Clinical features of 17 cases of X linked agammaglobulinemia
HE Jian-Xin, ZHAO Shun-Ying, JIANG Zai-Fang
Department of Respiration, Beijing Childrens' Hospital Affiliated to Capital University of Medical Sciences, Beijing 100045, China (Email: hejianxin70@163.com)
Abstract：OBJECTIVE: To investigate clinical features of X-linked agammaglobulinemia (XLA) in children. METHODS: The medical records of 17 children with XLA between January 2001 and April 2007 were reviewed. RESULTS: The age at first diagnosis in 88.2% of patients was more than 6 years, with a mean of 7.7 years. Twelve patients(70.6%) presented first symptoms over 2 years old, with a mean of 4.2 years. Respiratory infections as first symptoms and complaints occurred in 64.7% of the patients and 35.3% of the patients presented with polyarthritis. Skin and soft tissue infections were rarely seen in less than 1 year old group children. Abrupt sepsis and abscess in deep tissues were seen in the older children. CD4+ T cells decreased and CD8+ T cells increased in 9 patients and an inversed ratio of CD4+/ CD8+ was observed in 11 patients. CONCLUSIONS: Both the age presenting first symptoms and the age at first diagnosis in children with XLA in this study were later than the reported data. Respiratory infection was the most common manifestation. High prevalence of polyarthritis was observed. Abnormal T cell phenotypes occurred in more than one half of patients.
