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KMID : 0367419940370111615
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Journal of Korean Pediatric Society
1994 Volume.37 No. 11 p.1615 ~ p.1619
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A Case of Wiskott-Aldrich Syndrome
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이병근
장수희/조수영/황평한/김정수
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Abstract
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Wiskott-Aldrich Syndrome is an X-linked disorder characterized by recurrent infection, thrombocypenia and eczema. Various defects in cell-mediated immunity and deficient antibody reponse to carbohydrate antigens have been described. We
experienced
a
case of Wiskott-Aldrich Syndrome of 28 months old male patient. He has been suffered from multiple petechiae with bleeding, recurrent pyogenic infections and generalized eczema since 3 months of age.
@ES Immunological abnormalities are as follows:
@EN 1) Serum IgM was gradually decreased, while IgA and IgE were increased.
2) Antibody response against polysaccharide antigen (PRP) was not observed after 3 times of PRPT immunization.
3) CD4/CD8 ratio was reversed (0.6).
4) Proliferative response of mononuclear cells was significantly reduced, and CMI skin test also showed negative results.
A brief review of literature was made.
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KEYWORD
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