Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis

Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course. A co...

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Bibliographic Details
Published in:PloS one 2019-06, Vol.14 (6), p.e0216697-e0216697
Main Authors: Buchinsky, Farrel J, Valentino, William L, Ruszkay, Nicole, Powell, Evan, Derkay, Craig S, Seedat, Riaz Y, Uloza, Virgilijus, Dikkers, Frederik G, Tunkel, David E, Choi, Sukgi S, Mortelliti, Anthony J, Campisi, Paolo, Ospina, Juan C, Donne, Adam J, Sataloff, Robert T, Conley, Stephen F, McClay, John E, Friedman, Ellen M, Elden, Lisa, Tylor, Dale A, Rosen, Clark A, Smith, Libby J, Copley, Graeme J, Karas, David E, Schweinfurth, John M, Myer, 3rd, Charles M, Wiatrak, Brian J, Dohar, Joseph E, Sobol, Steven E, Bastian, Robert W, Smith, Richard J H, Smith, Marshall E, Wassie, Abebe M, Post, James C, Ehrlich, Garth D
Format: Article
Language:English
Subjects:
Adult
Age
Age Factors
Algorithms
Bayesian analysis
Biology and life sciences
Child, Preschool
Children
Children & youth
Communication
Condylomata Acuminata - epidemiology
Data analysis
Development and progression
Diagnosis
Diagnostic systems
Disease age factors
Female
Health care networks
Hospitals
Human papillomavirus
Human papillomavirus 11 - physiology
Human papillomavirus 6 - physiology
Humans
Infant
Infant, Newborn
Information management
Larynx
Male
Medical diagnosis
Medicine
Medicine and Health Sciences
Mothers
Otolaryngology
Papilloma
Papillomavirus infections
Papillomavirus Infections - diagnosis
Papillomavirus Infections - epidemiology
Papillomavirus Infections - surgery
Papillomavirus Infections - virology
Parenting
Pediatrics
People and Places
Rare diseases
Recurrence (Disease)
Respiratory tract
Respiratory tract diseases
Respiratory Tract Infections - diagnosis
Respiratory Tract Infections - epidemiology
Respiratory Tract Infections - surgery
Respiratory Tract Infections - virology
Statistical analysis
Surgery
Tumors
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Description
Summary:Recurrent Respiratory Papillomatosis (RRP) is a rare disease characterized by the growth of papillomas in the airway and especially the larynx. The clinical course is highly variable among individuals and there is poor understanding of the factors that drive an aggressive vs an indolent course. A convenience cohort of 339 affected subjects with papillomas positive for only HPV6 or HPV11 and clinical course data available for 1 year or more, from a large multicenter international study were included. Exploratory data analysis was conducted followed by inferential analyses with frequentist and Bayesian statistics. We examined 339 subjects: 82% were diagnosed prior to the age of 18 years, 65% were infected with HPV6, and 69% had an aggressive clinical course. When comparing age at diagnosis with clinical course, the probability of aggressiveness is high for children under five years of age then drops rapidly. For patients diagnosed after the age of 10 years, an indolent course is more common. After accounting for confounding between HPV11 and young age, HPV type was minimally associated with aggressiveness. Fast and Frugal Trees (FFTs) were utilized to determine which algorithms yield the highest accuracy to classify patients as having an indolent or aggressive clinical course and consistently created a branch for diagnostic age at ~5 years old. There was no reliable strong association between clinical course and socioeconomic or parental factors. In the largest cohort of its type, we have identified a critical age at diagnosis which demarcates a more aggressive from less aggressive clinical course.
ISSN:1932-6203
1932-6203
DOI:10.1371/journal.pone.0216697