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Hemophilia growth and development study. Design, methods, and entry data

M W Hilgartner1, S M Donfield, A Willoughby

  • 1Division of Pediatric Hematology and Oncology, New York Hospital-Cornell Medical Center, New York 10021.

The American Journal of Pediatric Hematology/Oncology
|May 1, 1993
PubMed
Summary
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Human immunodeficiency virus (HIV) infection impacts children with hemophilia, increasing risks for abnormal growth and immune function. Chronic illness may also affect neuropsychological performance in these youth.

Area of Science:

  • Pediatric Health
  • Infectious Diseases
  • Hematology

Background:

  • Hemophilia is a genetic bleeding disorder.
  • Human immunodeficiency virus (HIV) infection poses significant health challenges.
  • Co-infection of HIV in children with hemophilia requires comprehensive health monitoring.

Purpose of the Study:

  • To investigate the impact of HIV infection on physical growth, sexual maturation, immune function, and neurological/neuropsychological functioning in children and adolescents with hemophilia.
  • To present baseline data from a multicenter longitudinal epidemiologic study.

Main Methods:

  • A cohort of 333 males aged 6–19 with moderate to severe hemophilia underwent baseline examination.
  • Participants were categorized into HIV-seropositive (62%) and HIV-seronegative (38%) groups.

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  • Data collected included physical measurements, sexual maturation assessments, immune markers, and neuropsychological tests.

    • Main Results:

    • HIV-positive participants showed significantly higher rates of poor height-for-age attainment (3x likelihood, p=0.05) and abnormal antibody levels (3x likelihood, p<0.001).
    • Delays in sexual maturation were observed more frequently in HIV-positive individuals (2x likelihood, p=0.414).
    • HIV-positive participants were 50% more likely to exhibit lower scores in three neuropsychological areas.

    Conclusions:

    • HIV infection is associated with increased likelihood of abnormal growth and immune dysfunction in children with hemophilia.
    • Neuropsychological deficits may be linked to the chronic illness burden in both HIV-positive and HIV-negative hemophilia patients.
    • Further data collection including a non-HIV infected, non-hemophiliac sibling comparison group is ongoing.