[Intra-abdominal infections]. / Intraabdominelle Infektionen.

We present 3 patients with intraabdominal infections. The diagnostic difficulties are caused by the atypical presentation of many of these infections. Fever, deterioration of general condition and loss of appetite and weight are uncharacteristic signs that lead to a delay in correct diagnosis and the beginning of therapy. In the following paper we discuss septic pylephlebitis, spontaneous bacterial peritonitis and renal abscesses as important representatives of intraabdominal infections.

[Meningitis in adults]. / Die Meningitis beim Erwachsenen.

The clinical presentation of a 38-year-old female patient with acute onset of fever and decreased level of consciousness is described. The clinical reasoning concerning differential diagnosis, therapeutic options, workup of the patient and possible bacterial etiologies, which is usually part of the physician's initial encounter with the patient, is included in the case presentation. This summary of a workshop offers a concise review of clinical aspects of bacterial meningitis.

[Left ventricular insufficiency: systolic versus diastolic dysfunction]. / Linksventrikuläre Insuffizienz: systolische versus diastolische Dysfunktion.

Left ventricular failure has been subdivided into different forms. Systolic pump failure (= systolic dysfunction) and diastolic filling failure (= diastolic dysfunction) are important entities in the overall framework of heart failure. The clinical patterns of both are presented in light of 2 case reports: systolic dysfunction involves the combination of left ventricular failure, cardiomegaly and depressed systolic ejection fraction. Diastolic dysfunction is accompanied by pulmonary congestion in the presence of a normal or only slightly enlarged ventricle and a normal ejection fraction. Prognosis of systolic dysfunction is poor, with a 5-year survival rate of 40%, compared to 70% in patients with isolated diastolic dysfunction. Medical treatment of systolic dysfunction is based primarily on ACE-inhibitors followed by diuretics and digitalis. Betablockers in low doses and spironolactone can provide additional benefit. Calcium channel blockers are rarely indicated, due to their negative inotropic effects. In patients with diastolic dysfunction, however, they are the first choice because of their positive lusitropic effect on relaxation and ventricular filling. ACE-inhibitors are suitable in hypertensive heart disease, while diuretics and betablockers are second line drugs. Digitalis should be avoided since worsening of diastolic function may occur.

Fatal hematophagic histiocytosis after granulocyte-macrophage colony-stimulating factor and chemotherapy for high-grade malignant lymphoma.

Fatal hematophagic histiocytosis occurred in two patients after they had received granulocyte-macrophage colony-stimulating factor (GM-CSF) in addition to chemotherapy for malignant non-Hodgkin's lymphoma. In one patient GM-CSF promoted the activity of subclinical hematophagic histiocytosis, resulting in severe pancytopenia and multiorgan failure. In the other patient the syndrome that caused persistent bone marrow failure began after the institution of GM-CSF therapy. Exogenous GM-CSF appears to upregulate preexisting hematophagic histiocytosis and may even contribute to its de novo initiation. It is therefore conceivable that endogenous GM-CSF also plays an essential role in the pathogenesis of this syndrome.

[Pericardial empyema as a rare complication of pneumococcal pneumonia]. / Das Perikardempyem als seltene Komplikation der Pneumokokken-Pneumonie.

The case histories of 3 patients hospitalised for severe pneumococcal pneumonia are reported. Electrocardiography showed generalized ST-segment elevations and echocardiography revealed pericardial effusion. Pericardiocentesis was performed and analysis of the punctate fluid was consistent with empyema. The pericardial empyema was removed by percutaneous drainage in one patient and by thoracoscopy in 2. During the course of the disease, 2 patients developed constrictive pericarditis after 4 to 6 weeks, necessitating epi- and pericardectomy in one. When patients with pleuropneumonia present generalised ST-segment elevations in the ECG, the possibility of pericardial involvement should be evaluated by echocardiography. The pericardial empyema must be removed as soon as possible, ideally by thoracoscopic drainage. Percutaneous drainage often fails to evacuate the empyema completely and does not prevent recurrent effusions. Constrictive pericarditis occurs early in the course of the disease and is a serious complication. When a patient suffering from constrictive pericarditis remains symptomatic despite optimal conservative therapy, pericardectomy should be performed. However, as only one of our three patients required the procedure, it should not be routinely performed.

[Thoracic peri-aortic fibrosis and Ormond disease]. / Thorakale periaortale Fibrose und Morbus Ormond.

Two cases of thoracic periaortal fibrosis as a manifestation of retroperitoneal fibrosis (Ormond's disease) are shown on CT and MRI. Thoracic periaortal fibrosis can result in an inflammatory aneurysmo with chronic dissection. Manifestation of thoracic periaortal fibrosis may typically occur intermittently over decades.

