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This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 
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:: Volume 12, Issue 3 (8-2010) ::
J Babol Univ Med Sci. 2010; Volume 12 Back to browse issues page
Chediak-Higashi Syndrome Report a Case
J Ghaffari, * , H Karami, , M Ghasemi , Z Daneshmandi , E Mohammadzadeh
Abstract:   (11523 Views)
BACKGROUND AND OBJECTIVE: Chediak-higashi syndrome (CHS) is a rare disorder with multiorgan involvement and autosomal recessive inheritance. Clinical signs and symptoms include occulo-cutaneous albinism, bleeding tendency due to platelet dysfunction and recurrent bacterial infections. The aim of this report was to introduce a case with suitable prevention and treatment.
CASE: A 21 month-old girl was admitted to Abo-Ali Sina hospital because of fever and vomiting. She had been admitted three times with lower respiratory infection at 14th day, 1st month and 18th month of age and improved in every hospital admission. Clinical examination showed multiple hypo pigmentation in face and extremity. Iris was bluish and her hair was silver like color without strabismus and nystagmus. There was hepatosplenomegaly. Chediak- higashi syndrome was diagnosed on the basis of clinical signs and symptoms, ultrasonography and positive related diagnostic tests for CHS. The patient was treated with prophylactic antibiotic, vitamin C and referred to BM transplant center in Tehran for definite treatment.
CONCLUSION: Chediak-higashi syndrome is a rare primary immune deficiency syndrome which could have a favorable outcome with early diagnosis and treatment.
Keywords: Chediak-higashi, Albinism, Neutrophil granules
Full-Text [PDF 1016 kb]   (1728 Downloads)    
Type of Study: Research | Subject: Biochemical
Accepted: 2014/06/6 | Published: 2014/06/6


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Ghaffari, J, Karami, H, Ghasemi M, Daneshmandi Z, Mohammadzadeh E. Chediak-Higashi Syndrome Report a Case. J Babol Univ Med Sci 2010; 12 (3) :79-84
URL: http://jbums.org/article-1-3636-en.html


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Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Volume 12, Issue 3 (8-2010) Back to browse issues page
مجله علمی دانشگاه علوم پزشکی بابل Journal of Babol University of Medical Sciences

The Journal of Babol University of Medical Sciences is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
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