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Outcomes of Living-donor Liver Transplantation Using Grafts Heterozygous for α-1 Antitrypsin Gene Mutations

Doshi, Sahil D.1; Wood, Linda2; Abt, Peter L., MD2; Olthoff, Kim M., MD2; Shaked, Abraham, MD, PhD2; Goldberg, David S., MD3,4; Bittermann, Therese, MD3

doi: 10.1097/TP.0000000000002493
Original Clinical Science—Liver
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Background. Patients heterozygous for an abnormal α-1 antitrypsin (A1AT) mutation may have an increased risk of liver disease in the setting of a secondary contributing factor.

Methods. This single-center retrospective cohort study compared donor and recipient outcomes of A1AT heterozygous versus normal phenotype adult living-donor liver transplants (LDLTs).

Results. Between 2010 and 2016, 11 A1AT heterozygous donors and 10 recipients were compared to 57 normal donors and 41 recipients. There were no significant differences in sex, age, or race/ethnicity by A1AT phenotype. Heterozygous donors had significantly lower serum A1AT (median 100 mg/dL versus 131 mg/dL; P < 0.001). Median liver volume at 3 months post-LDLT was not different among donors or their recipients (1164 mm3 in heterozygous versus 1257 mm3 in normal [P = 0.449] for donors; 1563 mm3 versus 1606 mm3 [P = 0.387], respectively, for recipients). Recipient serum alkaline phosphatase at 1 month and 1 year post-LDLT was significantly higher in recipients of A1AT heterozygous grafts (160 U/L versus 99.5 U/L; P = 0.025 at 1 mo) but did not persist at 2 years. In addition, there was no association between A1AT level and liver volume at 3 months posttransplant in donors or recipients.

Conclusions. Patients with a heterozygous A1AT mutation should be considered for living-liver donation.

1 Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.

2 Division of Transplant Surgery, Department of Surgery, University of Pennsylvania, Philadelphia, PA.

3 Division of Gastroenterology, Department of Medicine, University of Pennsylvania, Philadelphia, PA.

4 Center for Clinical Epidemiology and Biostatistics, Department of Biostatistics and Epidemiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.

Received 30 July 2018. Revision received 18 September 2018.

Accepted 8 October 2018.

The authors declare no funding or conflicts of interest.

S.D.D. participated in the conception and design of the work, analysis and interpretation of data, and drafting and critical revision of the work. L.W. participated in the data acquisition and critical revision of the work. P.L.A. participated in the Critical revision of the work. K.L.O. participated in the Critical revision of the work. A.S. participated in the Critical revision of the work. D.S.G. participated in the Conception and design of the work, acquisition, analysis, and interpretation of data, and critical revision of the work. T.B. participated in the conception and design of the work, analysis and interpretation of data, and drafting and critical revision of the work.

Correspondence: Therese Bittermann, MD, 3400 Spruce St, 2 Dulles, Philadelphia, PA 19104. (Therese.Bittermann@uphs.upenn.edu).

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