Open heart surgery in a hemophiliac patient. Hematologic management.
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Biomedical subjects
Publications and source records attributed to Y Ravindranath.
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The neuroblastic tumours originate from primordial neural crest cells that normally develop into sympathetic nervous system, including the adrenal medulla. Neuroblastoma is the most intriguing pediatric neoplasm displaying diverse clinical and biologic characteristics and natural history. It has the highest rate of spontaneous regression of all human cancers, yet exhibits extremely malignant behaviour in older children with regional and disseminated disease. In the last 30 years, only a nominal improvement has occurred in the outlook of older children with metastatic disease at diagnosis. Tremendous gains in understanding of the biology of neuroblastoma in recent years have led to development of risk-related therapy based on age, stage and biological characteristics of neuroblastoma.
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Infantile or congenital cases of thrombotic microangiopathy have been reported that were familial and characterized by ongoing microangiopathic hemolysis and thrombocytopenia in the absence of regular fresh-frozen plasma transfusions. The authors describe a child with congenital microangiopathic hemolytic anemia and thrombocytopenia (CMHAT) who has received regular fresh-frozen plasma transfusions since infancy and has never had thrombotic complications. von Willebrand factor (vWF)-cleaving protease activity was studied in the patient's pretransfusion and posttransfusion plasma samples as well as in her parents' plasma. The effects of the patient's and a control subject's plasma on human microvascular endothelial cells were also investigated. Unusually large vWF multimers were present in the patient's plasma both before transfusion (thrombocytopenic) and after transfusion. Unlike cases of chronic relapsing thrombotic thrombocytopenic purpura, vWF-cleaving protease activity was present and treatment of cultured human endothelial cells with the patient's plasma did not induce apoptosis. These findings suggest that the patient with CMHAT may represent a different group in the broad spectrum of thrombotic microangiopathies.
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