[The cardiac effects of Anthracycline].
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Biomedical subjects
Publications and source records attributed to E Jantunen.
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A middle aged man showed retroperitoneal fibrosis with signs of vasculitis verified by biopsy of the retroperitoneal mass. Antineutrophil cytoplasmic antibodies (ANCA) with cytoplasmic staining pattern (cANCA) were strongly positive, although there were no clinical or histological signs of Wegener's granulomatosis. No cases of cANCA associated retroperitoneal fibrosis have been described except in connection with Wegener's granulomatosis. ANCA have been found to associate with any inflammatory disorders. This observation may broaden the clinical spectrum of cANCA positive vasculitides.
Mucormycotic infections caused by fungi of the families Rhizopus, Mucor or Absidia are rare and usually associated with diabetes or immunosuppression. We describe a patient with invasive necrotizing cutaneous mucormycosis caused by Absidia corymbifera shortly after allogeneic BMT. The infection was successfully treated with surgical debridement and liposomal amphotericin B for 6 weeks. Recognition of these rare infections requires a high index of suspicion. These patients should be evaluated with tissue biopsy and cultures and treated without delay.
Multiple myeloma is characterized by bone disease including osteoporosis, osteolytic lesions, pathological fractures and hypercalcaemia leading to pain, immobilization and decrease in the quality of life. Clodronate, a bisphosphonate, has been shown to be effective in the treatment of hypercalcaemia in patients with multiple myeloma. In addition, clodronate reduces the progression of osteolytic lesions and the amount of vertebral fractures and may also relieve pain in these patients. Recent studies suggest that oral clodronate should be considered in the adjunctive treatment of all patients with active multiple myeloma independently of the presence of bone lesions at diagnosis. Due to its safety and efficacy, clodronate seems to have gained an important role in the management of patients with multiple myeloma.
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Gray platelet syndrome (GPS) is a rare bleeding disorder characterized by thrombocytopenia, agranular gray platelets in blood films, and almost total absence of platelet alpha-granules and their constituents. We describe here a rare case of GPS with myelofibrosis and splenomegaly indicating extramedullary hematopoiesis. Splenectomy was followed by normalization of platelet count but the bleeding diathesis continued. Based on a follow-up of more than 15 years, the slight myelofibrosis in our patient seems to be non-progressive. We also summarize the major clinical and laboratory features of previously published cases of GPS in order to obtain more comprehensive understanding of this rare disorder. From the clinical point of view, the bleeding tendency in this syndrome generally varies from mild to moderate, and no specific treatment is usually needed. Careful examination of peripheral blood films is necessary for the diagnosis of this rare syndrome.
Giant platelet disorders (GPD) refer to rare, usually inherited states characterized by abnormally large platelets, thrombocytopenia and bleeding tendency of variable severity. This review summarizes major clinical and laboratory features of three GPDs (Bernard-Soulier syndrome, May-Hegglin anomaly and gray platelet syndrome). Differential diagnosis between immunological thrombocytopenia and GPDs is important. Although rare, giant platelet disorders should be borne in mind, since bleeding tendency in some individuals may be severe and knowledge of bleeding diathesis is of importance before delivery or surgical procedures also in less symptomatic individuals.
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Cryofibrinogenaemia refers to the presence of cold-precipitable proteins in plasma but not in serum. It is usually associated with malignancy, thromboembolic diseases or various inflammatory processes; rarely it may be essential. The most common clinical presentations of cryofibrinogenaemia are cold-intolerance, purpura, skin necrosis and ulcers. We describe a middle-aged woman with essential cryofibrinogenaemia, leukocytoclastic vasculitis, and chronic purpura for over 25 years with several exacerbations. In patients with otherwise unexplained purpura or skin necrosis, determination of plasma cryofibrinogen should be considered.
The purpose of this study was to examine the support provided to the relatives of aged people in hospitals by different sources, the demand for support among them and the role of nursing personnel in providing support. The study was conducted in April 1990. Questionnaires were completed by 96 relatives and 82 members of nursing personnel on nine wards. Both emotionally and informatively, the relatives were provided better support by unofficial than by official auxiliaries. The discontent concerned mainly informative support. The relatives felt that the nursing personnel had provided them less support than the personnel believed to have given. What was required was more information of the condition and the treatment of the aged person as well as general discussion with the personnel. The preparedness of the nursing personnel to provide support to relatives was considered better by relatives than by the personnel themselves.
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