Delays in maturation among adolescents with hemophilia and a history of inhibitors.
Inhibitory antibodies to factors 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 multicenter Hemophilia Growth and Development Study. A total of 333 children and adolescents (mean age, 12.4 years) were enrolled from 1989 to 1990 and followed for 7 years. Of these, 18% (n = 60) had a history of inhibitors. Bone age among HIV(-) adolescents with a history of inhibitors lagged 9 or more months behind those without inhibitors at every age from 12 to 15 years. 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 with those without inhibitors. Delays were greater among HIV(+) patients with a history of inhibitors compared with 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.
Donfield
Sharyne M
SM
Department of Biostatistics, Rho, Chapel Hill, NC, USA. sdonfield@rhoworld.com
Lynn
Henry S
HS
Lail
Alice E
AE
Hoots
W Keith
WK
Berntorp
Erik
E
Gomperts
Edward D
ED
D000293:Adolescent; D000328:Adult; D000906:Antibodies / Q000009:adverse effects; D019774:Blood Coagulation Factor Inhibitors / Q000009:adverse effects*; D001827:Body Height / Q000502:physiology; D001846:Bone Development / Q000502:physiology; D002648:Child; D015331:Cohort Studies; D005164:Factor IX / Q000276:immunology; D005169:Factor VIII / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006130:Growth Disorders / Q000453:epidemiology* / Q000209:etiology*; D015658:HIV Infections / Q000150:complications; D015497:HIV-1; D006467:Hemophilia A / Q000150:complications* / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D008297:Male; D011627:Puberty / Q000502:physiology; D013739:Testosterone / Q000097:blood
D000906:Antibodies; D019774:Blood Coagulation Factor Inhibitors; D013739:Testosterone; D005169:Factor VIII; D005164:Factor IX
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