A Case of Hereditary Hemorrhagic Telangiectasia Diagnosed through Spontaneous Hemothorax / 결핵및호흡기질환
Tuberculosis and Respiratory Diseases
; : 50-54, 2012.
Article
em Ko
| WPRIM
| ID: wpr-101777
Biblioteca responsável:
WPRO
ABSTRACT
Hereditary hemorrhagic telangiectasia (HHT, Osler-Weber-Rendu disease) is a rare autosomal dominant disease characterized by heterogenous multisystemic dysplasia of the vascular tissue. Prevalence of HHT is 1 in 5,000~8,000. HHT commonly presents with recurrent epistaxis, but may have more serious consequences if visceral vascular beds are involved. Approximately 30~50% of HHT cases also present with pulmonary arteriovenous malformation (PAVM). Spontaneous hemothorax is less common, and PAVM is one of the causes leading to hemothorax. Our case involved an 18-year-old female who had suddenly developed right chest pain. The reason for chest pain was due to right spontaneous hemothorax accompanied by PAVM in the right middle lobe. The patient was additionally diagnosed with HHT upon examination of her family history, specifically through her mother's symptom that included recurrent epistaxis and mucosal telangiectasia.
Palavras-chave
Texto completo:
1
Base de dados:
WPRIM
Assunto principal:
Malformações Arteriovenosas
/
Telangiectasia Hemorrágica Hereditária
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Telangiectasia
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Dor no Peito
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Epistaxe
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Prevalência
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Hemotórax
Tipo de estudo:
Diagnostic_studies
/
Prevalence_studies
Limite:
Adolescent
/
Female
/
Humans
Idioma:
Ko
Revista:
Tuberculosis and Respiratory Diseases
Ano de publicação:
2012
Tipo de documento:
Article