Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysis
Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant condition causing systemic fibrovascular dysplasia. It has an incidence of 1-2/100,000. Phenotypic variation is extreme ranging from asymptomatic to severely symptomatic, from cases with no o...
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Format: | Article |
Language: | English |
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Wolters Kluwer Medknow Publications
2016-01-01
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Series: | Lung India |
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Online Access: | http://www.lungindia.com/article.asp?issn=0970-2113;year=2016;volume=33;issue=3;spage=313;epage=316;aulast=Khoja |
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author | Amir M Khoja Rahul K Jalan Dheeraj L Jain Omkar V Kajale |
author_facet | Amir M Khoja Rahul K Jalan Dheeraj L Jain Omkar V Kajale |
author_sort | Amir M Khoja |
collection | DOAJ |
description | Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant condition causing systemic fibrovascular dysplasia. It has an incidence of 1-2/100,000. Phenotypic variation is extreme ranging from asymptomatic to severely symptomatic, from cases with no or few mucocutaneous lesions to those with diffuse cutaneous telangiectasia. We discuss a case of Osler-Weber-Rendu disease causing diffuse cutaneous telangiectasia and hemoptysis. The patient presented with complaints of hemoptysis and was extensively examined and investigated before being diagnosed with Osler-Weber-Rendu disease. We successfully managed the patient′s hemoptysis by bronchial artery embolization. This case emphasizes the need for careful examination and investigation and to consider such rare diseases when all the common causes of hemoptysis are ruled out. An early and proper diagnosis will lead to more effective management of such a rare disease with few treatment options available. |
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issn | 0970-2113 0974-598X |
language | English |
last_indexed | 2024-12-20T04:08:05Z |
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spelling | doaj.art-900e3d29a9674f729e00c337783778ab2022-12-21T19:53:58ZengWolters Kluwer Medknow PublicationsLung India0970-21130974-598X2016-01-0133331331610.4103/0970-2113.180878Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysisAmir M KhojaRahul K JalanDheeraj L JainOmkar V KajaleOsler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant condition causing systemic fibrovascular dysplasia. It has an incidence of 1-2/100,000. Phenotypic variation is extreme ranging from asymptomatic to severely symptomatic, from cases with no or few mucocutaneous lesions to those with diffuse cutaneous telangiectasia. We discuss a case of Osler-Weber-Rendu disease causing diffuse cutaneous telangiectasia and hemoptysis. The patient presented with complaints of hemoptysis and was extensively examined and investigated before being diagnosed with Osler-Weber-Rendu disease. We successfully managed the patient′s hemoptysis by bronchial artery embolization. This case emphasizes the need for careful examination and investigation and to consider such rare diseases when all the common causes of hemoptysis are ruled out. An early and proper diagnosis will lead to more effective management of such a rare disease with few treatment options available.http://www.lungindia.com/article.asp?issn=0970-2113;year=2016;volume=33;issue=3;spage=313;epage=316;aulast=KhojaBronchial artery embolizationhemoptysisOsler-Weber-Rendu diseasetelangiectasia |
spellingShingle | Amir M Khoja Rahul K Jalan Dheeraj L Jain Omkar V Kajale Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysis Lung India Bronchial artery embolization hemoptysis Osler-Weber-Rendu disease telangiectasia |
title | Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysis |
title_full | Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysis |
title_fullStr | Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysis |
title_full_unstemmed | Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysis |
title_short | Osler-Weber-Rendu disease: A rare cause of recurrent hemoptysis |
title_sort | osler weber rendu disease a rare cause of recurrent hemoptysis |
topic | Bronchial artery embolization hemoptysis Osler-Weber-Rendu disease telangiectasia |
url | http://www.lungindia.com/article.asp?issn=0970-2113;year=2016;volume=33;issue=3;spage=313;epage=316;aulast=Khoja |
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