[Rhabdomyolysis in patients treated with simvastatin and cyclosporin: role of the hepatic cytochrome P450 enzyme system activity]. / Rhabdomyolyse bei mit Simvastatin und Ciclosporin behandelten Patienten: Rolle der Aktivität des Cytochrom-P450-Enzymsystems der Leber.

We describe two patients treated with a combination of cyclosporin and simvastatin who had to be hospitalized due to rhabdomyolysis. As suggested by reduced cyclosporin clearance, both patients had impaired activity of the hepatic cytochrome P450 enzyme system, which may have contributed to the development of rhabdomyolysis. After cessation of treatment with simvastatin and intensive hydration, both patients recovered within one week. While rhabdomyolysis has been described in several patients receiving the combination lovastatin/cyclosporin, so far only one case has been reported in patients treated with simvastatin/cyclosporin. Our cases therefore suggest that this complication may be more frequent than previously suspected. In patients treated with cyclosporin, HMG-CoA reductase inhibitors should be used cautiously, and concomitant administration of drugs inhibiting the hepatic cytochrome P450 enzyme system should be avoided.

Hemosuccus pancreaticus as a source of obscure upper gastrointestinal bleeding: three cases and literature review.

We report three patients with chronic pancreatitis and recurrent episodes of severe upper gastrointestinal bleeding caused by hemosuccus pancreaticus. No bleeding source could be identified despite repeated endoscopies. In two patients, angiography disclosed a fistula between a peripancreatic artery and a pancreatic pseudocyst. In one patient, a fistula between the splenic artery and the pancreatic duct was found intraoperatively. No rebleeding occurred after operation. In cases of obscure upper gastrointestinal hemorrhage, the pancreas should be considered as a bleeding source, especially in patients with chronic pancreatitis.

[T-cell rich B-cell lymphoma associated with hemophagocytic syndrome]. / T-Zell reiches B-Zell Lymphom assoziiert mit hämophagozytischem Syndrom.

Report of a T-cell rich B-cell lymphoma (TCRBCL) in a 43 years old man with an associated haemophagocytic syndrome (HS). At presentation the haemophagocytic cells involved the same organs as the lymphoma, i.e. spleen, liver, abdominal lymph nodes and bone marrow. As supportive measure to alleviate chemotherapy-induced granulocytopenia the cytokine granulocyte-macrophage colony-stimulating factor (GM-CSF) was given. After an initial improvement of the blood granulocyte count pancytopenia developed again, resulting in fatal sepsis. Autopsy demonstrated massive proliferation of macrophages in the bone marrow with haemophagocytosis as morphological correlation to the pancytopenia. The observation that exogenous GM-CSF enhanced the preexistent HS primarily reactive to the TCRBCL raises the question if endogenous GM-CSF may play a role in triggering a HS. The observed association of TCRBCL and HS has not been reported so far.

Bleeding anorectal varices: successful treatment with transjugular intrahepatic portosystemic shunting (TIPS).

A patient with severe recurrent rectal bleeding from anorectal varices due to portal hypertension because of hepatitis C virus related liver cirrhosis is presented. As illustrated by the report, it is essential to differentiate bleeding anorectal varices from bleeding haemorrhoids because treatment is different. In our patient, implantation of a transjugular intrahepatic portosystemic shunt (TIPS) led to an impressive regression of the anorectal varices, which could be demonstrated by sigmoidoscopy, endosonography, and magnetic resonance imaging. Recurrent rectal bleeding in a patient with portal hypertension should alert the physician to consider anorectal varices. Endoscopic ultra-sound and magnetic resonance imaging are new and non-invasive modalities for diagnosis and post-treatment control.

[Cytokeratin-positive angiosarcoma of the adrenal gland]. / Zytokeratin-positives Angiosarkom der Nebenniere.

Cytological, biopsy and autopsy findings in a patients suffering from massively metastasizing adrenal angiosarcoma are reported. Histogenetic typing of the tumour initially manifestating itself by osseous and liver metastases was problematic with regard to its partially epithelioid structure and its positivity upon cytokeratin immunostaining. Of relevance for the correct typing was the finding that the tumour cells in addition exhibited positivity for vascular markers. This case confirms literature data according to which cytokeratin expression not infrequently may be encountered in endothelial neoplasms and which by no means should lead to exclude such a tentative diagnosis.

[Acute fatty liver in pregnancy: clinical and histopathological course. Case report]. / Akute Schwangerschaftsfettleber: Klinischer und histopathologischer Verlauf. Kasuistik

Acute fatty liver of pregnancy is a rare disease which may be letal if diagnosis is missed. The pathogenesis is not completely clear, but there is some evidence that some cases have been associated with a genetic deficiency of fatty acid beta-oxidation. Other predisposing factors include primiparity, multiple pregnancy, male fetal sex and pre-eclampsia. Clinical presentation and laboratory findings are often unspecific. Increasing serum aminotransferases are characteristic in the early stage of the disease. Liver biopsy establishes the diagnosis and typically shows microvesicular, centrilobular fatty changes of hepatocytes. Differential diagnosis includes the HELLP-Syndrome, cholestasis of pregnancy, pre-eclampsia and viral or drug induced hepatitis. Without adequate treatment liver failure with coagulopathy and encephalopathy may develop. Two cases of acute fatty liver in pregnancy in an early stage are presented. Clinical and histopathological findings as well as diagnostic and therapeutic procedures are discussed.

[Alcoholic ketoacidosis]. / Alkoholische Ketoazidose.

Alcoholic ketoacidosis (AKA) is an important and probably underdiagnosed differential diagnosis for metabolic acidosis with an increased anion gap. It occurs in patients with prolonged ethanol intake. After a brief period of starvation induced by alcoholic gastritis patients typically present with vomiting, abdominal pain and Kussmaul breathing. Routine testing for urine or serum ketones with ketostix may be negative, since they do not detect beta-hydroxybutyrate and this is characteristically elevated in AKA. We present three cases of AKA and discuss diagnosis, pathophysiology and management of this disorder.

[Hepatitis-C virus and hepatoma]. / Hepatitis-C-Virus und Hepatom.

To clarify the relationship between hepatitis C virus (HCV) infection and hepatocellular carcinoma (HCC), frozen serum samples from 213 patients with histologically proven liver cirrhosis alone (96 alcoholics, 59 HBsAg positive, 29 non-A, non-B hepatitis, 29 cryptogenic) and 40 patients with liver cirrhosis and HCC (12 alcoholics, 7 HBsAg positive, 7 non-A, non-B hepatitis, 14 cryptogenic) were analyzed for antibodies to hepatitis C virus (anti-HCV) with the ortho-HCV-ELISA. The results were as follows. 50 of 253 (20%) patients were anti-HCV positive. The prevalence of anti-HCV was significantly higher in patients with HCC than in patients without HCC (14 of 40 [35%] vs 36 of 213 [17%]; p less than 0.001). In anti-HCV-positive patients HCC were significantly more frequent than in anti-HCV-negative patients (14 of 50 [28%] vs 26 of 203 [13%]; p less than 0.001). The significantly higher occurrence of HCC in anti-HCV-positive patients was not related to other known risk factors such as alcoholism or chronic hepatitis B virus (HBV) infection. Patients with HCV infection as the only risk factor also had a significantly higher occurrence of HCC (12 of 38 [32%] vs 26 of 203 [13%]; p less than 0.001). Our data suggest that chronic HCV infection plays an important role in the pathogenesis of HCC, in particular in patients with cirrhosis unrelated to alcohol or HBV infection.

Dorsocranial liver resection and direct hepatoatrial anastomosis for hepatic venous outflow obstruction: long-term outcome and functional results.

OBJECTIVES: The management of hepatic venous outflow obstruction, usually known as Budd-Chiari syndrome, remains complex despite a variety of treatments. METHODS: We present the results from 16 patients with Budd-Chiari syndrome who underwent dorsocranial liver resection and direct hepatoatrial anastomosis over a 10-yr period. The inferior caval vein was occluded in 10 patients. RESULTS: Operative mortality was 12.5% (2/16). During a mean follow-up of 7.2 yr, three patients required reoperation, two of whom had veno-occlusive disease. The late mortality was 14% (2/14). Clinical status improved in terms of: abdominal pain, 81 versus 14%; lower limb edema, 56 versus 14%; hematemesis, 19% versus 0%; hepatomegaly, 94 versus 36%; esophageal varices, 56 versus 7%; splenomegaly, 56 versus 21%; and ascites, 87 versus 29%. Mean serum bilirubin (micromol/l) fell from 40.2 to 27.1 (p = 0.005), and serum albumin remained unchanged. A patent hepatoatrial anastomosis was demonstrated in 10/12 survivors. The actuarial survival rate was 74.2% at 5 and 10 yr. CONCLUSIONS: Thus, in patients with outflow obstruction of the major hepatic veins, transcaval dorsocranial liver resection and direct hepatoatrial anastomosis recreate an adequate hepatic runoff. Moreover, this procedure is appropriate for patients with occlusion of the inferior caval vein, obviates or defers the need for liver transplantation, and prevents recurrence of thrombosis.