Biomedical subjects
Publications and source records attributed to C Stey.
We report the case of a young patient presenting with cervical lymphadenopathy and interstitial pulmonary infiltrates due to Hodgkin's Disease. Although lung involvement regressed under chemotherapy, we observed new alveolar infiltrates during treatment. Steroid administration after exclusion of an infectious cause was followed by rapid clinical and radiological improvement, indicating the probable presence of pulmonary bleomycine toxicity.
BACKGROUND: Takayasu's arteritis is a chronic systemic inflammatory disease that usually affects the aorta, its primary branches and occasionally the pulmonary and coronary arteries. Female gender in reproductive age and Asian origin are known factors associated with higher disease prevalence. The clinical manifestations vary considerably and are typically caused by limb or organ ischemia illness and fever. The estimated incidence rate in the western world is 2.6 cases per million persons per year. Occasionally, exertional dyspnea can be the sole primary clinical manifestation of Takayasu's arteritis. CASE PRESENTATION: We report the case of a 57-year-old woman who was referred to our institution with increasing exertional dyspnea caused by pulmonary artery involvement in Takayasu's arteritis. In a review of the literature we discuss demographic data, clinical and radiographic findings and available therapeutic options. CONCLUSIONS: Dyspnea due to pulmonary artery involvement can be the initial symptom of Takayasu's arteritis. Simple clinical tests, including a complete pulse-status and blood pressure measuring at both arms can lead to the right diagnosis and should always be done beyond the auscultation of the heart and lungs in patients with dyspnea.
The Pilot study "Quality Development through the Internet" by the Horten Center is an integral part of a concept on dissemination of medical knowledge. It offers participants the possibility to compare interactively their own and their peers' performance with their competence and with accepted guidelines within a web-based Audit-Cycle. The participants can discuss the evidence-based recommendations which are offered. In addition, changes in behavior and care can be documented objectively.
Guidelines on the treatment of asthma emphasize the importance of long-term control of the disease. The leukotriene receptor antagonists are a new class of maintenance therapy for the treatment of chronic asthma. In Switzerland two of these receptor antagonists are available, zafirlukast (Accolate) and montelukast (Singulair). Compared with placebo leukotriene antagonists are active and improve mild-to moderate asthma in terms of improving lung function and symptoms and decreasing exacerbations. However compared to low dose inhaled steroids LTRA are less effective and more expensive. Inhaled steroids therefore remain the preferred and most cost-effective therapy for treating chronic asthma. Among patients requiring moderate to high doses of inhaled corticosteroids to maintain asthma control LTRA allow for reduction of the doses. The best response to LTRA show patients who have aspirin-sensitive asthma.
Asthma is a common, chronic inflammatory disease of the airways associated with pronounced health and economic consequences. Identification and control of asthma triggers and an adequate pharmacological therapy are main components. But for a successful asthma care consistent education is another critical aspect for management that has to be considered. In this article several practical considerations for managing asthma in adults, with emphasis on the importance of patient education and partnership in care are described.
Sarcoidosis manifests itself with different symptoms and can thereby follow an acute or chronic course. We will discuss one case each. Case 1 involves a 41-year-old patient with Loefgren's syndrome, showing the typical trias of erythema nodosum, bilateral hilar lymphadenopathy and acute polyarthritis. With systemic steroid treatment the symptoms disappeared rapidly. In case 2 we present a 39-year-old-man with chronic sarcoidosis for several years with no vital threat. His organ involvement includes chronic dacryocystitis. Systemic steroids relieved his symptoms and the dacryo-rhinostomy could perhaps have been prevented had the therapy been started earlier. Evaluating the diagnostic and therapeutic procedure can be difficult because there are no standard guidelines. A vital threat demands rapid diagnosis and effective therapy. The chronic illness requires a thorough evaluation of the appropriate therapy. We discuss the criteria for diagnosis, therapy and follow up.
