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Transplant Immunology 33 (2015) 27–29

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Transplant Immunology

journal homepage: www.elsevier.com/locate/trim

A lesson from kidney transplantation among identical twins: Case report

and literature review☆
Zhengsheng Rao, Zhongli Huang, Turun Song, Tao Lin ⁎
Department of Urology, West China Hospital, Sichuan University, Guoxue Xiang #37, Chengdu 610041 Sichuan, People's Republic of China.

a r t i c l e i n f o a b s t r a c t

Article history: There continues to be disagreement related to the appropriate therapeutic regimen to be used when the donor
Received 22 June 2015 and the recipient in kidney transplant operations are identical twins. Here we present two cases of kidney
Accepted 15 July 2015 transplantation between identical twins. Both recipients had end-stage renal disease (ESRD) caused by primary
Available online 17 July 2015
nephropathy. We also present information gleaned from a literature review of similar cases. The first recipient
was a 26-year-old man who experienced biopsy-proven IgA nephropathy 10 months post-transplantation.
Kidney transplantation
Mycophenolate mofetil (MMF), angiotensin receptor blockers (ARBs), and steroids were used to reverse this
Identical twins pathologic condition. Till now, 76 months post-transplantation, the patient is stable, and the new kidney is
Immunosuppression functioning well. The second recipient was a 20-year-old woman who had hematuria and proteinuria 3 months
post-transplantation, and crescent glomerulonephritis with mild to moderate interstitial injury was proven by
biopsy 11 months postoperatively. This patient did not respond to various treatments and resumed hemodialysis
15 months post-transplantation. These case studies show that immunosuppressive therapy should be main-
tained in kidney transplant recipients who are identical twins with ESRD caused by initial nephropathy.
© 2015 Elsevier B.V. All rights reserved.

1. Introduction regimens when the donor and the recipient of the kidney transplant
are identical twins must be considered [13–16].
Kidney transplantation is the optimal treatment choice in patients Here we present two cases of kidney transplantation between
with end-stage renal disease (ESRD). The first successful kidney trans- identical twins, including dilemmas faced by the healthcare providers
plantation was performed in Boston by Joseph Murray on December related to determining the best therapeutic regimens. We also present
23, 1954 [1]. The recipient received a kidney from his monozygotic information gleaned from a literature review of similar cases.
twin brother, did not take immunosuppressive medications, and died
of a myocardial infarction 9 years postoperatively [2,3]. Following this
first kidney transplantation, a large number of successful renal trans- 1.1. Case 1
plantations between identical twins were reported in the 1950s and
1960s [4–9]. A 26-year-old male with ESRD was admitted to an organ trans-
The outcome of kidney transplantation in human leukocyte antigen plant center for a kidney transplant. Before his admission, he
(HLA)-mismatched living and deceased-donor transplants has greatly underwent 1 month of hemodialysis, during which dyspnea and
improved with the introduction of potent immunosuppressive thera- orthopnea were observed. The surgery was performed on January 13,
pies [10]. However, the outcomes are very different for homozygous 2009, with an organ harvested from his identical twin brother, the ap-
twins. Due to the extreme genetic similarity between homozygous propriateness of which was confirmed by genetic testing via short tan-
twins, immunosuppressive regimens can theoretically be avoided. dem repeat (STR) DNA analysis. He had a normal serum creatinine level
However, any individual who receives a new kidney has a high risk of of 116.7 μmol/L on postoperative day 2. The patient's postoperative
the initial kidney disease returning just as much as new-onset nephrop- medications included intravenous methylprednisolone for 1 week and
athy. Furthermore, immunosuppressive regimens can treat both patho- subsequent oral prednisone for 1 month. A graft biopsy was performed
logical conditions [11,12]. Thus, the use of immunosuppressive 10 months postoperatively because of the emergence of proteinuria and
hematuria. The biopsy findings led to a diagnosis of IgA nephropathy
☆ No conflicts of interest among authors.
(Lee II–III). Subsequently, a regimen of MMF (250 mg/day), steroids
⁎ Corresponding author. (10 mg/day), and ARBs was initiated, and the hematuria and protein-
E-mail address: kidney5@163.com (T. Lin). uria gradually subsided. The patient has continued to administer

0966-3274/© 2015 Elsevier B.V. All rights reserved.
28 Z. Rao et al. / Transplant Immunology 33 (2015) 27–29

