim of this study was to provide a better knowledge about the radiological features of this less frequent kind of tumor. In these 20 cases, the correct diagnosis of bile duct lesion was provided in 100% by E.R.C.P., P.T.C., U.S. as well as C.T., and in 42% by arteriography. The correct diagnosis of tumor was made by E.R.C.P. in 86%, by P.T.C. in 88%, by U.S. in 61%, by C.T. in 63%, and by arteriography in 25%. A correct diagnosis of tumor could be reached in all cases by combination of several examination techniques. The anatomopathological diagnosis was: bile duct adenocarcinoma (7 cases), adenocarcinoma of the ampulla of Vater (4), villous adenoma of Vater's ampulla (2), cystadenoma (1), cystadenocarcinoma (1), hepatocellular carcinoma (1), apudoma (1), and metastases (3).

Percutaneous transhepatic cholangioscopy in combination with intracorporeal electrohydraulic lithotripsy was successfully applied in 3 patients with biliary tract stones which could not be removed by endoscopic techniques. In a fourth patient, cholangioscopy was used as a diagnostic tool for the evaluation of a right hepatic duct stenosis above a hepatico-jejunostomy. The percutaneous transhepatic tract was dilated up to an 18-French size over a period of 1 week. Maturation of the tract was then awaited for another week before cholangioscopic procedures were started. The only complication noticed consisted of haemobilia that occurred in 1 patient after introduction of the initial percutaneous external-internal drainage catheter. Percutaneous cholangioscopy is a promising new technique in our diagnostic and therapeutic strategy in patients with complex biliary tract disorders.

In the film-based organization of communicating radiological results to the referring physician, the different media (text, images, graphics, voice) are separated. When using computer technology, multimedia reports containing links between these different media can be used. This changes the way radiological reports are generated, accessed, and possibly discussed. We performed experiments in a clinical setting using two different metaphors for communicating multimedia information. In the 'paper metaphor', labels in the report text are linked to annotations in selected images. In the 'slide presentation metaphor', annotated images are presented synchronously to a spoken report. With both systems additional interaction between radiologist and referring physician is supported using multimedia 'electronic mail'. The experiments indicate that multimedia does not only significantly increase the efficiency of information transfer, but also has the potential to make reporting itself more efficient. Given that the amount of image-related information keeps growing, multimedia links are a promising method to give efficient access to the most relevant information.

Background/Aims: Incomplete septal cirrhosis (ISC) is a form of macronodular cirrhosis characterized by slender, incomplete septa that demarcate inconspicuous nodules. Its clinical features have not been investigated in a large series. The aims of this study were to review the clinical symptoms and evolution of ISC in 42 patients. Methods: Forty-two patients with at least one liver biopsy strongly suggestive of ISC were selected for the study covering a period between 1968 and 1987. Data for these patients were compared with the evolution of 49 patients with classical macronodular cirrhosis after chronic active hepatitis type B or C. Results: Possible etiological factors for ISC were alcohol abuse, arsenic treatment, and hepatitis B infection. In three cases, a genetic factor could not be excluded. Patients with ISC had significantly lower serum concentrations of transaminases and bilirubin at diagnosis. Compared with macronodular cirrhosis, bleeding varices were more frequent (57% vs. 22%) in ISC. Ten-year survivals in the ISC and the macronodular cirrhosis groups were 54% and 57%, respectively. Conclusions: ISC represents a relatively stable burnt-out form of macronodular cirrhosis with an unusually high incidence of variceal bleeding. This could be explained by a superimposed insufficiency of the portal vascular supply.

1. alpha 1-antitrypsin is an antiprotease that inhibits the neutrophil elastase enzyme, and belongs to a family of structurally related serine proteinase inhibitors (serpins). Its methionine358 residue determines the specificity for elastase. 2. The normal M-type alpha 1-antitrypsin is mainly synthesized in the liver parenchymal cells and transported to the plasma. Abnormal Z-mutant alpha 1-antitrypsin is retained in the endoplasmic reticulum, which leads to its intracellular accumulation and to markedly decreased plasma levels. 3. In normal conditions, alpha 1-antitrypsin protects the lungs from destruction by the proteolytic neutrophil elastase. A protease/antiprotease imbalance in the lung is responsible for the development of emphysema in severe alpha 1-antitrypsin deficiency and in cigarette smokers, and accounts for the marked acceleration of the lung disease in smoking alpha 1-antitrypsin deficient patients. Smoking has to be avoided in alpha 1-antitrypsin deficient patients. Replacement therapy with plasma-derived alpha 1-antitrypsin seems indicated in alpha 1-antitrypsin deficient patients with emphysema. 4. Intracellular accumulation of abnormal Z-alpha 1-antitrypsin molecules in liver parenchymal cells may lead to liver disease, ranging from neonatal cholestasis to adulthood cirrhosis and hepatocellular carcinoma. End-stage liver disease can be treated by liver transplantation, which is followed by a phenotypic conversion. 5. Diagnosis of alpha 1-antitrypsin deficiency related disease relies on the presence of a low serum concentration of alpha 1-antitrypsin, and of periodic-acid Schiff positive globules in the liver parenchymal cells. Isoelectric focusing of the serum identifies the protease inhibitor phenotype. The protease inhibitor phenotype is determined by the independent expression of the two parental alpha 1-antitrypsin alleles. It is determinant of the serum level and of the risk for development of lung or liver disease.

