HEMOPHILIA GROWTH AND DEVELOPMENT STUDY - DESIGN, METHODS, AND ENTRY DATA

Purpose: The study design, research questions, and baseline data are p resented from a multicenter longitudinal epidemiologic investigation o f the impact of human immunodeficiency virus (HIV) infection on three areas of functioning in children and adolescents with hemophilia: phys ical growth and sexual maturation, immune function, and neurological a nd neuropsychological functioning. Patients and Methods: Sixty-nine pe rcent (n = 333) of a population of males between the ages of 6 and 19 years with moderate to severe hemophilia participated in a comprehensi ve baseline examination. Approximately 62% of the study group was HIV seropositive and 38% HIV seronegative. Results: Adjusted for age, HIV- positive participants were three times as likely as HIV-negative parti cipants to exhibit declines in height-for-age attainment (p = 0.05), t wice as likely to have delays in sexual maturation (p = 0.414), and mo re than three times as likely to exhibit antibody levels that were not indicative of prior exposure or vaccination (p < 0.001). In addition, analysis of a comprehensive battery of neuropsychological tests showe d that HIV-positive participants were 50% more likely to show scores a pproximately 1 SD below expected levels in three of nine functional ar eas (p = NS) compared with HIV-negative participants. Conclusions: Ent ry data for this study reveal a greater likelihood for HIV-positive ch ildren and adolescents with moderate to severe hemophilia to have abno rmal growth, and to exhibit antibody levels not indicative of prior ex posure to disease or vaccination, which could be attributed to HIV inf ection. Lowered neuropsychological test performance in both groups may be attributable to the deleterious effects of chronic illness. Data a re being collected from a group of non-HIV infected non-hemophiliac si blings for comparison.