Objective: To explore the clinical manifestation, immune abnormality and outcome of disseminated Bacille Calmette-Guérin (BCG) infection in children.

Method: The clinical data of 18 children with disseminated BCG infection seen from January 2000 to December 2007 were analyzed retrospectively.

Result: Thirteen of the children were male among 18 patients. Disseminated infection first appeared in armpit lymph nodes ipsilateral to the vaccination site, then spread to lungs in 15, lymphnodes of mediastinum or abdominal cavity in 18, skin and soft tissues in 5, skeletons in 4, liver in 4, spleen in 8, kidney, adrenal gland or meninges in 3. Twelve children were diagnosed to have primary immunodeficiency; 3 had severe combined immunodeficiency (SCID); 7 had chronic granulomatous disease (CGD), 2 had IL-12/IFN-gamma passageway deficiency. Eleven of the 18 patients died, and the remaining 7 patients were followed up from 1 to 9 years and are alive at present, but presented recurrent skin and bone tuberculosis in 4 and recurrent other infection in 3.

Conclusion: Most Children with disseminated BCG infection had primary immunodeficiency. CGD and IL-12/IFN-gamma passageway deficiency accounted for considerable proportion, so special immune function should be detected in these patients. The prognosis was poor. The type of the immunodeficiency diseases should be identified in early stage and the specific immune treatment should be given to the patients.

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