The epidemiology of juvenile onset recurrent respiratory papillomatosis derived from a population level national database.
OBJECTIVE
To develop a national database of cases of juvenile onset recurrent respiratory papillomatosis (JoRRP) in Canada, to calculate trends in incidence and prevalence of JoRRP from January 1994 to December 2007 at the national and regional level, and to mathematically model the natural history of JoRRP.


METHODS
Retrospective, multicenter study.


METHODS
Patient demographics, clinical presentation, human papillomavirus status, method and timing of treatment, and indicators of disease severity were captured with a standardized case report form. Operative records were retrospectively scored using the Derkay-Coltrera staging system for each operative intervention. Trends in incidence and prevalence of JoRRP from 1994 to 2007 were calculated at a national and regional level using national population census data. A multivariable mixed effects linear model was used to explore the effect of surgery-specific variables on the intersurgical interval. Nonlinear least-squares regression was used to model the natural history of JoRRP.


RESULTS
Development of a national database of children with JoRRP identified 243 cases who underwent 3,021 surgical procedures. The national incidence of JoRRP from 1994 to 2007 was 0.24 per 100,000 children aged 14 years and younger. The prevalence was 1.11 per 100,000 children. The natural history of JoRRP followed a nonlinear time course with 64% of cases having a decreasing annual rate of surgery over time.


CONCLUSIONS
A Canadian national database of children with JoRRP was successfully developed. Modeling of the natural history of JoRRP may have important clinical and research implications.
Campisi
Paolo
P
Hospital for Sick Children, Toronto, Ontario, Canada.
Hawkes
Michael
M
Simpson
Kim
K
D000293:Adolescent; D017668:Age of Onset; D002170:Canada / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007822:Laryngeal Neoplasms / Q000453:epidemiology*; D016018:Least-Squares Analysis; D016014:Linear Models; D008297:Male; D009364:Neoplasm Recurrence, Local; D010212:Papilloma / Q000453:epidemiology*; D015995:Prevalence; D012189:Retrospective Studies