Journal article
Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis
PloS one, Vol.14(6), pp.e0216697-e0216697
06/13/2019
DOI: 10.1371/journal.pone.0216697
PMCID: PMC6563955
PMID: 31194767
Abstract
Background
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.
Methods
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.
Results
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 similar to 5 years old. There was no reliable strong association between clinical course and socioeconomic or parental factors.
Conclusion
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.
Details
- Title: Subtitle
- Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis
- Creators
- Farrel J Buchinsky - Respiratory Papillomatosis Program, Pittsburgh, United StatesWilliam L Valentino - Drexel UniversityNicole Ruszkay - Drexel UniversityEvan Powell - Pittsburgh Clinical Genomics Laboratory, Pittsburgh, United StatesCraig S Derkay - Department of Otolaryngology-Head and Neck Surgery, Norfolk, United StatesRiaz Y Seedat - Department of Otorhinolaryngology, Bloemfontein, South AfricaVirgilijus Uloza - Department of Otorhinolaryngology, Kaunas, LithuaniaFrederik G Dikkers - Ear, Nose and ThroatDavid E Tunkel - Johns Hopkins MedicineSukgi S Choi - Department of Otolaryngology and Communication Enhancement, Boston, United StatesAnthony J Mortelliti - Syracuse UniversityPaolo Campisi - Hospital for Sick ChildrenJuan C Ospina - Unit of Otorhinolaryngology and Maxillofacial Surgery, Bogotá, ColombiaAdam J Donne - Alder Hey Children's NHS Foundation TrustRobert T Sataloff - Drexel UniversityStephen F Conley - Department of Otolaryngology and Communication Sciences, Milwaukee, United StatesJohn E McClay - Frisco ENT for Children, Dallas, United StatesEllen M Friedman - Otolaryngology and Pediatrics, Houston, United StatesLisa Elden - Department Otolaryngology, Philadelphia, United StatesDale A Tylor - ENT Associates of Santa Barbara, Santa Barbara, United StatesClark A Rosen - Department of Otolaryngology, San Francisco, United StatesLibby J Smith - University of PittsburghGraeme J Copley - Boston Children's HospitalDavid E Karas - Yale UniversityJohn M Schweinfurth - Department of Otolaryngology, Jackson, United StatesCharles M Myer - Department of Otolaryngology-Head and Neck Surgery, Cincinnati, United StatesBrian J Wiatrak - Pediatric Otolaryngology, Birmingham, United StatesJoseph E Dohar - Division of Pediatric Otolaryngology, Pittsburgh, United StatesSteven E Sobol - Department Otolaryngology, Philadelphia, United StatesRobert W Bastian - Bastian Voice InstituteRichard J. H Smith - Division of Pediatric Otolaryngology, Iowa City, United StatesMarshall E Smith - University of UtahAbebe M Wassie - Addis Ababa UniversityJames C Post - Respiratory Papillomatosis Program, Pittsburgh, United StatesGarth D Ehrlich - Drexel University
- Resource Type
- Journal article
- Publication Details
- PloS one, Vol.14(6), pp.e0216697-e0216697
- DOI
- 10.1371/journal.pone.0216697
- PMID
- 31194767
- PMCID
- PMC6563955
- NLM abbreviation
- PLoS One
- ISSN
- 1932-6203
- eISSN
- 1932-6203
- Publisher
- PUBLIC LIBRARY SCIENCE
- Number of pages
- 19
- Grant note
- R01 DC008841 / National Institute on Deafness and Other Communication Disorders (NIDCD); United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute on Deafness & Other Communication Disorders (NIDCD)
- Language
- English
- Date published
- 06/13/2019
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
- Record Identifier
- 9984256932102771
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