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:: Volume 21, Issue 3 (6-2016) ::
__Armaghane Danesh__ 2016, 21(3): 314-320 Back to browse issues page
Chediak-Higashi: a case report
A Paymard1, M Jawadi2, R Sadegh1, KH Oshvandi3, Z Zarei4, S Mirzaee5, Z Saidinejad6, M Behnam Moghaddam1, A Khalili7
1- Department of Nursing, Yasuj University of Medical Scince, Yasuj, Iran
2- Department of Nursing, Yazd University of Medical Scince, Yazd, Iran.
3- Department of Nursing, Hamedan University of Medical Scince, Hamedan, Iran,
4- Department of Nursing, Shiraz University of Medical Scince, Shiraz, Iran,
5- Department of Critical Care Nursing, Yasuj University of Medical Scince, Yasuj, Iran,6Department of infectious Disease, Yazd University of Medical Scince,Yazd , Iran
6- Department of infectious Disease, Yasuj University of Medical Scince, Yasuj, Iran
7- Department of Nursing, Hamedan University of Medical Scince, Hamedan, Iran. , arash5920@yahoo.com
Abstract:   (7714 Views)

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.

Keywords: Chediak higashi syndrome, blood disorders, prolonged fever
Full-Text [PDF 165 kb]   (1233 Downloads)    
Type of Study: case report | Subject: Special
Received: 2015/12/11 | Accepted: 2016/05/14 | Published: 2016/06/7
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Paymard A, Jawadi M, Sadegh R, Oshvandi K, Zarei Z, Mirzaee S, et al . Chediak-Higashi: a case report. ارمغان دانش. 2016; 21 (3) :314-320
URL: http://armaghanj.yums.ac.ir/article-1-1166-en.html


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Volume 21, Issue 3 (6-2016) Back to browse issues page
ارمغان دانش Armaghane Danesh
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