Hemolytic uremic syndrome （HUS） is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and patho-genetic classifications. New findings in genetics and, in ...Hemolytic uremic syndrome （HUS） is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and patho-genetic classifications. New findings in genetics and, in particular, mutations of genes that encode the complement-regulatory proteins have improved our understanding of atypical HUS. Similarly, the comple-ment proteins are clearly involved in all types of thrombotic microangiopathy： typical HUS, atypical HUS and thrombotic thrombocytopenic purpura （TTP）. Fur-thermore, several secondary HUS appear to be related to abnormalities in complement genes in predisposed patients. The authors highlight the therapeutic as-pects of this rare disease, examining both “traditional therapy” （including plasma therapy, kidney and kidney-liver transplantation） and “new therapies”. The latter include anti-Shiga-toxin antibodies and anti-C5 mono-clonal antibody “eculizumab”. Eculizumab has been recently launched for the treatment of the atypical HUS, but it appears to be effective in the treatment of typical HUS and in TTP. Future therapies are in phases Ⅰ and Ⅱ. They include anti-C5 antibodies, which are more purifed, less immunogenic and absorbed orally and, anti-C3 antibodies, which are more powerful, but potentially less safe. Additionally, infusions of recombinant complement-regulatory proteins are a potential future therapy.展开更多
<strong>Purpose:</strong> The purpose of this study is to provide technical information on commissioning the TrueBeam STx 6 MV flattening-filter free (FFF) beam in the RayStation treatment planning system ...<strong>Purpose:</strong> The purpose of this study is to provide technical information on commissioning the TrueBeam STx 6 MV flattening-filter free (FFF) beam in the RayStation treatment planning system (TPS) for stereotactic radiosurgery (SRS) and stereotactic body radiation therapy (SBRT) treatments. <strong>Methods:</strong> For beam modeling, percent depth dose curves, profiles and output factors for jaw-collimated fields and stereotactic cones as well as X-jaws transmission were measured. For multi-leaf collimator (MLC) modeling, MLC model parameters such as offset, gain, curvature, leaf tip width, tongue and groove and transmission were determined and output factors for MLC-collimated fields were measured. Absolute dose calibration was also performed. For beam model and MLC model validation, the American Association of Physicists in Medicine Task Group-119 plans, clinical SRS and SBRT plans and end-to-end testing were performed. <strong>Results:</strong> Beam characteristics of the 6 MV FFF beam agreed well with those in the literature. Validation results showed that our beam model and MLC model were acceptable for SRS and SBRT treatments. <strong>Conclusions:</strong> The technical information and dosimetric data provided in this study will be a useful reference for other clinics/institutions which will commission the same machine energy in the RayStation TPS.展开更多
文摘Hemolytic uremic syndrome （HUS） is a rare disease. In this work the authors review the recent findings on HUS, considering the different etiologic and patho-genetic classifications. New findings in genetics and, in particular, mutations of genes that encode the complement-regulatory proteins have improved our understanding of atypical HUS. Similarly, the comple-ment proteins are clearly involved in all types of thrombotic microangiopathy： typical HUS, atypical HUS and thrombotic thrombocytopenic purpura （TTP）. Fur-thermore, several secondary HUS appear to be related to abnormalities in complement genes in predisposed patients. The authors highlight the therapeutic as-pects of this rare disease, examining both “traditional therapy” （including plasma therapy, kidney and kidney-liver transplantation） and “new therapies”. The latter include anti-Shiga-toxin antibodies and anti-C5 mono-clonal antibody “eculizumab”. Eculizumab has been recently launched for the treatment of the atypical HUS, but it appears to be effective in the treatment of typical HUS and in TTP. Future therapies are in phases Ⅰ and Ⅱ. They include anti-C5 antibodies, which are more purifed, less immunogenic and absorbed orally and, anti-C3 antibodies, which are more powerful, but potentially less safe. Additionally, infusions of recombinant complement-regulatory proteins are a potential future therapy.
文摘<strong>Purpose:</strong> The purpose of this study is to provide technical information on commissioning the TrueBeam STx 6 MV flattening-filter free (FFF) beam in the RayStation treatment planning system (TPS) for stereotactic radiosurgery (SRS) and stereotactic body radiation therapy (SBRT) treatments. <strong>Methods:</strong> For beam modeling, percent depth dose curves, profiles and output factors for jaw-collimated fields and stereotactic cones as well as X-jaws transmission were measured. For multi-leaf collimator (MLC) modeling, MLC model parameters such as offset, gain, curvature, leaf tip width, tongue and groove and transmission were determined and output factors for MLC-collimated fields were measured. Absolute dose calibration was also performed. For beam model and MLC model validation, the American Association of Physicists in Medicine Task Group-119 plans, clinical SRS and SBRT plans and end-to-end testing were performed. <strong>Results:</strong> Beam characteristics of the 6 MV FFF beam agreed well with those in the literature. Validation results showed that our beam model and MLC model were acceptable for SRS and SBRT treatments. <strong>Conclusions:</strong> The technical information and dosimetric data provided in this study will be a useful reference for other clinics/institutions which will commission the same machine energy in the RayStation TPS.
