Acute Pulmonary Manifestation of Type IV Ehlers-Danlos Syndrome in a 19-Year-Old Patient with Hemoptysis

Abstract Ehlers-Danlos syndrome type IV is a rare inherited autosomal dominant disease, caused by a defect or deficiency of type III collagen encoded by the COL3A1 gene. This disorder confers an anomalous fragility of blood vessels, uterus, hollow viscera, skin and lung. We describe a case and the pulmonary changes of a 19-year-old male with Ehlers-Danlos syndrome type IV presenting abundant hemoptysis.

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Bibliographic Details
Main Authors: Sousa,Célia, Almeida,Leonor Mendonça, Carvalho,André
Format: Digital revista
Language:English
Published: Sociedade Portuguesa de Radiologia e Medicina Nuclear 2020
Online Access:http://scielo.pt/scielo.php?script=sci_arttext&pid=S2183-13512020000300031
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