Detailed Abstract
[E-poster - Liver (Transplantation)]
[EP 014] Combined Liver-Kidney Transplantation for Primary Hyperoxaluria Type 1: First Case Report From Nepal
Akanand SINGH1, Narayan Prasad BHUSHAL2, Barun SHRESTHA3, Santosh CHHETRI4, Kashyap DAHAL4, Tshering Wangdi SHERPA3, Adhyashree KARKI3, Mukund Raj JOSHI1, Tanka Prasad BOHARA1
1Department of Liver Transplantation and Hepatobiliary Surgery, KIST Medical College and Teaching Hospital, Nepal 2Department of Surgery, Division of Transplant Surgery, KIST Medical College and Teaching Hospital, Nepal, 3Department of Gastroenterology, KIST Medical College and Teaching Hospital, Nepal, 4Department of Nephrology, KIST Medical College and Teaching Hospital, Nepal
Background : Combined liver-kidney transplantation emerges as a pivotal therapeutic intervention for individuals afflicted with primary hyperoxaluria type 1 (PH1), a rare genetic disorder characterized by excessive oxalate production leading to systemic complications. Here, we delve into the rationale, challenges, and outcomes associated with the combined liver-kidney transplantation (CLKT) as a definitive treatment of PH1.
Methods : We presented a case of 34-year-old female who underwent CLKT for PH1. Three and half year back, she developed pain abdomen and decreased urine output. On evaluation she was found to have renal calculi, renal parenchymal disease and increased serum creatinine level. Since then she was on maintenance hemodialysis (MHD) till CLKT. On genetic evaluation, a homozygous c.33dupC (p. Lys 12 Glnfs*156) pathogenic variant in AGXT gene was observed.
Results : Her plasma oxalate level was persistently high which may lead to kidney graft failure so we started high flux dialysis daily for continuous 9 days and it comes to 27.33 umol/L from 84.05 umol/L. We performed living donors combined liver-kidney transplantation on her. Her liver donor was her 35-year-old husband and her kidney donor was her 32-year-old sister in law. Laparoscopic donor nephrectomy and open surgery for modified right lobe of liver was performed on donors. She received induction with basiliximab and methylprednisolone, followed by maintenance immunosuppression with tacrolimus, mycophenolate mofetile and prednisone. Intraoperative continuous renal replacement therapy (CRRT) was started and in view of systemic hyperoxaluria we continued CRRT till post-operative day five. She was discharged on post-operative day nineteen.
Conclusions : In, conclusion, combined liver-kidney transplantation stands as a critical intervention for individuals with PH1, offering a comprehensive solution in this rare genetic disorder. While challenges persist, advancements in transplantation techniques and a deeper understanding of PH1 contribute to increasingly favorable outcomes, providing hope for quality of life for affected individuals.
Methods : We presented a case of 34-year-old female who underwent CLKT for PH1. Three and half year back, she developed pain abdomen and decreased urine output. On evaluation she was found to have renal calculi, renal parenchymal disease and increased serum creatinine level. Since then she was on maintenance hemodialysis (MHD) till CLKT. On genetic evaluation, a homozygous c.33dupC (p. Lys 12 Glnfs*156) pathogenic variant in AGXT gene was observed.
Results : Her plasma oxalate level was persistently high which may lead to kidney graft failure so we started high flux dialysis daily for continuous 9 days and it comes to 27.33 umol/L from 84.05 umol/L. We performed living donors combined liver-kidney transplantation on her. Her liver donor was her 35-year-old husband and her kidney donor was her 32-year-old sister in law. Laparoscopic donor nephrectomy and open surgery for modified right lobe of liver was performed on donors. She received induction with basiliximab and methylprednisolone, followed by maintenance immunosuppression with tacrolimus, mycophenolate mofetile and prednisone. Intraoperative continuous renal replacement therapy (CRRT) was started and in view of systemic hyperoxaluria we continued CRRT till post-operative day five. She was discharged on post-operative day nineteen.
Conclusions : In, conclusion, combined liver-kidney transplantation stands as a critical intervention for individuals with PH1, offering a comprehensive solution in this rare genetic disorder. While challenges persist, advancements in transplantation techniques and a deeper understanding of PH1 contribute to increasingly favorable outcomes, providing hope for quality of life for affected individuals.
SESSION
E-poster
E-Session 03/21 ALL DAY