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Volume 36, Number 3, September 2003

Hyper-IgM syndrome: a case report

I-Jen Wang, Shiuan-Jenn Wang, Dah-Chin Yan, Syh-Jae Lin, Bor-Luen Chiang
Department of Pediatrics, Taipei Hospital, Department of Health, Taiwan, ROC



Hyperimmunoglobulin M syndrome is a rare primary immunodeficiency disorder. We report a case of a 6-month-old boy who suffered from developmental delays, frequent respiratory tract infection, and unusual fungal and bacterial infection. X-linked hyperimmunoglobulin M syndrome was ultimately diagnosed with decreasing immunoglobulin-G, A, and E (immunoglobulin G = 51.3 mg/dL, immunoglobulin A = 8.32 mg/dL, immunoglobulin E <17.5 mg/dL), elevating immunoglobulin M (immunoglobulin M = 140 mg/dL), and decreasing T-cell expression of the CD40 ligand over flow cytometry. Seizure episodes and hypotonia developed with greater signal intensity at the putamen in a brain magnetic resonance imaging, which is compatible with hypoxic ischemic encephalopathy. This is the youngest proven case of hyper-IgM syndrome in Taiwan ever reported.



J Microbiol Immunol Infect 2003;36:215-217.