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Liver Transplantation in Patients with Sickle Cell Disease in the United States

Hogen, Rachel and Kim, Michelle and Lee, Yelim and Lo, Mary and Kaur, Navpreet and Kahn, Jeff and Chopra, Shefali and Qazi, Yasir and Sedra, Ashraf and Kim, Jim and O'Brien, Lauren and Genyk, Yuri and Sher, Linda and Emamaullee, Juliet (2020) Liver Transplantation in Patients with Sickle Cell Disease in the United States. Journal of Surgical Research, 255 . pp. 23-32. ISSN 0022-4804.

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Background: Up to 30% of patients with sickle cell disease (SCD) develop chronic liver disease via etiologies including sickle cell hepatopathy, acquired viral hepatitis, or secondary hemochromatosis. It is unclear how many patients with SCD ultimately undergo liver transplantation (LT) and what factors are associated with survival after LT. In this study, we examined LT outcomes in these patients by reviewing the Scientific Registry of Transplant Recipients (SRTR) and our institutional experience. Methods: Analysis of the SRTR identified 23 LT recipients and five simultaneous liver and kidney transplantation (SLKT) recipients with SCD. Patient demographics and graft and patient survival were analyzed. Two patients with SCD at our institution underwent SLKT. Results: Review of the SRTR revealed that recipients with SCD had significantly higher model for end-stage liver disease scores (33 versus 21, P = 0.004), preoperative intensive care unit admission (43.5% versus 19.1%, P = 0.007), preoperative dialysis (17.4% versus 4.9%, P = 0.009), and were more likely to be status 1 (26.1% versus 12.1%, P = 0.041) when compared with the reference population of African American LT recipients. Despite being higher risk at the time of LT, patients with SCD had equivalent posttransplant graft and patient survival when compared with the reference population (P = 0.5 and P = 0.2, respectively) and a 2:1 propensity score–matched group (P = 0.5 and P = 0.2, respectively). Two recent SLKT recipients with SCD from our institution have performed well with stable allograft function. Conclusions: Data from the SRTR demonstrate that patients with SCD can expect equivalent graft and patient survival after LT despite exhibiting more comorbidities at the time of LT. The low number of patients with SCD who underwent LT in the SRTR in comparison with the rate of chronic liver disease in this population raises the question as to whether a disparity in access to LT exists for this complex population.

Item Type:Article
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URLURL TypeDescription
Genyk, Yuri0000-0001-9552-3995
Emamaullee, Juliet0000-0003-4238-3057
Additional Information:© 2020 Elsevier Inc. Received 7 January 2020, Revised 26 March 2020, Accepted 6 May 2020, Available online 12 June 2020.
Funding AgencyGrant Number
NIHR01 HL141857-01
Subject Keywords:Sickle cell disease; Liver transplantation; Scientific registry of transplant recipients; Health disparities
Record Number:CaltechAUTHORS:20200615-145722186
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Official Citation:Rachel Hogen, Michelle Kim, Yelim Lee, Mary Lo, Navpreet Kaur, Jeff Kahn, Shefali Chopra, Yasir Qazi, Ashraf Sedra, Jim Kim, Lauren O'Brien, Yuri Genyk, Linda Sher, Juliet Emamaullee, Liver Transplantation in Patients with Sickle Cell Disease in the United States, Journal of Surgical Research, Volume 255, 2020, Pages 23-32, ISSN 0022-4804, (
Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:103928
Deposited By: Tony Diaz
Deposited On:15 Jun 2020 22:04
Last Modified:15 Jun 2020 22:04

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