Deceased Donor Renal Transplantation Combined with Bilateral Nephrectomy in a Patient with Tuberous Sclerosis and Renal Failure

Introduction. A 27-year-old female patient with known tuberous sclerosis complex (TSC), polycystic kidneys with multiple large bilateral angiomyolipomas, and failing renal functions with prehemodialysis values (urea: 19 mmol/L; creatinine: 317 μmol/L; CKD-EPI 0,27) was admitted to our department for...

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Main Authors: R. Novotny, J. Chlupac, T. Marada, S. Bloudickova-Rajnochova, H. Vavrinova, L. Janousek, J. Fronek
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Transplantation
Online Access:http://dx.doi.org/10.1155/2019/2172163
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author R. Novotny
J. Chlupac
T. Marada
S. Bloudickova-Rajnochova
H. Vavrinova
L. Janousek
J. Fronek
author_facet R. Novotny
J. Chlupac
T. Marada
S. Bloudickova-Rajnochova
H. Vavrinova
L. Janousek
J. Fronek
author_sort R. Novotny
collection DOAJ
description Introduction. A 27-year-old female patient with known tuberous sclerosis complex (TSC), polycystic kidneys with multiple large bilateral angiomyolipomas, and failing renal functions with prehemodialysis values (urea: 19 mmol/L; creatinine: 317 μmol/L; CKD-EPI 0,27) was admitted to our department for pre-renal transplant evaluation. The patient was placed on the transplant waiting list as the living donor did not pass pretransplant workup and was subsequently contraindicated. Patient was placed on the “cadaverous kidney transplant waiting list”. Method. Computed tomography angiography revealed symptomatic PSA in the right kidney angiomyolipoma (AML). The patient underwent urgent transarterial embolisation of the PSA’s feeding vessel in the right kidney AML. Based on the “kidney transplant waiting list” order patient underwent a bilateral nephrectomy combined with transperitoneal renal allotransplantation of a cadaverous kidney graft through midline laparotomy, appendectomy, and cholecystectomy. Results. Postoperative period was complicated by delayed graft function caused by acute tubular necrosis requiring postoperative hemodialysis. The patient was discharged on the 17th postoperative day with a good renal graft function. Patient’s follow-up is currently 23 months with good graft function (urea: 9 mmol/L; creatinine: 100 μmol/L). Conclusion. Renal transplantation combined with radical nephrectomy provides a definitive treatment for TSC renal manifestations.
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institution Kabale University
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spelling doaj-art-1dc7eca144554f43ac04738d04a693db2025-02-03T05:58:36ZengWileyCase Reports in Transplantation2090-69432090-69512019-01-01201910.1155/2019/21721632172163Deceased Donor Renal Transplantation Combined with Bilateral Nephrectomy in a Patient with Tuberous Sclerosis and Renal FailureR. Novotny0J. Chlupac1T. Marada2S. Bloudickova-Rajnochova3H. Vavrinova4L. Janousek5J. Fronek6Transplant Surgery Department, Institute for Clinical and Experimental Medicine, Prague, Czech RepublicTransplant Surgery Department, Institute for Clinical and Experimental Medicine, Prague, Czech RepublicTransplant Surgery Department, Institute for Clinical and Experimental Medicine, Prague, Czech RepublicDepartment of Nephrology, Institute for Clinical and Experimental Medicine, Prague, Czech RepublicDepartment of Nephrology, Institute for Clinical and Experimental Medicine, Prague, Czech RepublicTransplant Surgery Department, Institute for Clinical and Experimental Medicine, Prague, Czech RepublicTransplant Surgery Department, Institute for Clinical and Experimental Medicine, Prague, Czech RepublicIntroduction. A 27-year-old female patient with known tuberous sclerosis complex (TSC), polycystic kidneys with multiple large bilateral angiomyolipomas, and failing renal functions with prehemodialysis values (urea: 19 mmol/L; creatinine: 317 μmol/L; CKD-EPI 0,27) was admitted to our department for pre-renal transplant evaluation. The patient was placed on the transplant waiting list as the living donor did not pass pretransplant workup and was subsequently contraindicated. Patient was placed on the “cadaverous kidney transplant waiting list”. Method. Computed tomography angiography revealed symptomatic PSA in the right kidney angiomyolipoma (AML). The patient underwent urgent transarterial embolisation of the PSA’s feeding vessel in the right kidney AML. Based on the “kidney transplant waiting list” order patient underwent a bilateral nephrectomy combined with transperitoneal renal allotransplantation of a cadaverous kidney graft through midline laparotomy, appendectomy, and cholecystectomy. Results. Postoperative period was complicated by delayed graft function caused by acute tubular necrosis requiring postoperative hemodialysis. The patient was discharged on the 17th postoperative day with a good renal graft function. Patient’s follow-up is currently 23 months with good graft function (urea: 9 mmol/L; creatinine: 100 μmol/L). Conclusion. Renal transplantation combined with radical nephrectomy provides a definitive treatment for TSC renal manifestations.http://dx.doi.org/10.1155/2019/2172163
spellingShingle R. Novotny
J. Chlupac
T. Marada
S. Bloudickova-Rajnochova
H. Vavrinova
L. Janousek
J. Fronek
Deceased Donor Renal Transplantation Combined with Bilateral Nephrectomy in a Patient with Tuberous Sclerosis and Renal Failure
Case Reports in Transplantation
title Deceased Donor Renal Transplantation Combined with Bilateral Nephrectomy in a Patient with Tuberous Sclerosis and Renal Failure
title_full Deceased Donor Renal Transplantation Combined with Bilateral Nephrectomy in a Patient with Tuberous Sclerosis and Renal Failure
title_fullStr Deceased Donor Renal Transplantation Combined with Bilateral Nephrectomy in a Patient with Tuberous Sclerosis and Renal Failure
title_full_unstemmed Deceased Donor Renal Transplantation Combined with Bilateral Nephrectomy in a Patient with Tuberous Sclerosis and Renal Failure
title_short Deceased Donor Renal Transplantation Combined with Bilateral Nephrectomy in a Patient with Tuberous Sclerosis and Renal Failure
title_sort deceased donor renal transplantation combined with bilateral nephrectomy in a patient with tuberous sclerosis and renal failure
url http://dx.doi.org/10.1155/2019/2172163
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