[Posthepatic obstructive jaundice caused by primary extragonadal germ-cell tumor in a patient with glucose-6-phosphatase dehydrogenase deficiency]. / Posthepatische Cholestase infolge primär-extragonadaler Manifestation eines Keimzelltumors bei einem Patienten mit Favismus.

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.

[Pneumoretroperitoneum and bilateral pneumothorax after endoscopic biliary sphincterotomy]. / Pneumoretroperitoneum und beidseitiger Pneumothorax nach endoskopischer Sphinkterotomie.

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.

[Recurrent upper gastrointestinal bleeding in a 61 year-old man with infrarenal abdominal aortic aneurysm]. / Rezidivierende obere gastrointestinale Blutungen bei einem 61-jährigen Patienten mit infrarenalem Bauchaortenaneurysma.

A 61-year-old man was admitted to hospital due to recurrent upper gastrointestinal bleeding. Four weeks ago, he had been treated with epinephrine and endoclips by endoscopy due to an arterial gastrointestinal bleeding. The patient had a history of coronary and peripheral artery disease, diabetes, and an abdominal aortic aneurysm. Urgent endoscopy suggested the presence of an ulcus Dieulafoy but no definitive bleeding source could be seen. Due to ongoing melena an abdominal computer tomography was performed and a primary aortoduodenal fistula was suspected caused by the infrarenal abdominal aortic aneurysm. Laparatomy was undertaken emergently and an aortoduodenal fistula was found in the descending part of the duodenum. Repair of the duodenal rent was performed and the aortic aneurysm was replaced by a Dacron prosthesis. The patient was transferred to the intensive care unit. 4 days after initial admission, he died due to septic shock.

[Unilateral swollen leg caused by a retroperitoneal ganglioneuroma]. / Retroperitoneales Ganglioneurom als Differenzialdiagnose einer einseitigen Beinschwellung.

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.

[A 16-year-old patient with dysphagia]. / Dysphagie bei einem 16-jährigen Patient.

A 16-year-old man with an 8-year history of progressive dysphagia was referred to hospital. There was no specific finding in his family history. Physical examination was unremarkable. Complete blood count, serum electrolytes, and liver and kidney function tests were normal. Barium swallow revealed an extrinsic impression of the upper esophagus posteriorly. Magnetic resonance angiography demonstrated an aberrant origin of the right subclavian artery, leaving the aorta below the left subclavian artery. The artery had a retroesophageal course, causing the esophageal narrowing. Due to the persistence and worsening of the patient's symptoms, resection and reconstructive bypass surgery were recommended. Surgical correction was performed through a combined right supraclavicular incision and left posterolateral thoracotomy. After application of a vascular clamp, the aberrant right subclavian artery was ligated almost at its origin, and an end-to-side anastomosis was made with the right common carotid artery. At the end of the operative procedure, good pulses were palpated in the right radial artery. Postoperatively, the patient tolerated a regular diet without symptoms of dysphagia and was discharged on postoperative day 7.

[Fibrinogen-guided thrombolysis with urokinase of a portal and mesenterial venous thrombosis during an acute episode of ulcerative colitis]. / Fibrinogengesteuerte Urokinaselyse einer Portal- und Mesenterialvenenthrombose bei akutem Schub einer Colitis ulcerosa.

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.

[Subcutaneously tunnelled cuffed venous hemodialysis catheters in chronic renal failure]. / Permanent-implantierte subkutane Vorhofkatheter bei terminaler Niereninsuffizienz.

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.

[Lambert-Eaton myasthenic syndrome with acute respiratory failure associated with small cell lung cancer]. / Pseudomyasthene Krise eines Lambert-Eaton-Syndroms bei kleinzelligem Bronchialkarzinom.

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.

[Arrhythmogenic right ventricular dysplasia as a cause of "sudden cardiac death" with survival]. / Arrhythmogene rechtsventrikuläre Dysplasie als Ursache eines überlebten plötzlichen Herztodes.

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.

[Ileus of the small intestine in intestinal marginal-zone B-cell lymphoma of mucoid-associated lymphoid tissue (MALT)]. / Dünndarmileus bei intestinalem Marginalzonen-B-Zell-Lymphom des MALT.

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.