The role of N-acetylcysteine (NAC) in the treatment of chronic bronchitis is unclear. Since a number of studies have been published on this topic, a systematic review of published studies seems justified. A systematic search (Medline, Embase, Cochrane Library, bibliographies, no language restriction) for published randomized trials comparing oral NAC with placebo in patients with chronic bronchitis was performed. Dichotomous data on prevention of exacerbation, improvement of symptoms and adverse effects were extracted from original reports. The relative benefit and number-needed-to-treat were calculated for both individual trials and combined data. Thirty-nine trials were retrieved; eleven (2,011 analysed patients), published 1976-1994, were regarded as relevant and valid according to preset criteria. In nine studies, 351 of 723 (48.5%) patients receiving NAC had no exacerbation compared with 229 of 733 (31.2%) patients receiving placebo (relative benefit 1.56 (95% confidence interval (CI) 1.37-1.77), number-needed-to-treat 5.8 (95% CI 4.5-8.1). There was no evidence of any effect of study period (12-24 weeks) or cumulative dose of NAC on efficacy. In five trials, 286 of 466 (61.4%) patients receiving NAC reported improvement of their symptoms compared with 160 of 462 (34.6%) patients receiving placebo (relative benefit 1.78 (95% CI 1.54-2.05), number-needed-to-treat 3.7 (95% CI 3.0-4.9)). With NAC, 68 of 666 (10.2%) patients reported gastrointestinal adverse effects compared with 73 of 671 (10.9%) taking placebo. With NAC, 79 of 1,207 (6.5%) patients withdrew from the study due to adverse effects, compared with 87 of 1,234 (7.1%) receiving placebo. In conclusion, with treatment periods of approximately 12-24 weeks, oral N-acetylcysteine reduces the risk of exacerbations and improves symptoms in patients with chronic bronchitis compared with placebo, without increasing the risk of adverse effects. Whether this benefit is sufficient to justify the routine and long-term use of N-acetylcysteine in all patients with chronic bronchitis should be addressed in further studies and cost-effectiveness analyses.
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The level of D-dimer in the blood reflects the level of lysed, cross-linked fibrin, and is useful in the diagnosis of clinically suspected deep venous thrombosis and pulmonary embolism. We compared two assays for the measurement of D-dimer levels, the whole-blood immunoassay SimpliRED which can be performed in two minutes, and the plasma immunoassay NycoCard. D-dimer levels were determined with these two techniques in 100 patients. With clinically suspected deep vein thrombosis and positive D-dimer levels a compression ultrasonography using a colour coded Duplex instrument, or a phlebography were done. For clinically suspected pulmonary embolism a ventilation/perfusion scan was done in patients with normal x-ray. Patients with radiographic abnormalities were investigated with spiral computed tomography or pulmonary arteriography. When both assays showed negative results a diagnostic procedure was only done if there was a high clinical suspicion for thromboembolism. Both assays showed a correct positive result in seven of the eight diagnosed thromboembolic events and one false negative result each. The SimpliRED assay exhibited a sensitivity of 87.5% and a specificity of 61%, the negative predictive value was 98.3%, positive predictive value 15.9%. The NycoCard assay displayed also a sensitivity of 87.5%, specificity was lower with 39.6%, the negative predictive value was 97.4%, positive predictive value 10.7%. The SimpliRED assay can be a valuable tool when laboratory facilities for a plasma immunoassay are not accessible. The negative predictive value is very high, therefore this test is considered to be helpful for excluding a thromboembolic event.
The association of bronchiolitis obliterans organizing pneumonia (BOOP) with polymyalgia rheumatica is rare, and only one case has previously been described. This study reports on the case of an 80 yr-old male who presented with malaise, nonproductive cough and exertional dyspnoea for several weeks, along with a history of bilateral shoulder and pelvic girdle pain of several months' duration. The chest radiograph revealed a pneumonic infiltrate in the right lower lobe, which was unresponsive to antibiotics. Bronchoscopy, bronchoalveolar lavage and a transbronchial lung biopsy established the diagnosis of BOOP. The patient improved consistently on steroids. As in other connective diseases, organizing pneumonia may be one of the early manifestations of polymyalgia rheumatica.
