Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis.
<h4>Background</h4>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 aggress...
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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Public Library of Science (PLoS)
2019-01-01
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| Series: | PLoS ONE |
| Online Access: | https://doi.org/10.1371/journal.pone.0216697 |
| _version_ | 1819140321269776384 |
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| author | Farrel J Buchinsky William L Valentino Nicole Ruszkay Evan Powell Craig S Derkay Riaz Y Seedat Virgilijus Uloza Frederik G Dikkers David E Tunkel Sukgi S Choi Anthony J Mortelliti Paolo Campisi Juan C Ospina Adam J Donne Robert T Sataloff Stephen F Conley John E McClay Ellen M Friedman Lisa Elden Dale A Tylor Clark A Rosen Libby J Smith Graeme J Copley David E Karas John M Schweinfurth Charles M Myer Brian J Wiatrak Joseph E Dohar Steven E Sobol Robert W Bastian Richard J H Smith Marshall E Smith Abebe M Wassie James C Post Garth D Ehrlich |
| author_facet | Farrel J Buchinsky William L Valentino Nicole Ruszkay Evan Powell Craig S Derkay Riaz Y Seedat Virgilijus Uloza Frederik G Dikkers David E Tunkel Sukgi S Choi Anthony J Mortelliti Paolo Campisi Juan C Ospina Adam J Donne Robert T Sataloff Stephen F Conley John E McClay Ellen M Friedman Lisa Elden Dale A Tylor Clark A Rosen Libby J Smith Graeme J Copley David E Karas John M Schweinfurth Charles M Myer Brian J Wiatrak Joseph E Dohar Steven E Sobol Robert W Bastian Richard J H Smith Marshall E Smith Abebe M Wassie James C Post Garth D Ehrlich |
| author_sort | Farrel J Buchinsky |
| collection | DOAJ |
| description | <h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusion</h4>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. |
| first_indexed | 2024-12-22T11:36:42Z |
| format | Article |
| id | doaj.art-9ae6df7ce2f54e3f81c43f2b54a50fdf |
| institution | Directory Open Access Journal |
| issn | 1932-6203 |
| language | English |
| last_indexed | 2024-12-22T11:36:42Z |
| publishDate | 2019-01-01 |
| publisher | Public Library of Science (PLoS) |
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| series | PLoS ONE |
| spelling | doaj.art-9ae6df7ce2f54e3f81c43f2b54a50fdf2022-12-21T18:27:25ZengPublic Library of Science (PLoS)PLoS ONE1932-62032019-01-01146e021669710.1371/journal.pone.0216697Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis.Farrel J BuchinskyWilliam L ValentinoNicole RuszkayEvan PowellCraig S DerkayRiaz Y SeedatVirgilijus UlozaFrederik G DikkersDavid E TunkelSukgi S ChoiAnthony J MortellitiPaolo CampisiJuan C OspinaAdam J DonneRobert T SataloffStephen F ConleyJohn E McClayEllen M FriedmanLisa EldenDale A TylorClark A RosenLibby J SmithGraeme J CopleyDavid E KarasJohn M SchweinfurthCharles M MyerBrian J WiatrakJoseph E DoharSteven E SobolRobert W BastianRichard J H SmithMarshall E SmithAbebe M WassieJames C PostGarth D Ehrlich<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusion</h4>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.https://doi.org/10.1371/journal.pone.0216697 |
| spellingShingle | Farrel J Buchinsky William L Valentino Nicole Ruszkay Evan Powell Craig S Derkay Riaz Y Seedat Virgilijus Uloza Frederik G Dikkers David E Tunkel Sukgi S Choi Anthony J Mortelliti Paolo Campisi Juan C Ospina Adam J Donne Robert T Sataloff Stephen F Conley John E McClay Ellen M Friedman Lisa Elden Dale A Tylor Clark A Rosen Libby J Smith Graeme J Copley David E Karas John M Schweinfurth Charles M Myer Brian J Wiatrak Joseph E Dohar Steven E Sobol Robert W Bastian Richard J H Smith Marshall E Smith Abebe M Wassie James C Post Garth D Ehrlich Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis. PLoS ONE |
| title | Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis. |
| title_full | Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis. |
| title_fullStr | Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis. |
| title_full_unstemmed | Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis. |
| title_short | Age at diagnosis, but not HPV type, is strongly associated with clinical course in recurrent respiratory papillomatosis. |
| title_sort | age at diagnosis but not hpv type is strongly associated with clinical course in recurrent respiratory papillomatosis |
| url | https://doi.org/10.1371/journal.pone.0216697 |
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