Abnormalities in the metabolism of sex hormones are frequently observed in cirrhotic patients, especially in chronic alcoholics. Signs of hypogonadism with disturbed reproductive and endocrine gonadal functions are found in men as well as in women. Primary hypogonadism as well as hypothalamic-pituitary inhibition seem to play a role. Primary gonadal insufficiency is the result of a direct toxic effect of ethanol and of acetaldehyde on the gonads with inhibition of LH binding to the Leydig cells, with inhibition of the enzymes responsible for the formation of sex hormones, and with inhibition of the intratesticular activation of vitamin A. Hypothalamic-pituitary hypogonadism also results from a direct toxic effect of ethanol as well as from the increasing concentrations of oestrogens in the plasma. Men with alcoholic cirrhosis may also be characterized by signs of feminization, which mainly result from an increased peripheral conversion of androgens. In men who abstain from alcohol, a spontaneous recovery of sexual functions can occur, especially when no testicular atrophy is found and when the response of gonadotrophins after stimulation with LH-RH is normal. Therapeutically, only the administration of non-aromatizable androgens in high doses seems to lead to recovery of potency.

Two patients with cirrhosis are presented who developed retrosternal pain and dysphagia immediately after sclerotherapy of esophageal varices. Extensive submucosal bleeding of the esophageal wall was demonstrated radiologically and endoscopically. Complete resolution occurred spontaneously and did not lead to residual complications such as strictures. Intramural hematoma of the esophagus is an unusual complication after endoscopic variceal sclerotherapy.

Four young males with chronic renal failure and absent or stagnant puberty were treated with testosterone esters. Endocrine evaluation before therapy showed low plasma follicle stimulating hormone (FSH) levels and relatively high luteinizing hormone (LH). Following therapy skeletal maturation accelerated more than growth velocity, resulting in a lower predicted adult height. In three patients osteoporosis increased or rickets developed. Testosterone therapy was effective in developing sex characteristics, but endogenous pubertal development was not stimulated. Growth velocity was increased, but the effect on growth was more than outweighed by bone age acceleration.

We report our experience with percutaneous transhepatic cholecystostomy in 10 elderly patients with acute cholecystitis, complicated by empyema formation. Most of these patients has severe underlying disease, rendering them at high risk for surgical intervention. In all patients, the percutaneous procedure was followed by a rapid regression of clinical symptoms and of radiologic abnormalities. Six were considered inoperable. Three of these remain free of biliary symptoms, respectively 22, 10, and 7 months after percutaneous cholecystostomy. Three others died of nonbiliary disease 1-4 months after cholecystostomy. Three patients underwent successful elective cholecystostomy 1-5 wk after percutaneous cholecystostomy. In one patient, cholecystectomy had to be performed because of recurrence of hydrops, 1 wk after catheter removal. In our opinion, percutaneous transhepatic cholecystostomy is a safe and effective procedure in the treatment of elderly patients with acute complicated cholecystitis. It can be followed by elective cholecystectomy in good surgical candidates, or by an expectant conservative management in high surgical risk patients.

It was discovered that eight patients with complications of non-cirrhotic portal hypertension had received an arsenical preparation for psoriasis as Fowler's solution some years age. Seven of them were admitted for bleeding oesophageal varices. Upon admission, splenomegaly and hypersplenism were present. Liver tests were normal and palmar skin keratosis and melanosis were noted. Liver biopsy of six patients showed features of incomplete septal cirrhosis. Malignant skin lesions were present in half of the patients. One patient died from lung carcinoma and another from an ovarium neoplasm. Chronic arsenic intake should be actively looked for in all patients with psoriasis and non-cirrhotic portal hypertension. They should be followed up for many years for development of malignant lesions in skin, lung and liver. Liver abnormalities present in the biopsies are often minor and may escape detection.