Fevers and mouth ulcers.

Abstract

Mouth ulcers are commonly caused by infection but may be due to neutropenia. The most common form of hyper-IgM syndrome is of X-linked inheritance and caused by CD40 ligand gene mutations. Consider hyper-IgM syndrome in a male child with recurrent bacterial or opportunistic infections, neutropenia, hypogammaglobulinaemia (IgG and IgA) and normal T- and B-cell counts. In X-linked hyper-IgM syndrome: - the serum IgM concentration is normal in about 50% of cases. - transient or persistent neutropenia occurs in 70% of cases. First-line therapeutic options for hyper-IgM syndrome include regular intravenous immunoglobulin and prophylactic trimethoprimsulphamethoxazole.

• Authors: J Teo, M Codarini
• Journal: Journal of paediatrics and child health (J Paediatr Child Health) Vol. 37 Issue 5 Pg. 507-9 (Oct 2001) ISSN: 1034-4810 [Print] Australia
• PMID: 11885719 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin M
• CD40 Ligand

Topics

• CD40 Ligand (genetics)
• Diagnosis, Differential
• Fever (immunology)
• Genetic Linkage
• Humans
• Hypergammaglobulinemia (diagnosis)
• Immunoglobulin M (blood)
• Infant
• Male
• Oral Ulcer (immunology)
• X Chromosome (genetics)