Respiratory complications of Ehlers-Danlos syndrome type IV | Macquarie University ResearchOnline

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Title: Respiratory complications of Ehlers-Danlos syndrome type IV
Related: Clinical genetics, Vol. 50, Issue 6, (1996), p.510-514
DOI: 10.1111/j.1399-0004.1996.tb02724.x
Publisher: Wiley-Blackwell Publishing
Date: 1996
Author/Creator: Dowton, S. Bruce
Author/Creator: Pincott, Stuart
Author/Creator: Demmer, Laurie
Description: Pulmonary complications are described in a case of Ehlers-Danlos syndrome type IV, established by studies of collagen biosynthesis. At age 20.5 years the patient, who had previously suffered a spontaneous colonic perforation, developed intermittent recurrent hemoptysis and had a spontaneous hemopneumothorax. At presentation, imaging studies revealed multiple scattered cavitary lesions in both lungs. On separate occasions large parenchymal cysts ensued and subsequently regressed. Reviews of other reported patients indicate that pulmonary complications do occur in patients with Ehlers-Danlos syndrome type IV but have not resulted directly in patient mortality.
Description: 5 page(s)
Subject Keyword: Ehlers-Danlos syndrome
Subject Keyword: Pneumothorax
Subject Keyword: Pulmonary cyst
Resource Type: journal article
Organisation: Macquarie University. Office of the Vice-Chancellor

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