| A Case of Wiskott-Aldrich Syndrome |
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Byoung Geun Lee, Soo Hee Chang, Soo Young Cho, Pyoung Han Hwang, Jung Soo Kim |
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Department of Pediatrics, Chonbuk National University Medical School, Chonju, Korea |
| Wiskott-Aldrich 증후군 1례 |
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이병근, 장수희, 조수영, 황평한, 김정수 |
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전북대학교 의과대학 소아과학교실 |
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| Abstract |
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Wiskott-Aldrich Syndrome is an X-linked disorder characterized by recurrent infection, thrombocytopenia and eczema. Various defects in cell-mediated immunity and deficient antibody response 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.
Immunological abnormalities are as follows:
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 PRP-T 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. |
| Key Words:
Wiskott-Aldrich Syndrome, Immunity |
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