UNASSIGNED: History and clinical findings   A 57-year-old @entity1 with a history of @entity1030 GOLD D and homozygotic alpha-1-antitrypsin deficiency presented with aggravated dyspnoea, @entity1163 and left thoracic @entity662 . 12 years previously Stanford type B dissection had been conservatively treated. Examinations   Chest radiograph revealed extended infiltrates of the left lower lobe. Seen together with elevated inflammatory values and the @entity1  s hemodynamic instability it pointed at a pneumonia-driven septic shock. In view of a progressive @entity1576 and an acute drop in haemoglobin computed tomography was performed. It showed rupture of an aneurysmatic @entity358 with consecutive @entity548 . Treatment and course   Because of persistent @entity548 and @entity1576 requiring invasive ventilation, thoracotomy was performed. @entity358 was sutured and left lower lobe resected. After initial postoperative stabilization @entity292 demanded re-thoracotomy and parenchymal lesions of left upper lobe were sutured. In consequence of progressive @entity295 and @entity177 @entity1 died 15 days after initial surgical @entity548 control. Conclusion   Though homozygotic alpha-1-antitrypsin deficiency is primarily associated with lung and @entity409 , extrapulmonary and extrahepatic manifestation may occur. In view of consistent case reports and plausible pathogenetic mechanisms, @entity455 comprising @entity438 and dissection due to compromised vascular integrity in alpha-1-antitrypsin deficient individuals is conceivable, but lacks scientific proof.

[Spontaneous rupture of XXXX in homozygotic alpha-1-antitrypsin deficiency.]

@entity1 :: ('9606', 'Species') :: ['woman', 'patient'] @entity662 :: ('MESH:D002637', 'Disease') :: ['chest pain'] @entity295 :: ('MESH:D018805', 'Disease') :: ['sepsis'] @entity455 :: ('MESH:D014652', 'Disease') :: ['vascular complications'] @entity548 :: ('MESH:D006470', 'Disease') :: ['intraparenchymal bleeding', 'bleeding'] @entity409 :: ('MESH:D008107', 'Disease') :: ['liver disease'] @entity1163 :: ('MESH:D013575', 'Disease') :: ['presyncope'] @entity177 :: ('MESH:D051437', 'Disease') :: ['manifest multiorgan failure'] @entity358 :: ('MESH:D008171', 'Disease') :: ['Left lower pulmonary artery', 'left lower pulmonary artery'] @entity292 :: ('MESH:D005402', 'Disease') :: ['bronchopleural fistula'] @entity1576 :: ('MESH:D012131', 'Disease') :: ['respiratory failure', 'respiratory insufficiency'] @entity1030 :: ('MESH:D029424', 'Disease') :: ['emphysematous COPD'] @entity438 :: ('MESH:D002532', 'Disease') :: ['arterial aneurysms']
@entity358 :: (MESH:D008171,Disease) :: ['aneurysmatic pulmonary artery']
