title: Alpha-1 antitrypsin deficiency and splenic artery aneurysm rupture: an association?
abstract: OBJECTIVE
  Theoretically, patients with alpha 1-antitrypsin deficiency may be vulnerable to the development of splenic artery aneurysms. alpha-1 antitrypsin deficiency can induce cirrhosis with portal hypertension, and resulting protease-antiprotease imbalances may exaggerate arterial wall weakness due to proteolysis of arterial structural proteins. A splenic artery aneurysm rupture 7 days after liver transplantation provoked a reassessment of the incidence of this phenomenon in a liver transplant population.
  
  
  METHODS
  Case records from three institutions and the results of a survey sent to 126 liver transplantation programs in the United Network for Organ Sharing database were reviewed. The incidence of splenic artery aneurysm rupture in the peritransplantation period, etiology of liver disease associated with this phenomenon, and recommendations regarding management of splenic artery aneurysms was assessed.
  
  
  RESULTS
  Twenty-one cases of splenic artery aneurysm rupture were identified. alpha-1 antitrypsin deficiency was the most common cause of cirrhosis in the majority of identified patients who presented with splenic artery aneurysm rupture, which was associated with a mortality rate of 57%. Respondents to the survey indicated that a preoperative evaluation was warranted if a splenic artery aneurysm was suspected; however, no consensus regarding management exists.
  
  
  CONCLUSIONS
  The presence and risk of rupture of splenic artery aneurysms may be greater in patients with alpha-1 antitrypsin deficiency. If identified before rupture, an aggressive approach to diagnosing and treating these aneurysms should be initiated. At present, no consensus exists regarding the management of splenic artery aneurysms.
authors.affiliation: Division of Transplantation, Tulane University School of Medicine, New Orleans, Louisiana, USA.
authors.forename: P J
authors.initials: PJ
authors.lastname: Gaglio
authors.forename: F
authors.initials: F
authors.lastname: Regenstein
authors.forename: D
authors.initials: D
authors.lastname: Slakey
authors.forename: S
authors.initials: S
authors.lastname: Cheng
authors.forename: H
authors.initials: H
authors.lastname: Takiff
authors.forename: R
authors.initials: R
authors.lastname: Rinker
authors.forename: D
authors.initials: D
authors.lastname: Dick
authors.forename: S N
authors.initials: SN
authors.lastname: Thung
mesh_terms: D000293:Adolescent; D000328:Adult; D017542:Aneurysm, Ruptured / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D005260:Female; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology / Q000601:surgery; D008107:Liver Diseases / Q000150:complications / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D012189:Retrospective Studies; D013157:Splenic Artery; D011795:Surveys and Questionnaires; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications*