ESPN 51th Annual Meeting

ESPN 2018


 
Combined or Sequential Liver and Kidney Transplantation in Children With Primary Hyperoxaluria Type-1: A Single-Center Experience
Burcu Bulum 1 Ali Özer 2 Tonguç Utku Yılmaz 2 Vildan Ertekin 3 Meltem Güner Can 4 İbrahim Halil Demir 5 Agop Çıtak 6 Ercüment Gürlüler 2 Remzi Emiroğlu 2

1- DEPARTMENT OF PEDIATRIC NEPHROLOGY, ACIBADEM MEHMET ALI AYDINLAR UNIVERSITY SCHOOL OF MEDICINE, ATAKENT ACIBADEM HOSPITAL, İSTANBUL, TURKEY.
2- DEPARTMENT OF TRANSPLANTATION CENTER, ACIBADEM MEHMET ALI AYDINLAR UNIVERSITY SCHOOL OF MEDICINE, ATAKENT ACIBADEM HOSPITAL, İSTANBUL, TURKEY.
3- Department of Pediatric Gastroenterology, Acıbadem Mehmet Ali Aydınlar University School of Medicine, Atakent Acıbadem Hospital, İstanbul, Turkey.
4- Department of Anesthesiology and Reanimation, Acıbadem Mehmet Ali Aydınlar University School of Medicine, Atakent Acıbadem Hospital, İstanbul, Turkey.
5- Department of Pediatric Cardiology, Atakent Acıbadem Hospital, İstanbul, Turkey.
6- Department of Pediatric Intensive Care Unit, Acıbadem Mehmet Ali Aydınlar University School of Medicine, Atakent Acıbadem Hospital, İstanbul, Turkey.
 
Introduction:

 

Primary hyperoxaluria type 1 (PH1) is a rare autosomal recessive disorder caused by mutations in the AGXT gene which leads to end stage renal disease (ESRD). Combined or sequential liver and kidney transplantation (CLKT or SLKT) is the definitive therapy for these patients.

Material and methods:

 

Here we report three pediatric PH1 patients with ESRD who had undergone CLKT or SLKT at our center.

Results:

 

Case 1: A 10.5 year-old girl was presented with kidney stones and nephrocalcinosis at two years. She had an exitus brother who had PH1 and consanguineous parents. She had homozygous c.584T>G (p.Met195Arg) mutation. She has been on hemodialysis (HD) since she was five and had severe systemic oxalosis. SLKT was performed from her mother. Her liver function tests are normal at first year of LT. She still has high urinary oxalate levels and creatinine is 1.1 mg/dl after eight months of KT.

Case 2: A 10 year-old boy was presented with kidney stones two years ago. He had consanguineous parents and an exitus brother with PH1. He had homozygous c.322T>C (p.TRP108Arg) mutation. HD was started four months ago. He hadn’t systemic oxalosis. CLKT was performed from his father. His liver and renal function tests (Cre: 0.6 mg/dl), and urinary oxalate levels are normal sixth months after CLKT.

Case 3: A six year-old boy who was presented with obstructive kidney stones and nephrocalcinosis was diagnosed with PH1 and started HD three months ago. He had consanguineous parents, and a brother and an exitus cousin with PH1. He had homozygous c.322T>C (p.TRP108Arg) mutation. He hadn’t systemic oxalosis. SLKT was performed from his mother. He has high urinary oxalate levels but normal renal functions (Cre: 0.6 mg/dl) at the first month of KT, and normal liver functions at fourth month of LT. 

Conclusions:

CLKT or SLKT are very uncommon procedures which should be performed by experienced surgical and multidisciplinary teams provides encouraging results in young children who had PH1. In our experience, we have achieved satisfactory results with meticulous preparation, successful surgery and very careful post operative management.