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Ehlers-Danlos syndrome (EDS) is a heterogeneous group of inherited connective-tissue disorders characterized by joint hypermobility, cutaneous fragility, and hyperextensibility. The collagen defect has been identified in at least six of the many types of Ehlers-Danlos syndrome. Changes in the mechanical properties of the bronchial airways and lung parenchyma may trigger the observed increased tendency of the airways to collapse and lung atelectasis. This is a case of a 33 year old Caucasian female who presented with acute hypoxic respiratory failure secondary to persistent right lower lobe atelectasis despite optimal medical management, pulmonary toilet, and multiple bronchoscopies. She also had an incidental finding of lung arteriovenous malformation (AVM) which can occur in EDS patients.

american journal of medical case reports

Acute Respiratory Failure Secondary to Persistent Lobar Atelectasis in Ehlers-Danlos Syndrome Patient and the Role of Connective Tissue Disease in Altering the Mechanical Properties of the Lungs