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The clinical manifestations of HIV infection in children involve a broad spectrum of conditions ranging from mild symptoms to AIDS. Knowledge of the disease and survival patterns of these children are needed to plan for future needs and develop baseline information to evaluate newer prophylactic or therapeutic management options.To identify AIDS-defining conditions and estimate post-AIDS diagnosis survival among HIV-infected children.Disease patterns and survival after the diagnosis of AIDS-defining conditions were studied in 126 children who were identified through a multisite university-based active surveillance system in California from January, 1989, through August, 1993. Hospital medical records were periodically reviewed and data were abstracted onto standardized forms designed for pediatric HIV surveillance. We determined the length of survival between AIDS diagnosis and death and evaluated the impact of disease patterns on survival using Kaplan-Meier's product-limit method and Cox proportional hazards regression.The median age at diagnosis was 13 months for children with perinatally acquired infection and 101.5 months for children infected through other routes of transmission. Pneumocystis carinii pneumonia and lymphoid interstitial pneumonia were the most common AIDS-defining conditions among perinatal cases, whereas the disease patterns observed among nonperinatal cases were more varied. The median postdiagnosis survival for the cohort was 26 months.Survival time did not differ significantly by race/ethnicity, sex or route of transmission. Respiratory candidiasis and wasting syndrome had significant negative impact on survival but P. carinii pneumonia was not associated with shorter survival. Zidovudine or other antiviral therapies was associated with increased survival.