Initial results from the national registry for juvenile-onset recurrent respiratory papillomatosis. RRP Task Force.
OBJECTIVE
To characterize the spectrum of juvenile-onset recurrent respiratory papillomatosis (RRP) in the United States and to obtain data about the natural course of the disease and its response to treatment.


METHODS
Twenty tertiary-care pediatric otolaryngology centers throughout the United States.


METHODS
All patients with active RRP aged less than 18 years at the participating sites.


METHODS
Number of surgical procedures performed per year, progression of papillomas to previously nondiseased anatomical sites, drug interventions and other adjuvant therapy, and need for tracheostomy.


RESULTS
Data were collected from 399 children enrolled from January, 1, 1997, through December 31, 1998. There were 51.9% male; 62.7% white, 28.3% black, 9.0% other or unknown racial group; 10.8% Hispanic ethnicity. Mean age at diagnosis was 3.8 years (range, 0.1-16.3 years) and mean duration of disease was 4.4 years (range, 0.03-18.9 years). The mean number of surgical procedures per child was 4.4 per year (range, 0.2-19.3 per year). Children whose RRP was diagnosed at younger ages (<3.0 years) were 3.6 times more likely to have more than 4 surgical procedures per year (P=.001) and almost 2 times more likely to have 2 or more anatomical sites affected (P=.008) than were children whose RRP was diagnosed at later ages (> or =3.0 years), after adjusting for sex, race, and years of treatment.


CONCLUSIONS
Children whose disease was diagnosed before age 3 years were more likely than children aged 3 years or older to have more severe disease as measured by the mean number of surgical procedures performed and by the number of anatomical sites affected. The registry will form the basis for future analysis on the outcome of disease, natural course of RRP under management strategies, prevention strategies, and public health importance.
Viral Exanthems and Herpesvirus Branch, Division of Viral and Rickettsial Diseases, National Center for Infectious Disease, Centers for Disease Control and Prevention, Atlanta, GA 30333, USA. lra0@cdc.gov
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LR
Armstrong
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Derkay
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Reeves
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D010212:Papilloma / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D011379:Prognosis; D012042:Registries / Q000706:statistics & numerical data*; D012086:Reoperation; D012142:Respiratory Tract Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D016896:Treatment Outcome