Nihon rinsho. Japanese journal of clinical medicine
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Recent genetic and molecular technology have shown that genetic abnormalities related to the cardiac ion channels can be a cause of some hereditary arrhythmic diseases. Until now, advanced analysis has proceeded especially in congenital long QT syndrome, and six different subtypes have been identified in Romano-Ward syndrome and 2 subtypes in Jervell & Lange-Nielsen syndrome. Since the mechanism of QT interval prolongation in each subtype is based on the malfunction of different cardiac ion channels, the same pharmacological treatment may show different antiarrhythmic effects for each subtype. In this paper, we review some of the hereditary arrhythmic diseases and discuss the possibility of gene-specific treatment in such diseases.
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STI571(imatinib) selectively inhibits the ABL-tyrosine kinase, the activity of which is activated by the formation of chimeric BCR/ABL. Phase I study in USA showed a remarkable effectiveness of STI571 in interferon-refractory chronic myelogenous leukemia with little adverse effects. STI571 will plausibly become the first choice drug prior to stem cell transplantation and interferon in the treatment of this leukemia.