[Bile duct cysts in adults]. / Gallengangzysten beim Erwachsenen.

INTRODUCTION: Bile duct cysts are rare, congenital dilations of the intrahepatic and/or extrahepatic biliary tract. Most of them present during childhood. The classical triad right upper quadrant pain, jaundice and abdominal mass is present only in a few instances. We report here the bile duct cysts which were diagnosed at our institution from 1989 to 1996. METHODS: 3245 consecutive endoscopic retrograde cholangiopancreatograms (ERCP) were evaluated retrospectively. Diagnosis was made when localized cystic dilations of the intrahepatic and/or extrahepatic biliary tract were present. Diffuse dilations of the intrahepatic and extrahepatic biliary tract were excluded. RESULTS: Bile duct cysts were found in 20 patients (17 females, 3 males) among 3245 ERCPs. Their mean age was 56 +/- 20 (median 64, range 10 to 83) years. The cyst types (according to the Alonso-Lej classification with the Todani modification) were type I in 11 (55%), type II, III and IV in two instances each (10%), and type V (or Caroli's disease) in 3 patients (15%). Leading symptoms were cholestasis in 14 patients, 10 of whom had abdominal pain, jaundice in 4 patients, and single cases of pancreatitis, cholangitis, and abdominal mass. In 2 patients the diagnosis was made incidentally. 10 patients had bile duct stones. We performed endoscopic sphincterotomy in 15 patients with concretions or persistent symptoms, 3 patients had cyst resection. One of these, with a type I cyst, already had a disseminated cholangiocarcinoma. 10 of 17 patients without cyst resection are currently symptom-free after complete removal of all gallstones. One male patient with cholecystolithiasis, who is not operable due to advanced liver disease, has recurrent cholangitis, 4 patients have died from causes unrelated to the bile duct cysts, and 2 patients are lost to follow up. CONCLUSION: Bile duct cysts in adults are rare. There is a preponderance in the female gender, and the most common type is the extrahepatic (choledochal) cyst. The leading symptoms are cholestasis and right upper quadrant pain. There is an increased risk of cholangiocarcinoma. In young patients the cysts should be entirely removed to prevent malignancy. Older persons are usually symptomless after complete removal of gallstones.

Influence of hepatitis G virus infection on liver disease.

The influence of hepatitis G virus (HGV) infection on disease activity in hepatitis C related and unrelated liver disease was investigated in 254 individuals using an EIA polymerase chain reaction assay for HGV. One hundred patients had chronic hepatitis C, 26 primary biliary cirrhosis, and 30 alcoholic liver cirrhosis. In addition, 51 hepatitis B surface antigen (HBsAg)-positive and 47 anti-hepatitis C virus (HCV)-positive blood donors were screened. Hepatitis G virus was detected in 18% of patients with chronic hepatitis C, 13% of patients with alcoholic liver cirrhosis, 11% of patients with primary biliary cirrhosis, 10% of anti-HCV-positive blood donors, and 2% of HBsAg-positive blood donors. Virus load and alanine aminotransferase (ALT) levels did not differ significantly in patients with HCV alone versus patients coinfected with HCV and HGV. However, mild liver fibrosis correlated with HGV coinfection. Hepatitis G virus did not influence ALT levels or liver damage in liver disease unrelated to viral infection.

[The absence of autoantibodies does not rule out autoimmune hepatitis]. / Das Fehlen von Autoantikörpern schliesst eine Autoimmunhepatitis nicht aus.

UNLABELLED: The diagnosis of autoimmune hepatitis is mainly based on the finding of characteristic autoantibodies. Untreated patients have a poor prognosis because of rapid development of cirrhosis. Immunosuppressive treatment most often leads to remission. We report on 2 female patients aged 44 and 53 with autoimmune hepatitis. Initially no significant titers of autoantibodies against nuclear, cytosolic or microsomal components of the hepatocyte could be demonstrated. Seropositivity for autoantibodies was demonstrated 6 and 8 weeks later in the course. Immunosuppressive therapy with steroids resulted in rapid decrease of transaminases with persistent remission of autoimmune hepatitis. CONCLUSION: seropositivity for characteristic autoantibodies is not an absolute criterion for the diagnosis of autoimmune hepatitis. Patients with hepatitis of unknown origin should therefore be given a steroid treatment trial.