MMF. No rejection episodes have been observed, and the graft has been with proteinuria (total 24 h urine protein: 0.32 g) 41 months after
functioning stably for 6 years. discontinuing immunosuppression and was cured after the adminis-
tration of ARBs. Another patient was diagnosed by biopsy with rapid-
1.2. Case 2 ly progressive glomerulonephritis 28 months after discontinuing
immunosuppressive treatment and resumed hemodialysis 42 months
A 20-year-old woman was diagnosed with ESRD. Two years later, after immunosuppressive therapy was withdrawn. The third patient
she underwent hemodialysis for 2 months. Subsequently, on October had biopsy-proven focal segmental glomerulosclerosis (FSGS) 8 years
28, 2011, she received a living kidney from her monozygotic twin sis- after discontinuing immunosuppressive therapy and then underwent
ter, the appropriateness of which was confirmed by STR DNA analy- peritoneal dialysis.
sis. After transplantation, both intravenous methylprednisolone and In our cases, the transplant appropriateness of both monozygotic
oral prednisone were administered for 1 week. At 3 months, hematu- twins was confirmed by genetic testing via STR DNA analysis. Both of
ria and proteinuria were observed, and her creatinine level rose to the recipients were diagnosed with chronic glomerulonephritis pre-
150.7 μmol/L. A relapse of the nephropathy was suspected, but the transplantation. However, it was difficult to differentiate the recurrence
patient refused biopsy because of personal reasons. Then tacrolimus of the original kidney disease from new-onset nephropathy in the grafts
(Tac), MMF, and steroids were administered. However, her condition because the primary renal diseases were unknown because the native
worsened; her creatinine increased to 192 μmol/L, and obvious gross kidneys had not been biopsied. In the first case, we succeeded in
hematuria was observed 11 months postoperatively. A graft biopsy treating the patient with ARBs and immunosuppressive therapy. His
at 12 months showed crescent glomerulonephritis with mild to proteinuria and hematuria markedly decreased, and he has maintained
moderate interstitial injury. Thereafter, due to a repeating increased stable renal function for 6 years. In case two, immunosuppressive ther-
level of creatinine, a sequential treatment including methylprednis- apy was withdrawn early after transplantation and was not resumed
olone (200 mg IV/day for 5 days), cyclophosphamide (0.2 g every until hematuria and proteinuria were detected. However, treatment
other day), and pure ultrafiltration was administered over the course with strong immunosuppressive agents that are usually used to slow
of three hospitalizations in the next three months. However, these the rapid progression of acute glomerulonephritis failed. Thus, it is
interventions failed to stop the progression of the disease. Due to the clearly important to know the primary disorder in kidney transplant re-
deteriorating graft function, she resumed hemodialysis 15 months cipients' native kidneys, particularly if the recipient is an identical twin.
post-transplantation. In conclusion, immunosuppressive therapy for identical twins re-
ceiving a kidney transplant presents unique challenges. Multiple factors
2. Discussion must be considered before immunosuppressive therapy is discontinued,
including the recipient's primary diseases, transplant history, postoper-
Whether immunosuppressive therapy is needed and how long it ative course, and immunization status.
should be maintained post-kidney transplantation among monozygotic
twins remain controversial. Theoretically, identical twins are 100% ge- Contribution of the authors
netically similar [17], but we cannot overlook the effect of intrauterine
gene mutations that may cause phenotypic and genotypic divergence Zhengsheng Rao: performed the research/study, collected the data,
between monozygotic twins. In addition, the main arguments against analyzed the data, and wrote the paper.
immunosuppressive therapy focus on the side effects, including hyper- Zhongli Huang: collected the data, analyzed the data, and wrote the
tension, diabetes, infection, bone disease, nephrotoxicity, and lack of paper.
effectiveness in stopping the recurrence of the original nephropathy Turun Song: performed the research/study, collected the data, and
or new-onset kidney diseases [18–21]. analyzed the data.
The literature supports the view that not all such patients need im- Tao Lin: designed the research/study and edited the manuscript.
munosuppressive therapy [20,22,23]. Kessaris et al. [20] retrospectively
analyzed the outcomes in 120 cases of renal transplantation in twins in
the United Sates and did not find a significant difference in the 5-year Acknowledgments
graft survival rates between recipients who received immunosuppres-
sive therapy (n = 82) and those who did not (n = 38) (93.9 and 84%, No funding supported.
respectively, log rank; P = 0.12). Of the 82 patients who received
immunosuppressive therapy, 29 received maintenance therapy and References
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