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1: J Paediatr Child Health. 2001 Oct;37(5):507-9.Click here to read Links

Fevers and mouth ulcers.

Teo J, Codarini M.

The Children's Hospital at Westmead, NSW, Australia.

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.

PMID: 11885719 [PubMed - indexed for MEDLINE]

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