[Rescue thrombectomy after stent implantation in a degenerating aortocoronary bypass]. / Rescue-Thrombektomie nach Stentimplantation in einem degenerierten aortokoronaren Venenbypass.

BACKGROUND: Distal showering of atherosclerotic debris or thrombus is a feared event during percutaneous angioplasty of degenerated vein grafts. CASE REPORT: We report on a 66-year-old male patient with a history of coronary artery disease who was admitted into hospital with an acute coronary syndrome. He had had coronary artery bypass graft surgery in 1995. His chest pain resolved under treatment with aspirin, heparin, betablocker, and nitrate. A diagnostic angiogram demonstrated two critical stenoses of the left anterior descending graft. After a continuous infusion of tirofiban administered for 24 h prior to elective angioplasty, a JR 4SH 6 F guiding catheter (Cordis) was positioned. The lesions were crossed with a 0.014" Galeo F wire (Biotronik). A 4 mm x 16 mm stent graft (Jostent, Jomed) was deployed in the distal stenosis and a 4.5 mm x 32 mm self-expandable stent (Magic Wallstent, Boston Scientific) in the proximal stenosis. Thereafter, the patient suffered from chest pain, and the ECG showed ST elevation from V1 to V5. Angiography revealed total occlusion of the left anterior descending artery. An X-Sizer catheter (EndiCOR Medical, Inc.) was introduced and advanced through the vein graft with slow back-and-forth movements. After several aspirations, blood flow was significantly improved. A modest CK elevation (127 U/l) indicated a rather small loss of myocardial tissue. CONCLUSION: Percutaneous revascularization of narrowed aortocoronary saphenous vein grafts is associated with increased risk of distal embolization and "no reflow". The X-Sizer catheter system is compatible with commercially available guiding catheters, and capable of retrieving thrombotic debris after macroembolization from degenerated saphenous vein grafts including the native coronary artery.

Chemical and immunological characteristics of four different L-asparaginase preparations.

We studied the differences in protein composition and immunologic reactivity of two E. coli-derived L-asparaginase (l-Asp) preparations (I and II), Erwinia-Asp (III) and PEG-modified E. coli l-Asp (IV). On gel filtration, each of preparations I-III showed three major peaks at 100, 270 and 460 KD, all with enzyme activity, whereas PEG-Asp showed peaks at 35 and 220 KD. On SDS-PAGE one major subunit could be identified at 32 KD (I and II) or 40 KD (III), whereas PEG-modified l-Asp could only be detected by lowering the polyacrylamide concentration and gave a single band above 200 KD. Using a polyclonal rabbit antibody generated against preparation I, only the E. coli l-Asp preparations (I and II) formed precipitin lines on Ouchterlony double diffusion. After freezing and thawing, preparation IV also reacted with this antibody. In sera from patients treated with preparation I, antibodies (detected by ELISA) reacted with preparations I and II but not with preparations III and IV. These results indicate that Erwinia-Asp (III) and PEG-Asp (IV) are distinct from E. coli preparations (I and II) by molecular weight and immunological behavior. They also provide an experimental rationale for the use of Erwinia-Asp as well as PEG-Asp in E. coli Asp-sensitized patients.

Elimination of l-asparaginase in children treated for acute lymphoblastic leukemia.

The elimination of l-asparaginase (l-Asp) was studied in 8 children treated for acute lymphoblastic leukemia according to the CoALL 82 protocol. The patients received four doses of l-Asp as a single agent during induction chemotherapy. We studied the elimination of l-Asp during the first infusion in 1 child, during the second in 3, during the third in 2 and during the forth in 2 children. Using Western blot technique and an experimental rabbit antibody to l-Asp, we were able to detect a single band at 32 kilodaltons (KD) in the serum of all patients between 4 and 36 h after infusion. The molecular weight remained unchanged and no other bands occurred during the time of observation. The detection limit of this method was calculated to 5 micrograms/ml using radial immunodiffusion. Incubation of l-Asp with pooled normal human serum caused no degradation of the enzyme during 48 h at 37 degrees C. Neither the total enzyme nor fragments were detectable in the urine of the patients collected during 8 h after l-Asp infusion. From these results we conclude that l-Asp is not cleaved by proteases in humans. The enzyme is most probably eliminated by the reticuloendothelial system.