A Case of Job Syndrome-Experience with Thymopentin.
- Author:
Myung Hyun SOHN
1
;
Kyung Hwa PARK
;
Byung Ju JEONG
;
Kyu Earn KIM
;
Ki Young LEE
Author Information
1. Department of Pediatrics Yonsei University College of Medicine, Seoul, Korea.
- Publication Type:Case Report
- Keywords:
Job syndrome;
Thymopatin
- MeSH:
Abscess;
Child;
Dermatitis;
Eczema;
Eosinophilia;
Female;
Humans;
Immunoglobulin E;
Job Syndrome;
Neutrophils;
Pneumonia, Staphylococcal;
Skin;
Thymopentin*
- From:Pediatric Allergy and Respiratory Disease
1997;7(1):122-129
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The Job syndrome is a primary immunodeficiency disorder characterized by markedly elevated serum IgE levels, recurrent severe infections of the skin and sinoplmonary tract, including staphylococcal pneumonias, chronic eczematoid dermatitis, coarse facial features, and mild eosinophilia. In 1966, Davis et al discribed two patients with severe eczema, chroic sinopulmonary infections, and recurrent staphylococcal abscesses originally. The reason for immunocompromise in the Job syndrome remains unclear, but a chemotactic defect in neutrophils has been described. We have experienced a case of Job syndrome in a 9-year-old girl who had suffered from severe recurrent multiple infections since 2 month of age. After treatment with thymopentin, she was improved clinically. A brief review of related literature is presented.