Journal of the Korean Pediatric Society 1994;37(11):1615-1619.
Published online November 15, 1994.
A Case of Wiskott-Aldrich Syndrome
Byoung Geun Lee, Soo Hee Chang, Soo Young Cho, Pyoung Han Hwang, Jung Soo Kim
Department of Pediatrics, Chonbuk National University Medical School, Chonju, Korea
Wiskott-Aldrich 증후군 1례
이병근, 장수희, 조수영, 황평한, 김정수
전북대학교 의과대학 소아과학교실
Abstract
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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