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History and Admission Findings: In patients with glucose-6-phosphatase dehydrogenase (G6PD) deficiency (favism) jaundice is usually caused by hemolysis due to stress, infection or following the application of drugs. We report on a 74-year-old Italian with known G6PD deficiency complaining of jaundice, weight loss and abdominal pain. Physical examination revealed jaundice of the eyes. Scrotal examination by palpation and ultrasound showed no abnormalities., Investigations: Serum levels of beta-human chorionic gonadotropin and alpha-fetoprotein were within normal limits, total bilirubin was extremely elevated, with predominant direct bilirubin. Abdominal ultrasound showed posthepatic blockage of bile flow with a dilated ductus hepatocholedochus (DHC) in the absence of gallstones. Enlarged, multiple contrast-stained paraaortic and retroperitoneal lymph nodes were detected by endoscopic ultrasound and magnetic resonance imaging. Due to failed endoscopic retrograde cholangiopancreatography, visualization of the biliary tree by percutaneous transhepatic cholangiography (PTC) was performed showing an occlusion of the DHC., Therapy and Course: After successful stent-implantation by PTC with decompression of the biliary tree, the jaundice disappeared. Computer tomography-guided percutaneous biopsy of a retroperitoneal lymph node was performed for histological evaluation showing a primary extragonadal nonseminomatous germ cell tumor. According to the histology (embryonic carcinoma) and clinical stage of the tumor systemic chemotherapy was initiated including cisplatin, etoposide and ifosfamide. After the first cycle of chemotherapy the patient suffered from pneumonia leading to septic shock. Twenty-seven days after admission, the patient died of multiple organ failure., Conclusion: Extragonadal germ-cell tumor presenting as retroperitoneal lymph nodes with obstructive jaundice has to be considered in the differential diagnosis of cholestasis., (© Georg Thieme Verlag KG Stuttgart · New York.)

History and Admission Findings: A 39-year-old obese woman underwent endoscopic retrograde cholangiopancreatography with elective endoscopic biliary sphincterotomy (papillotomy) for symptomatic retained stones in the common bile duct which were extracted completely after added lithotripsy. Three hours later the patient developed profound subcutaneous emphysema of the face, neck and chest wall and shortness of breath, but had no abdominal pain. Physical examination revealed bilaterally diminished breath sounds and a distended and hyper-resonant abdomen, but no evidence of peritonitis. The patient was afebrile and hemodynamically stable., Investigations: An emergency contrast-enhanced computed tomography (CT) of the chest and abdomen was performed. It demonstrated a bilateral pneumothorax, pneumomediastinum, pneumoperitoneum and pneumoretroperitoneum, in addition to extensive subcutaneous emphysema. There was no evidence of extraluminal leakage of contrast medium or intraperitoneal fluid on the CT., Therapy and Clinical Course: Because of the increasing respiratory distress an intercostal drain was placed in the left pneumothorax and broad-spectrum antibiotics were administered. No drain was placed in the right lung. A follow-up CT after three days showed decreasing pneumomediastinum, pneumoperitoneum and pneumoretroperitoneum as well as resolution of the bilateral pneumothorax. The patient made an uneventful recovery and was discharged home seven days after the intervention., Conclusion: Pneumothorax after endoscopic biliary sphincterotomy is a rare but serious complication that should be kept in mind after postinterventional development of shortness of breath., (Georg Thieme Verlag KG Stuttgart, New York.)

History and Admission Findings: An 84-year-old man was admitted to our hospital because of a swollen left leg resembling deep venous thrombosis. Clinical conditions favoring thrombosis such as surgery, trauma, malignant tumor or immobilization were not evident. His medical and family history were non-contributory. He denied bowel or bladder dysfunction and did not complain of any back pain, discomfort or neurological symptoms. The enlargement of the leg was painless and unilateral. There were no deficits on physical examination. The neurological status of the patient was unremarkable., Investigations: Ultrasonography was performed because deep vein thrombosis was suspected. Color-coded duplex revealed no thrombus in the veins of the left leg. Both femoral veins were compressible. The flow in the femoral vein was much less than in the other leg. Computed tomography demonstrated a solid lesion located in front of the spine displacing the inferior vena cava. CT-guided biopsy was performed. On histological examination it proved to be diagnostic of a ganglioneuroma., Treatment and Course: Initially low molecular weight heparin was administered. Surgical resection was recommended, but the patient opted not to undergo any further treatment at that time. Phenprocoumon was recommended to prevent deep vein thrombosis., Conclusions: Ganglioneuroma is a rare, benign, well-differentiated, slow-growing tumor composed of sympathetic ganglion cells and mature stroma with a good prognosis. Its appearance in elderly persons is extremely rare and may imitate deep vein thrombosis caused by vessel compression. The appropriate treatment is surgical and complete excision is curative.

History and Admission Findings: A 23-year-old woman with a 5-year history of ulcerative colitis was admitted to our hospital because of bloody diarrhea. Two years previously she had undergone a hemicolectomy for a right colonic stricture. A recurrence of inflammatory bowel disease was suspected and treatment with prednisolone begun. The symptoms improved gradually, but 7 days later she complained of lower abdominal pain. Physical examination revealed a soft abdomen, but bowel sounds were reduced., Investigations: The abdominal X-ray was unremarkable, but ultrasonography revealed moderate ascites and no blood flow in the portal vein on Doppler examination. The spleen was slightly enlarged. Contrast-enhanced abdominal magnetic resonance imaging (MRI) was performed immediately, revealing thrombosis of the portal and mesenteric veins., Treatment and Course: As there was no suggestion of intestinal necrosis, laparatomy was not considered necessary. Intravenous thrombolytic treatment with urokinase was given continuously (bolus of 250,000 units, followed by 200,000 units per hour), in order to lower the fibrinogen level to 100 - 150 mg/dl, together with unfractionated heparin, maintaining the activated partial thromboplastin time between 60 and 85 seconds. The thrombolytic treatment had to be stopped several times because of bloody diarrhea, but no transfusion was necessary. Two days after the start of thrombolytic treatment the abdominal pain and ascites ceased. Doppler sonography now demonstrated hepatopetal flow in the previously occluded portal vein. 4 days later, MRI revealed that the thrombus in the portal vein had dissolved and the portal vein was fully patent. The mesenteric vein was partially perfused, a residual thrombus extending into the portal vein. Tests for thrombophilia were negative. The thrombolytic therapy was stopped after 112 hours and the patient was treated with oral anticoagulation for 6 months. The patient recovered completely, with no evidence of portal hypertension during the following 6 months., Conclusions: Thrombolysis with urokinase, guided by the level of fibrinogen, may be an alternative, semi-invasive treatment option in acute thrombosis of the portal and mesenteric veins.

Background: Silastic cuffed catheters play an increasing role in providing long-term vascular access for hemodialysis. The reasons for this were related to increased patient age and an increased number of patients who had exhausted sites for vascular access. We report our experience with subcutaneously tunnelled cuffed hemodialysis catheters., Method: From May 1, 2001 to February 28, 2003, 258 consecutive hemodialysis catheters were placed in 203 patients (122 men, mean age 70 +/- 12 years) as access for hemodialysis (1.3 catheters/patient). Catheter implantation was explained to all patients and a protocol consent form was signed. The catheter was implanted via the internal jugular, subclavian or femoral vein and the correct final catheter position was determined fluoroscopically. Short and long-term catheter-associated complications were collected from the time of catheter insertion until a follow up of at least 6 months after implantation., Results: The mean duration of implantation procedure was 38 +/- 12 minutes with an initial clinical success rate of 100 % and a periprocedural complication rate of 6 %. The median catheter indwell time was 9 months with a primary patency rate of 72 % at the end of the follow-up. Inadequate flow rate < 200 ml/min was noted at one per 41 patient-months but sufficient blood flow was restored in 50 % of these patients with non- or semi-invasive interventions. Bacteremic episodes occurred at a rate of one episode per 16 patient-months. Catheter removal due to severe exit site infections or bacteremic episodes were necessary at one per 73 patient-months. There were no cases of bacterial endocarditis., Conclusion: Subcutaneously tunnelled cuffed venous hemodialysis catheters are a safe und highly feasible vascular access with a low complication rate and a long use-life especially for elder patients with limited life expectancy, exhausted sites for vascular access or in case of failing hemodialysis arteriovenous fistulas.

History and Admission Findings: A 53-year-old woman had suffered from progressive lower limb weakness for 8 weeks. She was not able to walk on her own. Two hours after admission endotracheal intubation and mechanical ventilation became necessary because of acute respiratory failure., Investigations: Chest radiograph and computerized tomography showed a huge lesion originating from the lower lobe of the left lung with mediastinal infiltration. Histological examination of bronchoscopical-guided biopsy disclosed small cell lung cancer. Electrophysiological studies revealed an incremental response on high-rate stimulation in the repetitive nerve stimulation test. Autoantibodies to P/C-type voltage-gated calcium channels were detected. Further, laboratory test results revealed hyponatremia (100 mmol/l) and elevated ADH (11 pg/ml)., Diagnosis, Treatment and Clinical Course: These results led to the diagnosis of paraneoplastic Lambert-Eaton myasthenic syndrome (LEMS) with acute respiratory failure caused by small cell lung cancer. Additionally, SIADH (syndrome of inappropriate antidiuretic hormone secretion) was diagnosed as another paraneoplastic disease. Because of a prolonged weaning process resulting from inadequacy of mechanical ventilation, administration of intravenous immunoglobulins and chemotherapy with cisplatin and etoposide were undertaken. This made successful extubation possible after 7 days. In spite of all supportive treatment, clinical deterioration occurred. The patient died 27 days after the small cell lung cancer had been diagnosed., Conclusion: The Lambert-Eaton myasthenic syndrome should be considered in cases of unexplained acute respiratory failure and clinical evidence of neoplastic disease.

History: A 42 year old woman was resuscitated from ventricular fibrillation. 5 months previously she had a syncope. Her nephew had died of sudden cardiac death at the age of 25 years., Investigations: There was no evidence for ST segment elevation, myocardial infarction or pulmonary embolism. The ECG showed right precordial T wave inversion. Coronary artery disease was excluded angiographically. Echocardiography and angiography revealed inferior wall akinesia of the right ventricle with normal left ventricular function and chamber size. Ventricular fibrillation could not be reproduced by programmed stimulation of the right ventricle during an electrophysiologic study. Results of endomyocardial biopsy of the right ventricle showed a focal fibrous infiltration of the myocardium. Magnetic resonance imaging confirmed inferior wall abnormalities of the right ventricle without typical fatty infiltration in the right ventricular myocardium., Clinical Course: The patient recovered rapidly without neurologic deficits. Arrhythmogenic right ventricular dysplasia was suspected, and a cardioverter defibrillator (ICD) was implanted. Within 6 months after implantation the ICD memory showed no evidence of ventricular fibrillation., Conclusion: Arrhythmogenic right ventricular dysplasia is an important cause of ventricular fibrillation with a potential risk of sudden cardiac death in young persons. Concealed arrhythmogenesis as an early manifestation of right ventricular dysplasia is difficult to detect.

History and Admission Findings: A 67-year-old man had complained of diffuse abdominal pain and constipation for 4 days without indication of any underlying disease. On admission there was no evidence of weight loss, fever or nocturnal sweating., Investigations: Physical examination revealed signs of an acute abdomen with high-pitched bowel sounds and diffuse abdominal guarding. The X-ray showed ileus of the small intestine which required emergency laparotomy. An obstructing conglomerate tumour was present in the area of the ileum, ca. 80 cm proximal to the caecum. It was removed by partial resection of the small intestine., Diagnosis: Ileus of the small intestine with a low-malignant marginal zone B-cell (non-Hodgkin) lymphoma of MALT type (mucoid-associated lymphoid tissue)., Treatment and Course: Postoperative staging indicated no further manifestation of the lymphoma. As no radical operation in resecting the tumour had been performed, combined radio- and chemotherapy was undertaken., Conclusion: Marginal B-cell lymphomas of the small intestine are only rarely seen in central Europe. Despite its usually slow growth this non-Hodgkin lymphoma of low malignancy can produce an acute mechanical ileus without prodromal symptoms. A multimodal therapeutic approach is often employed, but there are no established treatment strategies.