Submitted May 9, 2007
Accepted August 9, 2007
Delays in maturation among adolescents with hemophilia and a history of inhibitors
Sharyne M. Donfield*, Henry S. Lynn, Alice E. Lail, W. Keith Hoots, Erik Berntorp, and Edward D. Gomperts
Department of Biostatistics, Rho, Inc., Chapel Hill, NC, United States
Department of Biostatistics, School of Public Health, Fudan University, Shanghai, China
Gulf States Hemophilia & Thrombophilia Center, Houston Medical Center, Houston, TX, United States
Department for Coagulation Disorders, Malmo University Hospital, Malmo, Sweden
The Saban Research Institute, Childrens Hospital Los Angeles, Los Angeles, CA, United States
* Corresponding author; email: sdonfield{at}rhoworld.com.
Inhibitory antibodies to factor VIII or IX have the potential to affect a broad range of outcomes among people with hemophilia, however, their possible effect on growth and maturation has not been explored. We evaluated skeletal maturation (bone age), pubertal progression, serum testosterone levels, height velocity, and stature in the multi-center Hemophilia Growth and Development Study. Three hundred and thirty-three children and adolescents (mean age 12.4) were enrolled from 1989-90 and followed 7 years. Eighteen percent (n=60) had a history of inhibitors. Bone age among HIV-negative adolescents with a history of inhibitors lagged 9+ months behind those without inhibitors at every age from 12-15. Those with a history of inhibitors were older at every Tanner stage transition, attained a lower maximum growth velocity, and their serum testosterone levels were significantly lower compared to those without inhibitors. Delays were greater among HIV-positive subjects with a history of inhibitors compared to those without inhibitors; however, the differences were generally small and not statistically significant. The results of this investigation underscore the importance of monitoring the growth and maturation of children and adolescents with hemophilia, particularly those with inhibitors.
