A young male patient (NYHA III) presented with a severe pulmonary artery bifurcation stenosis of unknown origin. He underwent successful radical resection and endarterectomy of abundant endoluminal and perivascular inflammatory tissue. Surgery was performed with extracorporeal circulation (ECC) without transection of the aorta and the pulmonary artery bifurcation was enlarged with a patch. Right ventricular to peripheral pulmonary artery pressure gradient fell from 80 mmHg preoperatively to 25 mmHg 18 months postoperatively. The patient is fully rehabilitated and working (NYHA I). Histological examination confirmed Takayasu's arteritis as the underlying disease and medium-term follow-up angiography showed an almost normal pulmonary artery bifurcation. We conclude that the radical surgical approach is a valuable palliation in this disease.
An unusual case of Sjögren's syndrome presenting with severe anemia as the predominant clinical feature is described. Histological examination of a bone marrow biopsy specimen demonstrated that the patient's anemia was caused by myelitis and vasculitis of the small intraosseous vessels. Our report might stimulate a more thorough investigation of bone marrow in patients with connective tissue diseases and anemia.
Pneumonia was diagnosed radiologically in three patients (43, 54 and 58 years old, respectively), presenting with temperatures between 39 degrees and 40 degrees C, cough and weight loss. These signs persisted for 6, 7 and 13 weeks, respectively, but the pathogens could not be cultivated. Lung function analysis showed partial respiratory insufficiency with extensive restrictive impairment of ventilation. Samples of lung tissue were obtained in all three cases and histology revealed fibrosing alveolitis. In two patients serology yielded antibody titres of 1:512 and in one patient of 1:128, against Legionella pneumophila. Treatment with 1 g erythromycin three times daily was unsuccessful. Therefore, the patients were given prednisone at an initial dosage of 50-100 mg which was subsequently reduced. Lung function normalised during this treatment course, radiological findings and antibody titres receded. Hence, treatment with corticosteroids should be attempted if there is an urgent suspicion of fibrosing alveolitis caused by Legionella pneumophila, after having excluded a florid infectious pneumonia and after failure of erythromycin treatment.
32 forceps biopsies were performed in 30 patients with obstructive jaundice during percutaneous transhepatic biliary drainage procedures. In one patient an adequate specimen could not be obtained. In 25 of the remaining 31 cases diagnosis was confirmed histologically (malignant tumours: n = 22, benign stricture: n = 3). In 6 patients false-negative results were obtained. Transluminal biopsy is an easily performed adjunct to percutaneous transhepatic diagnostic interventions with minimal additional discomfort for the patient. In many cases percutaneous needle biopsy can be avoided. Forceps biopsy enables nonoperative histological diagnosis of small carcinomas of the bile ducts.
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