Details of Disease

General Information of Disease (ID: DISXL96I)

Disease Name	Respiratory papillomatosis
Synonyms	JORRP (type); laryngeal papilloma, recurrent; juvenile-onset recurrent respiratory papillomatosis (type); adult-onset recurrent respiratory papillomatosis (type); AORRP (type); respiratory papillomatosis, recurrent; juvenile laryngeal papilloma; RRP; recurrent respiratory papillomatosis
Disease Class	2F00: Respiratory papillomatosis
Definition	Recurrent respiratory papillomatosis is a rare respiratory disease characterized by the development of exophytic papillomas, affecting the mucosa of the upper aero-digestive tract (with a strong predilection for the larynx), caused by an infection with human papilloma virus. Symptoms at presentation may include hoarseness, chronic cough, dyspnea, recurrent upper respiratory infections, pneumonia, dysphagia, stridor, and/or failure to thrive.
Disease Hierarchy	DISGGAGJ: Respiratory disease DISUFSPX: Otorhinolaryngologic disease DISV4NMP: Papillomatosis DIS1OB2G: Head and neck neoplasm DISXL96I: Respiratory papillomatosis
ICD Code	ICD-11 ICD-11: 2F00.1
Disease Identifiers	MONDO ID MONDO_0018955 MESH ID C535297 UMLS CUI C1168198 MedGen ID 216998 Orphanet ID 60032 SNOMED CT ID 472827002

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)

This Disease is Treated as An Indication in 1 Approved Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
Propranolol	DM79NTF	Approved	Small molecular drug	[1]
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This Disease is Treated as An Indication in 2 Clinical Trial Drug(s)

Drug Name	Drug ID	Highest Status	Drug Type	REF
INO-3107	DMJ0CBX	Phase 1/2	NA	[2]
PRGN-2012	DMR00J2	Phase 1/2	Vaccine	[3]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)

This Disease Is Related to 1 DTT Molecule(s)

Gene Name	DTT ID	Evidence Level	Mode of Inheritance	REF
CCL17	TTMPHAE	Strong	Altered Expression	[4]
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This Disease Is Related to 5 DOT Molecule(s)

Gene Name	DOT ID	Evidence Level	Mode of Inheritance	REF
KIR3DS1	OTJWIO4T	Strong	Biomarker	[5]
PEX1	OTQJF0V7	Strong	Altered Expression	[6]
PEX3	OTGZ7ME2	Strong	Biomarker	[6]
RHBDL1	OTSCUCQV	Strong	Biomarker	[7]
RRBP1	OT4ZTPTM	Strong	Biomarker	[7]
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References

1	Propranolol FDA Label
2	ClinicalTrials.gov (NCT04398433) An Open-label Multi-center Study of INO-3107 With Electroporation (EP) in Subjects With HPV-6- and/or HPV-11-associated Recurrent Respiratory Papillomatosis (RRP). U.S.National Institutes of Health.
3	ClinicalTrials.gov (NCT04724980) A Phase 1/2 Study of Adjuvant PRGN-2012 in Adult Patients With Recurrent Respiratory Papillomatosis. U.S.National Institutes of Health.
4	Immune suppression in premalignant respiratory papillomas: enriched functional CD4+Foxp3+ regulatory T cells and PD-1/PD-L1/L2 expression.Clin Cancer Res. 2012 Apr 1;18(7):1925-35. doi: 10.1158/1078-0432.CCR-11-2941. Epub 2012 Feb 9.
5	Activating killer cell immunoglobulin-like receptors 3DS1 and 2DS1 protect against developing the severe form of recurrent respiratory papillomatosis.Hum Immunol. 2010 Feb;71(2):212-9. doi: 10.1016/j.humimm.2009.10.009. Epub 2009 Oct 25.
6	Demonstration of antibodies against human papillomavirus type-11 E6 and L2 proteins in patients with recurrent respiratory papillomatosis.Auris Nasus Larynx. 1997 Apr;24(2):185-91. doi: 10.1016/s0385-8146(96)00000-4.
7	Model of human recurrent respiratory papilloma on chicken embryo chorioallantoic membrane for tumor angiogenesis research.Histol Histopathol. 2017 Jul;32(7):699-710. doi: 10.14670/HH-11-831. Epub 2016 Oct 10.