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Compared with traditional echocardiographic parameters or LV strain, myocardial work (MW) derived from LV-PSL is a more advanced tool that combines deformation as well as hemodynamics through integration of global longitudinal strain and non-invasive LV systolic pressure.Over 95% of treated patients continued to be event free after 36 months, and 100% of patients survived this normally lethal disease. A self-inactivating lentiviral vector, EFS-ADA LV, was used to insert a functional ADA gene in autologous CD34+ hematopoietic stem and progenitor cells (HSPCs).
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