Acta medica Austriaca最新文献

Summary: We report on a 51-year-old female patient who was diagnosed in 2001 as suffering from plasmacytoma. The patient had had complaints of bilateral carpal tunnel syndrome in 1999, treated by a simple dissection without performing histological examination. Congestive heart failure had gradually developed since that time. In 2001 echocardiography revealed a pronounced thickening of the left ventricular wall with systolic and diastolic dysfunction. A κ-light chain M component and κ-light chain-restricted bone marrow plasmacytosis were detected. Amyloid deposits staining positive in the κ-light chain-restriction analysis were observed in a gastric biopsy. Taking into consideration all these findings, a plasmacytoma-associated systemic AL amyloidosis was diagnosed. Cyclophosphamide/prednisolone chemotherapy regimen led to complete haematological remission. Cardiac transplantation, combined with autologous peripheral blood stem cell graft, was considered as the next therapeutic step, but the patient died while on the waiting list for transplantation. Autopsy detected a highly hypertrophic myocardium with narrowed heart cavities. Microscopic examination revealed dense, pink, acellular, Congo red-staining and κ-immunoperoxidase-positive AL amyloid masses splitting the cardiomyocytes. The present case is remarkable as it demonstrates that carpal tunnel syndrome and congestive heart failure could be symptoms of plasma cell dyscrasia-associated amyloidoses.

Summary: We present a case of an immature teratoma of the liver and the mediastinum. A 21-year-old female patient presented with dyspnea, abdominal pain, and abdominal mass. Abdominal ultrasonography showed a liver tumor which is located in the right lobe and composed of cystic and solid elements. Computed tomography (CT) also showed a big mass which contained multiple high density, nodular, semi-solid, heterogenic structures in the liver and another mass which contained multiple low density cystic lesions in the anterior mediastinum. Right thoraco-abdominal approach was performed on the patient and a complete macroscopic resection of tumors was achieved without complications. Both tumours were evaluated as having an intact capsule. The mediastinal tumour extended to the diaphragm on the right side, and is dissected from the ‘surrounding lung, pericardium’ and excised from diaphragm. Right hepatic lobectomy was performed for liver tumour. Intraoperative frozen sections of the liver tumor revealed teratoma. But the final pathological diagnosis was immature teratoma. Chemotherapy was given after surgery. The patient tolerated the procedure well and her postoperative course was unremarkable. At the first follow up 4 months after surgery she was alive and well and there was no evidence of recurrence, but the patient died within seven months with hepatic recurrence and spreading metastasis.

Zusammenfassung: Bei Patienten mit chronischer Polyarthritis finden sich nicht nur eine gelenksnahe Osteopenie und Knochenerosionen, sondern auch eine generalisierte axiale und periphere Osteoporose, den entzündeten Gelenken weit entfernten Stellen. Die Pathogenese der Knochendichteverminderung bei chronischer Polyarthritis ist multifaktoriell begründet; die Krankheitsaktivität ist sicherlich ein bestimmender Faktor für den Knochenverlust. Weitere pathogenetisch relevante Faktoren sind die entzündungshemmende Therapie (insbesondere Glukokortikoide), die reduzierte Mobilität und ein Östrogen- und/oder Androgenmangel. In den letzten Jahren wurden die Gegenspieler Receptor activator of nuclear factor κB-ligand und Osteoprotegerin als zentrale Regulatoren der Osteoklastenentstehung- und aktivierung identifiziert. Die Produktion von Receptor activator of nuclear factor κB-ligand und Osteoprotegerin wird von verschiedensten Zytokinen, Wachstumsfaktoren und Hormonen beeinflußt. Im Synovium von Patienten mit chronischer Polyarthritis fördern Fibroblasten und aktivierte T-Zellen durch vermehrte Expression von Receptor activator of nuclear factor κB-ligand die Entstehung und Aktivierung von Osteoklasten. Infolgedessen stellen Osteoprotegerin und Receptor activator of nuclear factor κB-ligand wichtige molekulare Bindeglieder zwischen dem Immunsystem und dem Knochenmetabolismus bei chronischer Polyarthritis dar.

