Resumo

SOUSA, Célia; ALMEIDA, Leonor Mendonça  e  CARVALHO, André. Acute Pulmonary Manifestation of Type IV Ehlers-Danlos Syndrome in a 19-Year-Old Patient with Hemoptysis. Acta Radiol Port [online]. 2020, vol.32, n.3, pp.31-32.  Epub 14-Jul-2021. ISSN 2183-1351.  https://doi.org/10.25748/arp.20857.

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.

Palavras-chave : Vascular Ehlers-Danlos syndrome; Collagen type III; Hemoptysis; Diffuse alveolar hemorrhage; Lung hemorrhage; Pulmonary thromboembolism..

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