The study took twenty patients with Ehlers-Danlos syndrome with the goal of assessing “the frequency of respiratory abnormalities in this condition.” Five patients had “one episode of haemoptysis, but none had any defect of coagulation.” Recurrent sinusitis was noted “notably in those with the type I syndrome.” In looking deeper into the lung area “two patients had bullous lung disease, one of whom (type IV) had had three pneumothoraces and subsequent pleurodesis; he also had tracheomegaly (the Mounier-Kuhn abnormality).” The study then went on to focus on skeletal abnormalities and found minor ones “such as pectus excavatum…..particularly in patients with type IV disease” while “three patients had the straight back syndrome.” In focusing on lung volumes, the study noted that there “were no consistent spirometric or lung volume abnormalities, but eight patients had a raised gas transfer coefficient (Kco), possibly due to an increased intrapulmonary vascular volume. The study did qualify this finding by pointing out that there were two other patients with “very low values of Kco that were unexplained.”
A full copy of this abstract and the article can be found here
my body has been somewhat falling apart
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