Title of article
GRISCELLI SYNDROME; A CASE REPORT AND REVIEW OF THE LITERATURE
Author/Authors
SHAMSIAN، B. SH. نويسنده , , Arzanian، M.T نويسنده , , Alavi، S. نويسنده Assistant professor, Department of pediatric hematology-oncology, Mofid childrens hospital, Shaheed Beheshti University of medical sciences , , Zareifar، S. نويسنده Assistant professor, Department of pediatric hematology-oncology, Namazi Hospital, Shiraz University of medical sciences ,
Issue Information
فصلنامه با شماره پیاپی - سال 2007
Pages
5
From page
47
To page
51
Abstract
bstract:
Griscelli syndrome (GS) is a rare disease first described in 1978. It is inherited in autosomal recessive pattern. This disease is characterized by partial albinism, pigmentation dilution, cellular immunodeficiency, neurological involvement & uncontrolled phases of macrophage & lymphocyte activation.
We report a 5 months Old Iranian girl presenting with silver-gray hair,eyelashes and eyebrows, hepatosplenomegaly, pancytopenia, hemophagocytosis and progressive neurologic deterioration. Griscelli syndrome can be suggested according to her symptoms. The chemotherapy was not effective for her and she died due to multi organ failure.
Journal title
Iranian Journal of Child Neurology (IJCN)
Serial Year
2007
Journal title
Iranian Journal of Child Neurology (IJCN)
Record number
676468
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