Pulmonary Arteriovenous Malformations in Dyskeratosis Congenita

Pulmonary arteriovenous malformations (PAVMs) are rare lesions known to cause cyanosis due to abnormal communication between the pulmonary arteries and veins. They are commonly seen in association with hereditary hemorrhagic telangiectasia, congenital heart disease, hepatopulmonary syndrome, and por...

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Bibliographic Details
Published in:Pediatric dermatology 2015-07, Vol.32 (4), p.e165-e166
Main Authors: Samuel, Bennett P., Duffner, Ulrich A., Abdel-Mageed, Aly S., Vettukattil, Joseph J.
Format: Article
Language:English
Subjects:
Arteriovenous Malformations - diagnosis
Arteriovenous Malformations - etiology
Bone Marrow Transplantation - adverse effects
Child
Dyskeratosis Congenita - complications
Dyskeratosis Congenita - diagnosis
Humans
Male
Pulmonary Artery - abnormalities
Pulmonary Veins - abnormalities
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Summary:Pulmonary arteriovenous malformations (PAVMs) are rare lesions known to cause cyanosis due to abnormal communication between the pulmonary arteries and veins. They are commonly seen in association with hereditary hemorrhagic telangiectasia, congenital heart disease, hepatopulmonary syndrome, and portopulmonary shunting, but rarely in patients with dyskeratosis congenita (DC). We describe a patient previously diagnosed with DC confirmed to have microscopic PAVMs after bone marrow transplantation and discuss possible pathogenic mechanisms.
ISSN:0736-8046
1525-1470
DOI:10.1111/pde.12589