Combined liver-kidney transplantation for primary hyperoxaluria type I in children: Single Center Experience
Corresponding Author
Magd A. Kotb
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Correspondence
Magd Ahmed Kotb, Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt.
Email: [email protected]
Search for more papers by this authorAlaa F. Hamza
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Faculty of Medicine, Department of Pediatric Surgery, Ain Shams University, Cairo, Egypt
Search for more papers by this authorHesham Abd El Kader
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Faculty of Medicine, Department of Pediatric Surgery, Ain Shams University, Cairo, Egypt
Search for more papers by this authorMagda El Monayeri
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Faculty of Medicine, Department of Pathology, Ain Shams University, Cairo, Egypt
Search for more papers by this authorDalia S. Mosallam
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Search for more papers by this authorNazira Ali
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Search for more papers by this authorChristine William Shaker Basanti
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Search for more papers by this authorHafez Bazaraa
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Egyptian Group for Orphan Renal Diseases (EGORD), Cairo, Egypt
Search for more papers by this authorHany Abdelrahman
Pediatric Oncology, National Cancer Institute, Cairo University, Cairo, Egypt
Search for more papers by this authorMarwa M. Nabhan
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Egyptian Group for Orphan Renal Diseases (EGORD), Cairo, Egypt
Search for more papers by this authorHend Abd El Baky
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Search for more papers by this authorSally T. Mostafa El Sorogy
Department of Pediatrics, Public Mounira Hospital, Cairo, Egypt
Search for more papers by this authorInas E.M. Kamel
Department of Pediatrics, National Research Center, Cairo, Egypt
Search for more papers by this authorHoda Ismail
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Department of Pediatrics, Wadi El Nil Hospital, Cairo, Egypt
Search for more papers by this authorYasmin Ramadan
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Egyptian Group for Orphan Renal Diseases (EGORD), Cairo, Egypt
Search for more papers by this authorSafaa M. Abd El Rahman
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Pediatric Oncology, National Cancer Institute, Cairo University, Cairo, Egypt
Search for more papers by this authorNeveen A. Soliman
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Egyptian Group for Orphan Renal Diseases (EGORD), Cairo, Egypt
Search for more papers by this authorCorresponding Author
Magd A. Kotb
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Correspondence
Magd Ahmed Kotb, Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt.
Email: [email protected]
Search for more papers by this authorAlaa F. Hamza
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Faculty of Medicine, Department of Pediatric Surgery, Ain Shams University, Cairo, Egypt
Search for more papers by this authorHesham Abd El Kader
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Faculty of Medicine, Department of Pediatric Surgery, Ain Shams University, Cairo, Egypt
Search for more papers by this authorMagda El Monayeri
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Faculty of Medicine, Department of Pathology, Ain Shams University, Cairo, Egypt
Search for more papers by this authorDalia S. Mosallam
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Search for more papers by this authorNazira Ali
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Search for more papers by this authorChristine William Shaker Basanti
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Search for more papers by this authorHafez Bazaraa
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Egyptian Group for Orphan Renal Diseases (EGORD), Cairo, Egypt
Search for more papers by this authorHany Abdelrahman
Pediatric Oncology, National Cancer Institute, Cairo University, Cairo, Egypt
Search for more papers by this authorMarwa M. Nabhan
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Egyptian Group for Orphan Renal Diseases (EGORD), Cairo, Egypt
Search for more papers by this authorHend Abd El Baky
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Search for more papers by this authorSally T. Mostafa El Sorogy
Department of Pediatrics, Public Mounira Hospital, Cairo, Egypt
Search for more papers by this authorInas E.M. Kamel
Department of Pediatrics, National Research Center, Cairo, Egypt
Search for more papers by this authorHoda Ismail
Wadi El Nil Hospital, Pediatric Living-Related Liver Transplantation Team, Cairo, Egypt
Department of Pediatrics, Wadi El Nil Hospital, Cairo, Egypt
Search for more papers by this authorYasmin Ramadan
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Egyptian Group for Orphan Renal Diseases (EGORD), Cairo, Egypt
Search for more papers by this authorSafaa M. Abd El Rahman
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Pediatric Oncology, National Cancer Institute, Cairo University, Cairo, Egypt
Search for more papers by this authorNeveen A. Soliman
Pediatric Hepatology Unit, Faculty of Medicine, Department of Pediatrics, Cairo University, Cairo, Egypt
Department of Pediatrics, Center of Pediatric Nephrology & Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Egyptian Group for Orphan Renal Diseases (EGORD), Cairo, Egypt
Search for more papers by this authorAbstract
Primary hyperoxalurias are rare inborn errors of metabolism with deficiency of hepatic enzymes that lead to excessive urinary oxalate excretion and overproduction of oxalate which is deposited in various organs. Hyperoxaluria results in serious morbid-ity, end stage kidney disease (ESKD), and mortality if left untreated. Combined liver kidney transplantation (CLKT) is recognized as a management of ESKD for children with hyperoxaluria type 1 (PH1). This study aimed to report outcome of CLKT in a pediatric cohort of PH1 patients, through retrospective analysis of data of 8 children (2 girls and 6 boys) who presented by PH1 to Wadi El Nil Pediatric Living Related Liver Transplant Unit during 2001-2017. Mean age at transplant was 8.2 ± 4 years. Only three of the children underwent confirmatory genotyping. Three patients died prior to surgery on waiting list. The first attempt at CLKT was consecutive, and despite initial successful liver transplant, the girl died of biliary peritonitis prior to scheduled renal transplant. Of the four who underwent simultaneous CLKT, only two survived and are well, one with insignificant complications, and other suffered from abdominal Burkitt lymphoma managed by excision and resection anastomosis, four cycles of rituximab, cyclophosphamide, vincristine, and prednisone. The other two died, one due to uncontrollable bleeding within 36 hours of procedure, while the other died awaiting renal transplant after loss of renal graft to recurrent renal oxalosis 6 months post-transplant. PH1 with ESKD is a rare disease; simultaneous CLKT offers good quality of life for afflicted children. Graft shortage and renal graft loss to oxalosis challenge the outcome.
CONFLICT OF INTEREST
There are no competing interests, and no trial funding.
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