Pathogenesis of Osteoporosis in Rheumatoid Arthritis

Summary: Osteoporosis is a major clinical problem in rheumatoid arthritis. Patients with rheumatoid arthritis frequently not only present with juxtaarticular osteopenia and bone erosions but also with generalized axial and appendicular osteoporosis at sites distant from inflamed joints. The pathogenesis of bone loss in rheumatoid arthritis is multifactorial; disease activity certainly is a major determinant of bone mass. Further pathogenetic factors include effects of anti-inflammatory therapies (in particular glucocorticoids), reduced mobility, estrogen and/or androgen deficiency. Recently, receptor activator of nuclear factor κB ligand (RANKL) and osteoprotegerin (OPG), a decoy receptor for receptor activator of nuclear factor κB ligand, were identified as central regulators of osteoclast recruitment and activation. Osteoprotegerin and receptor activator of nuclear factor κB ligand production is modulated by several cytokines, growth factors and hormones. In rheumatoid synovium both fibroblasts and activated T cells express receptor activator of nuclear factor κB ligand and thereby promote osteoclast recruitment and activation. Thus, osteoprotegerin and receptor activator of nuclear factor κB ligand appear to represent important molecular links between the immune system and bone metabolism in rheumatoid arthritis.

Summary: Purpose : Report on outcome of intraluminal high-dose-rate iridium-192 (HDR-Ir ¹⁹² ) brachytherapy with or without external radiotherapy in patients with unresectable bile duct tumours suffering from symptoms of malignant obstructive jaundice. Material and methods : Fourteen patients (mean age: 63 years) who were unsuitable for surgical resection on preoperative evaluation/laparotomy or inoperable due to poor general condition were referred for palliative radiotherapy. After percutaneous transhepatic drainage, HDR-Ir ¹⁹² brachytherapy was performed with a single dose of 2.5 Gy. Brachytherapy was given twice a day with at least a 6-h interval for 2 days, 2 or 3 days apart, up to a total dose of 10 Gy. Five patients received small-volume external radiotherapy (RT) (45 – 50.4 Gy/1.8 Gy) additionally. Results : Palliation with relief of the aggravating symptoms of obstructive jaundice was achieved in all patients. The actuarial 2-year survival rate of all patients was 11.9 % with a median survival of 6.5 months. Patients treated with brachytherapy alone had a median survival of 4.5 months as compared with 6.5 months after combined internal and external irradiation (log rank, P = 0.95). Conclusion : Patients with advanced unresectable bile duct cancer face a dismal prognosis; however, biliary drainage, and intraluminal brachytherapy with or without external RT, seem to be able to improve quality of life in the remaining time span.

Summary: Today, positron emission tomography (PET) investigation using fluorodeoxyglucose (FDG) is the most sensitive and specific single modality established for localizing metastases of medullary thyroid cancer (MTC). Even at calcitonin serum levels below 20 pg/ml, tumour localization may be successful in individual cases. However, especially in such early tumour stages, the morphological correlation using PET is unsatisfactory. With the newly available fixed combination of PET and computed tomography (PET/CT), this drawback can be overcome as PET/CT allows a point-by-point correlation of pathological function with morphological structure. Conventional PET alone yielded an approx 80 % sensitivity and specificity in MTC: a result that will be surpassed by the combined PET/CT scanner.

