α-1 antitrypsin deficiency and splenic artery aneurysm rupture: An association?

P. J. Gaglio, F. Regenstein, D. Slakey, S. Cheng, H. Takiff, R. Rinker, D. Dick, S. N. Thung

Research output: Contribution to journalArticle

23 Citations (Scopus)

Abstract

OBJECTIVE: Theoretically, patients with alpha 1-antitrypsin deficiency may be vulnerable to the development of splenic artery aneurysms, α-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. α-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 α-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. (C) 2000 Am. Coll. of Gastroenterology.

Original languageEnglish (US)
Pages (from-to)1531-1534
Number of pages4
JournalAmerican Journal of Gastroenterology
Volume95
Issue number6
DOIs
StatePublished - Jun 2000
Externally publishedYes

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Splenic Artery
Aneurysm
Rupture
Liver Transplantation
Fibrosis
alpha 1-Antitrypsin Deficiency
Incidence
Portal Hypertension
Gastroenterology
Protease Inhibitors
Proteolysis
Liver Diseases
Peptide Hydrolases
Databases
Transplants

ASJC Scopus subject areas

  • Gastroenterology

Cite this

Gaglio, P. J., Regenstein, F., Slakey, D., Cheng, S., Takiff, H., Rinker, R., ... Thung, S. N. (2000). α-1 antitrypsin deficiency and splenic artery aneurysm rupture: An association? American Journal of Gastroenterology, 95(6), 1531-1534. https://doi.org/10.1016/S0002-9270(00)00882-0

α-1 antitrypsin deficiency and splenic artery aneurysm rupture : An association? / Gaglio, P. J.; Regenstein, F.; Slakey, D.; Cheng, S.; Takiff, H.; Rinker, R.; Dick, D.; Thung, S. N.

In: American Journal of Gastroenterology, Vol. 95, No. 6, 06.2000, p. 1531-1534.

Research output: Contribution to journalArticle

Gaglio, PJ, Regenstein, F, Slakey, D, Cheng, S, Takiff, H, Rinker, R, Dick, D & Thung, SN 2000, 'α-1 antitrypsin deficiency and splenic artery aneurysm rupture: An association?', American Journal of Gastroenterology, vol. 95, no. 6, pp. 1531-1534. https://doi.org/10.1016/S0002-9270(00)00882-0
Gaglio, P. J. ; Regenstein, F. ; Slakey, D. ; Cheng, S. ; Takiff, H. ; Rinker, R. ; Dick, D. ; Thung, S. N. / α-1 antitrypsin deficiency and splenic artery aneurysm rupture : An association?. In: American Journal of Gastroenterology. 2000 ; Vol. 95, No. 6. pp. 1531-1534.
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AU - Slakey, D.

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AU - Rinker, R.

AU - Dick, D.

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N2 - OBJECTIVE: Theoretically, patients with alpha 1-antitrypsin deficiency may be vulnerable to the development of splenic artery aneurysms, α-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. α-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 α-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. (C) 2000 Am. Coll. of Gastroenterology.

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