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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Main Authors: | , , |
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Format: | Digital revista |
Language: | English |
Published: |
Sociedade Portuguesa de Radiologia e Medicina Nuclear
2020
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Online Access: | http://scielo.pt/scielo.php?script=sci_arttext&pid=S2183-13512020000300031 |
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