Summary: Background : Previous studies have shown that some patients are not able to tolerate colonoscopy without sedation because of low pain threshold, anxiety, colonic sensitivity and anatomical variations. Benzodiazepines are most commonly used, often in combination with pethidine. Our study compares sedation with propofol to midazolam and colonoscopy without sedation. Methods : In this study 147 patients were examined. The patients were divided into three groups: The first group included patients in whom propofol was used for sedation, the second group included patients sedated with midazolam, and the third group was comprised of patients who received no sedation. Oxygen saturation and heart rate were recorded continuously by pulse oxymetry. Arterial blood pressure (BP) was monitored at 3-min intervals. Results : A decrease in blood pressure and heart rate was documented in the first two groups ( P < 0.001), whereas in group 3 we found an increase in both BP and heart rate ( P < 0.001). Oxygen saturation dropped below 90 % in 11/102 patients sedated with propofol and in 9/23 sedated with midazolam. Two of 22 patients in whom no sedation was used were found to have oxygen saturation below 90 %. The recovery time was shorter (7 min) in group 1 (propofol) than in group 2 (midazolam), in which it was found to be 20 min. Conclusions : Our results showed that propofol provided good sedation with excellent pain control, a short recovery time and no significant haemodynamic side effects.

Summary: Background : We assessed how many patients on long-term oral anticoagulation (OAC) (i) suffer from arterial hypertension (AH), (ii) are aware of AH, (iii) need improvement of their therapy and (iv) suffer from cerebral bleeding. Methods : Outpatients on long-term OAC were asked to measure blood pressure at least 4 times. Blood pressure was classified as normotensive if at least 75 % of all measurements were < 139/89 mm Hg; as mild/moderate AH if > 25 % of all measurements were 140 – 179 mm Hg systolic or 90 – 109 mm Hg diastolic; and as severe AH if > 25 % of all measurements were > 180 mm Hg systolic or > 110 mm Hg diastolic. Bleeding complications were registered. Results : Of the 235 patients (108 female, 67 ± 12 years), 80 % suffered from AH. Severe AH was present in 5 %. Only 56 % were aware of suffering from AH. An improvement of antihypertensive therapy was needed in 64 %. Over 225 days, only one cerebral bleeding occurred. Blood pressure was normotensive in 30 % with known AH. Conclusions : Blood pressure control seems better in OAC patients than in normals, if the patient is aware of AH. Patients with AH on OAC are not aware of AH in > 50 %. Repeated blood pressure measurements in OAC are recommended, even if patients are not aware of AH.

Cryoglobulins are proteins, usually immunoglobulins that precipitate at temperatures less than 37 degrees C and redissolve on rewarming. Cryoglobulinaemias are classified according to the type of immunoglobulins and are associated with a number of disorders including plasma cell dyscrasias, lymphoproliferative disorders, autoimmune diseases and hepatitis C. The exact pathogenesis of the condition is not known. The clinical manifestations are varied and are due to ischaemia of tissues caused by the occlusion of vessel lumen. This review discusses the clinical manifestations, the current hypotheses of pathogenesis, the role of hepatitis C and finally the possibilities of treatment of the disease.

Unlabelled: This retrospective study was designed to shed light on the incidence and age-dependence of potentially bleeding pathologies in the upper gastrointestinal tract of asymptomatic patients scheduled for oral anticoagulant therapy. Gastroduodenoscopy was routinely performed during screening studies. The incidence of abnormalities was compared with gastroscopy findings of patients with epigastric symptoms. Only pathologies likely to bleed were considered. These included gastric ulcers, duodenal ulcers, esophageal varices, esophagitis, erosions, malformations and hemorrhages.

Results: 18.23% of the patients (n = 746) undergoing gastroscopy prior to scheduled oral anticoagulant therapy were found to present with abnormalities versus 18.44% of those with epigastric symptoms (n = 1,627). In the group scheduled for oral anticoagulant therapy, the rate of pathologies did not significantly increase with increasing age.

Conclusions: The unexpectedly high incidence of potentially bleeding pathologies in asymptomatic patients scheduled for anti-coagulant therapy should prompt screening gastroduodenoscopies irrespective of the patients' age prior to instituting treatment.