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A central factor in the pathogenesis of inflammatory and fibrotic lung disease (adult respiratory distress syndrome, sarcoidosis, idiopathic pulmonary fibrosis) is the locally elevated number of alveolar macrophages (AM). An elevation in the production rate of AM, chemoattraction and differentiation of monocytes, or a diminution in the death rate might be underlying mechanisms. The aim of the present study was to investigate the modulatory role of endotoxin and cytokines on the death rate of human AM. Lipopolysaccharide (LPS) treatment resulted in a 4-fold increase (7.6 to 30.2%) of AM death. AM death was apoptotic as assessed by in situ DNA end labeling (ISDE), transmission electron microscopy, DNA gel electrophoresis, fluorometry of fragmented DNA, and an ELISA specific for histone-associated DNA fragments. Among the different bacterial cell wall components tested, LPS was the only inducer of apoptosis in human AM. None of the tested cytokines (interleukin-1 beta [IL-1 beta], IL-4, IL-6, IL-10, tumor necrosis factor-alpha [TNF-alpha], transforming growth factor-beta 2 [TGF-beta 2], interferon-gamma [IFN-gamma], macrophage colony-stimulating factor [M-CSF], granulocyte colony-stimulating factor [G-CSF], and granulocyte-macrophage colony-stimulating factor [GM-CSF]) was capable of enhancing the spontaneous rate of apoptosis. However, LPS-induced apoptosis was significantly enhanced by the macrophage-activating cytokine IFN-gamma, and reduced by the macrophage-deactivating cytokines IL-4, IL-10, and TGF-beta.
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.
A 71-year-old woman with a seven-year history of seronegative polyarthritis had to be intubated and ventilated because of acute respiratory failure. The chest radiograph and computed tomography revealed bilateral pleural effusions and massive enlargement of the thyroid which compressed the trachea. Concentration of the thyroid-stimulating hormone was slightly reduced, while that of peripheral thyroid hormone was normal. Histological examination of a goitre specimen after strumectomy showed macrofollicular goitre with massive amyloid deposits. Amyloid was subsequently also demonstrated in the gastrointestinal tract. The patient died 5 months later from respiratory failure. A second patient, 47 years old at hospitalization, had been suffering from arthritic psoriasis since she was a child and had been undergoing haemodialysis for renal failure caused by amyloid. She had undergone angiography because the haemodialysis shunt had become occluded. She had been admitted because of an increase in goitre size during the last 6 months and a malignancy was suspected. Computed tomography showed the thyroid enlargement with retrosternal extension and a nodule in the thyroid isthmus. After injection of contrast medium for the angiography she developed, on the basis of probably already existing hyperthyroidism with an increased level of thyroid-stimulating immunoglobulin, clinically and biochemically manifest hyperthyroidism, which was treated with thiamazole (15 mg daily) and propranolol (40 mg twice daily). Cytological examination of a fine-needle biopsy of thyroid tissue revealed amyloid deposits. The patient died 4 years later, shortly after bilateral hip replacement for femoral neck fractures. At autopsy a large amyloid goitre and amyloid deposits in the kidneys, gastrointestinal tract and coronary arteries were found.
A case of a malignant peritoneal mesothelioma in a 63-year-old male patient with a history of exposure to Thorotrast in 1945 is presented. There was no history of exposure to asbestos. The clinical manifestation was a serosal effusion, which required weekly ascites puncture until therapy with intraperitoneal bleomycin was initiated. The latter treatment led to a significant reduction of ascites without any influence on tumor progression. Unfortunately, intraperitoneal bleomycin was accompanied by pulmonary toxicity, but at a higher total dose than known for intravenous administration. Three years after diagnosis the patient is still alive, without relapse of ascites production after bleomycin had to be stopped. Considering the risk of pulmonary fibrosis with high-dose intraperitoneal bleomycin and the lack of efficacy on tumor reduction, bleomycin seems to offer no advantage with respect to cisplatin.
Autopsy findings are reported of a 51-year-old female patient with a 40-year history of mutilating psoriatic arthritis. As a result of this disease the patient developed reactive amyloidosis (AA-type) with generalized vascular involvement, amyloid kidneys and unusual amyloid goitre. The cause of death was ischaemic left ventricular failure due to massive amyloid deposits in the coronary arteries and left ventricular hypertrophy, the latter resulting from renal hypertension. Thus, amyloidosis and ischaemic heart disease were late complications of the underlying inflammatory disease.
A 34-year-old, previously healthy Yugoslav developed relapsing generalized anaphylactic reactions associated with life-threatening laryngospasm. After successful life support with volume-replacement, epinephrine, antihistamines and steroids, clinical workup disclosed a slight tenderness in the right upper abdomen and sonography a polycystic tumor with coarse calcification within the wall. The presumptive diagnosis of cystic liver echinococcosis with spontaneous rupture of a hydatid cyst was confirmed surgically and serologically. This case history shows that liver echinococcosis should be considered in otherwise unexplained anaphylaxis.
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