Intravenous high dose methylprednisolone for thrombotic thrombocytopenic purpura.
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Biomedical subjects
Publications and source records attributed to S Ozsoylu.
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We report a case of protein C deficiency which presented with purpura fulminans. The inheritance of protein C deficiency is discussed and the importance of warfarin (Coumadin) treatment in this conditions is emphasized.
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Two hundred fourteen children with acute nonlymphoblastic leukemia (ANLL) were diagnosed between July 1975 to January 1987 in our center. Only 106 (50%) of the children could be treated. An initial hematologic remission was attained in 61 (58%) with a median duration of hematologic remission of 7.5 months and median survival of 12 months. Five patients who completed 36 months of therapy are still in initial remission, less than or equal to 84 months. Exophthalmos at presentation, which is clearly related to shorter duration of remission, was observed in 11% of these children. In two cases, it was the first sign of leukemia, 6 and 7 months prior to obvious bone marrow involvement. Twenty percent of the 61 patients who achieved remission discontinued their chemotherapy during the course of treatment while they were in remission, which indicates a different problem in the treatment of these patients in Turkey. Despite administration of similar treatment protocols, the prognosis of our patients was worse than the results observed in developed countries. We conclude that poor socioeconomic and nutritional conditions should also be considered among the high-risk factors for children with ANLL, as experienced in our patients with acute lymphoblastic leukemia (ALL).
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Five out of eight patients with congenital pure red cell aplasia (PRCA), who became refractory or did not respond to conventional prednisone treatment (2 mg/kg), responded to high-dose intravenous methylprednisolone administration. The drug was given daily 30 mg/kg for 3 days, 20 mg/kg for 4 days, then subsequently 10, 5, and 2 mg/kg for a week, followed by 1 mg/kg until the hemoglobin level reached 12 g/dl. In the other three patients, although normoblasts appeared in the bone marrow, hemoglobin elevation was not observed. These three patients finally responded fully to a dose of 100 mg/kg intravenously. Therefore, we believe that resistance to corticosteroids could be overcome by increasing the dose in patients with Diamond-Blackfan syndrome. Four of these patients have been off the treatment for 2-13 months. Seven recurrences occurred in three patients 6-9 months following discontinuation of the treatment. However, all again responded to the same regimen.
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