Dtsch Arztebl Int
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Polycythemias are characterized by an increased concentration of red blood cells. Because blood cell counts are a routine investigation, these disorders present to non-hematologic physicians. Polycythemia vera (PV), an acquired stem cell disease, is the most important variant. ⋯ Erythropoietin, which is produced in the kidneys, and its receptor system in the bone marrow, are of critical importance in polycythemia. Congenital polycythemias are caused by mutations of the Erythropoietin-receptor gene, hemoglobin variants, 2,3-bisphosphoglycerate mutase deficiency or by disturbances of renal oxygen sensing. Acquired polycythemias can occur secondary to hypoxia at high altitudes, or primarily through acquired mutations in the EPO-receptor signaling system (JAK2 mutations). Alternatively they may be caused by pulmonary or renal disease. An artificial erythrocytosis is induced by athletes through doping. Differential diagnosis comprises erythropoietin determination, JAK2 mutation analysis and if necessary hemoglobin electrophoresis. Only PV requires immediate treatment, because of a high thromboembolic risk. Epidemiological studies on polycythemias in German speaking countries are urgently needed.
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We report the case of a woman who presented to the medical emergency room with upper thoracic inlet syndrome six months after being treated for cancer of the left breast with surgery, radiation, and chemotherapy. A port-related occlusion of the superior vena cava was diagnosed on the basis of the history, physical findings, and diagnostic images. ⋯ Implanted port systems facilitate the treatment of the chronically ill by enabling easy and safe venous access. As the number of patients with such systems is growing, there is an increased need for optimized handling and care, awareness of the risks (such as catheter occlusion, thrombosis, and infection), and recognition of complications when they arise.
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Deep vein thrombosis is associated with a risk of pulmonary embolism and post thrombotic syndrome (PTS). ⋯ The most important therapeutic measure is prompt and adequate anticoagulation with heparin or fondaparinux. Thrombolysis or thrombectomy is only indicated in highly selected severe cases. The risk of PTS can be reduced by immediate ongoing treatment with compression stockings. Prevention of relapse is achieved using vitamin K antagonists with a target INR of 2.0 to 3.0. The duration of anticoagulation should be tailored to the localisation and etiology of the thrombosis, from at least three months to indefinite treatment. The ongoing risk of bleeding secondary to anticoagulation should be reevaluated at regular intervals as a cost-benefit analysis. New anticoagulants for acute and long term treatment will soon be available for clinical use.
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The treatment of preterm infants at the lower margin of viability is carried out amid growing tension between increasing survival rates, uncertain clinical outcomes, and financial and ethical considerations. The three German speaking countries have released guidelines on this issue, based on a previous common guideline. That is why the differences in national guidelines between the three countries is of peculiar interest in respect of medical ethics. ⋯ All three guidelines call for a pragmatic approach. National guidelines can only predetermine the framework, with long-term collection of sound local data on morbidity and mortality forming a prerequisite for decision-making, and also in discussions with parents.