A Case of Wiskott-Aldrich Syndrome.
- Author:
Byoung Geun LEE
;
Soo Hee CHANG
;
Soo Young CHO
;
Pyoung Han HWANG
;
Jung Soo KIM
- Publication Type:Case Report
- MeSH:
Antibody Formation;
Child, Preschool;
Eczema;
Hemorrhage;
Humans;
Immunity, Cellular;
Immunization;
Immunoglobulin A;
Immunoglobulin E;
Immunoglobulin M;
Male;
Purpura;
Skin Tests;
Wiskott-Aldrich Syndrome*
- From:Journal of the Korean Pediatric Society
1994;37(11):1615-1619
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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. 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 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.
