ESPN 53rd Annual Meeting

ESPN 2021


 
First report of long-term outcome of Combined Liver and Kidney Transplantation in severe Protein C deficiency
MADURI RAJA 1 MANINDER BAL 1 DAVID V MILFORD 2 MUSHFEQUR R HAQ 1 RODNEY D GILBERT 1 PATRICK MCKIERNAN 2 ARVIND NAGRA 1

1- 1. SOUTHAMPTON CHILDREN’S HOSPITAL, UNIVERSITY HOSPITAL SOUTHAMPTON NHS FOUNDATION TRUST, TREMONA ROAD, SOUTHAMPTON SO16 6YD, UNITED KINGDOM
2- 2. BIRMINGHAM WOMEN’S AND CHILDREN’S HOSPITAL NHS FOUNDATION TRUST, STEELHOUSE LANE, BIRMINGHAM, B4 6NH, UK.
 
Introduction:

The long-term outcome of combined liver and kidney transplantation (CLKT) in a 13-year-old boy with end stage kidney disease secondary to homozygous protein C deficiency (PCD).

Material and methods:

Retrospective review of case notes, kidney function and protein C (PrC) levels from birth to 13 years of age (10 years post-transplantation); Cost comparison pre and post CLKT.

Results:

This is the first report of 10-year post-CLKT outcome in a child with PCD born with bilateral renal venous thrombosis resulting in end stage kidney disease. He had worsening kidney function due to recurrent thrombotic episodes secondary to undetectable PrC levels. On PCD diagnosis at 7 months of age he started activated PrC infusions (levels maintained >0.25IU/ml) to prevent thrombosis. He received a deceased donor CLKT at age 3 years. PrC levels were maintained above 100IU/ml peri-operatively to prevent thrombosis and were discontinued 48 hours post-CLKT when levels were 1.03IU/ml. 10 years post CLKT they remain normal (0.79IU/ml) with no thrombotic episodes since CLKT and he has had no further requirement for exogenous PrC. Kidney function: His creatinine improved rapidly from 350µmol/l pre-CLKT to 30µmol/l post-CLKT. Kidney function remained stable but deteriorated 6 years post-CLKT secondary to antibody mediated rejection. This responded to increased immunosuppression. Cost analysis: Pre-CLKT PrC infusions cost £182,000/year. The cost of the CLKT was approximately £130,000 for the first year. 10 years post-CKLT, over £1,000,000 has been saved on PrC infusions alone and no requirement for long-term dialysis.

Conclusions:

CLKT in PCD with early renal involvement restores hepatic synthesis of PrC and provides definitive renal replacement therapy. CLKT has few long-term complications and is a cost-effective treatment option.  This first reported case suggests CLKT should be considered as a treatment option in similar cases of PCD.