[Bromocriptine-induced pleuropneumopathy]. / Bromocriptin-induzierte Pleuropneumopathie.

A 64-year-old man was diagnosed to have Parkinson's disease when aged 42 years and since then has been treated with levodopa and benserazide (up to 875 mg daily). Bromocriptine (up to 35 mg daily) was added to the medication 9 years ago. 3 1/2 years ago he developed exertional dyspnoea (NYHA class II-III) and lost 5 kg in weight. Chest radiography demonstrated pleural effusion and interstitial pulmonary changes in both lung bases. Erythrocyte sedimentation rate was 37 mm in the first hour and the white cell count 10,400/microliters. Extensive tests failed to find malignant tumour or any infectious-inflammatory condition. As it was suspected that the pleuropulmonary changes were associated with the bromocriptine intake, it was discontinued and biperiden and selegiline substituted for it. The pleural effusion regressed almost completely within 8 weeks, and the laboratory tests pointing to inflammation disappeared completely. Clinical, biochemical and radiological tests have remained normal for the last 3 years. The clinical course makes a causal relationship between bromocriptine intake and the pleuropulmonary changes highly probable.

[Acute abdomen]. / Akutes Abdomen.

A 28-year-old turkish patient was admitted to hospital several times within the last years because of acute abdominal and thoracic pain. On each admission laboratory parameters indicative of an acute inflammatory process were initially found to be slightly increased; however, the cause for the complaints remained undetected. During a recent episode of acute abdominal pain a short increase of these laboratory parameters (particularly of CRP) could be documented, and since no other diagnostic sign indicative of an other disease was found, familial mediterranean fever was diagnosed. A basic therapy with colchicine was initiated, and since five months the patient remained mostly free of symptoms.

[Whipple's disease with reactive (AA) amyloidosis]. / Morbus Whipple mit reaktiver (AA-)Amyloidose.

A 59-year-old man had been known for two years to have a clearly elevated blood sedimentation reaction and intermittent arthralgia in the hand. His general condition had progressively deteriorated over the last six months, with weight loss, recurrent vomiting and constipation. At first malignant lymphoma had been suspected because numerous lymph nodes were enlarged. But a lymph-node biopsy revealed massive macrophages with PAS-positive inclusion material, diagnosed as Whipple's disease. The patient died suddenly of a heart attack on the day of diagnosis. At necropsy generalized amyloidosis with marked cardiac involvement was found. Immunohistochemically the amyloid deposits gave a strongly positive reaction to anti-AA, while other amyloid syndromes were excluded using appropriate anti-sera. This was therefore a case of reactive (AA) amyloidosis in the presence of Whipple's disease.

[Primary amyloidosis of the liver]. / Primäre Amyloidose der Leber.

For 2 years a 72-year-old man had suffered from nonspecific upper abdominal discomfort and hepatomegaly. The gamma-glutamate transaminase concentration was increased to 121 U/l, the erythrocyte sedimentation rate was 80 mm in the first hour. Histological examination of tissue from the enlarged liver (22 cm in the midclavicular line) revealed the diagnosis of amyloidosis. The gastric mucosa, duodenum and rectum were not involved. Two years later ascites developed; six months after this he was again hospitalized in hepatic coma. Now, for the first time, a type IgA-lambda paraprotein was demonstrated by serum immunoelectrophoresis. The patient died of slowly progressing anicteric liver failure after having been ill for a total of 4 1/2 years. At autopsy there were extensive amyloid deposits throughout the liver and spleen so that the structure of these organs was hardly recognizable. The amyloid deposits in the liver were restricted to the glomerular region, while there was no amyloid in the heart. Histochemical tests showed that the deposits were strongly positive to the anti-lambda antibody. This was thus a case of primary (AL-lambda) amyloidosis of the liver and spleen which had taken an unusually prolonged course, because the heart was not involved at all and the kidneys only slightly.