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Non-Hodgkin Lymphoma in a Child with Schimke Immuno-Osseous Dysplasia
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نویسنده
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Basiratnia Mitra ,Baradaran-Heravi Alireza ,Yavarian Majid ,Geramizadeh Bita ,Karimi Mehran
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منبع
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iranian journal of medical sciences - 2011 - دوره : 36 - شماره : 3 - صفحه:222 -225
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چکیده
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Schimke immuno-osseous dysplasia is a rare autosomal recessivemultisystem disorder characterized by steroid-resistantnephrotic syndrome, immunod eficiency, and spondyloepiphysealdysplasia. mutations in swi/snf2 related, matrix associated,actin dependent regulator of chromatin, subfamily a-like 1(smarcal1) gene are responsible for the disease.the present report describes, for the first time, a schimkeimmuno-osseous dysplasia child with smarcal1 missensemutation (r561h) and manifestations of intussusception secondaryto epstein-barr virus-negative non-hodgkin lymphoma,who expired due to septicemia following chemotherapy.the report emphasizes the necessity of more limited immunosuppressive protocols in schimke immuno-osseous dysplasiapatients with lymphoproliferative disorders
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کلیدواژه
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Schimke immunoosseous dysplasia ,lymphoproliferative ,intussusception
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آدرس
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shiraz university of medical sciences, Shiraz Nephro-Urology Research Center, ایران, University of British Columbia, Child and Family Research Institute, Department of Medical Genetics, Canada, shiraz university of medical sciences, Hematology Research Center, ایران, shiraz university of medical sciences, Department of Pathology, ایران, shiraz university of medical sciences, Hematology Research Center, ایران
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پست الکترونیکی
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m_basiratnia@yahoo.com
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Authors
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