TY - JOUR JF - yums-armaghan JO - armaghanj VL - 21 IS - 3 PY - 2016 Y1 - 2016/6/01 TI - Chediak-Higashi: a case report TT - چدیاک هیگاشی: گزارش یک مورد N2 - Background & aim: Chédiak–Higashi syndrome, a rare autosomal recessive disorder characterized by immune deficiency, bleeding diathesis, recurrent bacterial infections, albinism dynamic and progressive neurological disorder, as well as major organs such as the liver and spleen lymphocytic infiltration is determined. The aim of this study was to report a case of a rare manifestation of the disease. Case Report: The case-study in the present report was a 4-month-old female that two months after vaccination had intermittent fever associated with fever, weakness, which lasted more than a week. After blood tests, ultrasound and examination of blood cells, Chédiak–Higashi syndrome diagnosis was made. In terms of blood cells, pancytopenia, platelets and fresh frozen plasma were administered to the patient to prevent bleeding. After Buttock infections, the patient was suffering from septicemia and septic shock followed by a lack of response to resuscitation and cardiopulmonary arrest and then died. Conclusion: In this patient all Chediak Higashi syndrome, including long fever, loss of appetite, frequent infections, albinism variable and multiple neurological deficit was seen. Chdyak Higashi syndrome is treatable with early diagnosis and bone marrow transplantation. SP - 314 EP - 320 AU - Paymard, A AU - Jawadi, M AU - Sadegh, R AU - Oshvandi, KH AU - Zarei, Z AU - Mirzaee, S AU - Saidinejad, Z AU - Behnam Moghaddam, M AU - Khalili, A AD - Department of Nursing, Hamedan University of Medical Scince, Hamedan, Iran. KW - Chediak higashi syndrome KW - blood disorders KW - prolonged fever UR - http://armaghanj.yums.ac.ir/article-1-1166-en.html ER -