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Disseminated Mycobacterium avium complex (DMAC) infection occurs in HIV-infected children and adults with advanced immunosuppression. Prophylactic therapy has been shown to prevent this infection in adults with low CD4 cell counts. The United States Public Health Service and the Infectious Disease Society of America recently published guidelines (USPHS/CDC guidelines) for the prevention of opportunistic infections, including DMAC infection, in children and adults with HIV infection. These guidelines incorporate age-specific CD4 counts at which DMAC prophylaxis should be used.To determine (1) the extent to which the USPHS/CDC guidelines are being followed and (2) current practices for the prevention and management of DMAC infection in HIV-infected children.A questionnaire was sent to 65 centers that specialize in the care of HIV-infected children.Forty-one of 65 centers responded to the questionnaire. A strikingly low rate of adherence to age-specific criteria for DMAC prophylaxis was found for the age groups <1 year and 1 to 2 years (34 and 39%, respectively), in contrast to good adherence for the age groups 2 to 6 years (80%) and >6 years (93%) (<0.0001). Reasons for lack of adherence to the guidelines included a perception of low risk of DMAC in the youngest age groups and difficulty administering additional medications to these patients. The survey also documented substantial variability in DMAC prophylactic and treatment regimens and in susceptibility testing of M. avium complex isolates.