National Registry for Juvenile-Onset Recurrent Respiratory Papillomatosis
- 1 September 2003
- journal article
- research article
- Published by American Medical Association (AMA) in JAMA Otolaryngology–Head & Neck Surgery
- Vol. 129 (9), 976-82
- https://doi.org/10.1001/archotol.129.9.976
Abstract
Juvenile-onset recurrent respiratory papillomatosis (JORRP) is an infrequent but debilitating disease. Because JORRP is uncommon, it has proven difficult for studies at single institutions to accurately evaluate its natural history. To characterize the clinical spectrum of JORRP. Standardized retrospective and prospective medical record abstraction. Twenty-two tertiary-care pediatric otolaryngology centers throughout the United States. Patients All patients with JORRP younger than 18 years seen between January 1, 1996, and March 31, 2002. Demographics, age at diagnosis, anatomic sites of disease, longitudinal disease course, frequency of surgery, need for tracheotomy, and medication history. The registry includes 603 children. The mean age at diagnosis was 4.0 years. The children underwent a mean of 5.1 surgeries annually. Current age, rather than age at diagnosis, was the primary determinant of surgical frequency. The larynx was involved at the time of diagnosis in 96.1% of children, and 87.4% had only 1 anatomic site involved. Children with 1 site involved were significantly older at diagnosis (mean age, 3.9 years) than those with 2 sites (mean age, 2.9 years). Most (74.2%) had stable disease over time, 5.8% showed progression of papillomas to new sites, and 17.9% had no evidence of disease for at least 1 year. Children with disease progression were diagnosed at a significantly younger age than those who remained stable or became disease-free. Children who required tracheotomy were significantly more likely to have progressive disease. The registry has established the clinical course of JORRP in a large sample representative of the United States. Young age was the most important determinant of disease severity (frequency of surgery, extent of disease at diagnosis, and progression of disease). Addressing questions of pathogenesis and disease course will require a revised data collection instrument and molecular analysis of tissues.Keywords
This publication has 11 references indexed in Scilit:
- Reliability of a staging assessment system for recurrent respiratory papillomatosisInternational Journal of Pediatric Otorhinolaryngology, 2003
- Human Papillomavirus in LarynxThe Laryngoscope, 2002
- Molecular Identification of 7 Human Papillomavirus Types in Recurrent Respiratory PapillomatosisJAMA Otolaryngology–Head & Neck Surgery, 2000
- Incidence and Prevalence of Recurrent Respiratory Papillomatosis among Children in Atlanta and SeattleClinical Infectious Diseases, 2000
- Initial Results From the National Registry for Juvenile-Onset Recurrent Respiratory PapillomatosisJAMA Otolaryngology–Head & Neck Surgery, 1999
- A Staging System for Assessing Severity of Disease and Response to Therapy in Recurrent Respiratory PapillomatosisThe Laryngoscope, 1998
- Pediatric Respiratory Papillomatosis: Prognostic Role of Viral Typing and CofactorsThe Laryngoscope, 1997
- Sites of Predilection in Recurrent Respiratory PapillomatosisAnnals of Otology, Rhinology & Laryngology, 1993
- Laryngeal papillomatosis: correlation between severity of disease and presence of HPV 6 and 11 detected by in situ DNA hybridisation.Journal of Clinical Pathology, 1989
- Tracheotomy in children with recurrent respiratory papillomatosisHead & Neck, 1989