Pakistan Journal of Medical Sciences

Published by : PROFESSIONAL MEDICAL PUBLICATIONS

ISSN 1681-715X

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CASE REPORT

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Volume 24

April - June 2008 (Part-I)

Number  2


 

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Chediak-higashi syndrome

Emad uddin Siddiqui1, Shaheena Hanif2

ABSTRACT

Chediak Higashi Syndrome is a rare inherited autosomal recessive disorder of immune system. Susceptibility to infection due to phagocyte dysfunction ranges from recurrent skin infection to over whelming fatal systemic infection. A five years old male child was admitted on 31st March 2006 with the complaints of pallor, fever & ear discharge for 6 month. He was treated by several medications but had temporary relief. The ear discharge was bilateral, foul smelling and of yellowish color. Past history was significant with episodes of abscess involving the different parts of body. Myeloid precursors shows giant, purple stained granules mainly in metamyelocytes, band cells and mature neutrophils.

KEY WORDS: Chediak-Higashi syndrome, Pediatric, recurrent skin abscess.

Pak J Med Sci    April - June 2008 (Part-I)    Vol. 24 No. 2    328-330


1. Dr. Emad Uddin Siddiqui,
Sr. Instructor Pediatric ER. AKUH
2. Dr. Shaheena Hanif,
Sr. Registrar Pediatric CHK

Correspondence:

Dr. Emad Uddin Siddiqui,
R- 215-19 Alnoor Society,
Federal-B-Area,
Karachi - Pakistan.
E-mail: emaduz@yahoo.com

* Received for Publication: January 9, 2007
* Revision Received: December 27, 2007
* 2nd Revision Received: February 9, 2008
* Final Revision Accepted: February 12, 2008



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