Abstract: PUB228
Challenges in a Pediatric Donor with Horseshoe Kidney
Session Information
Category: Transplantation
- 2002 Transplantation: Clinical
Author
- Duggal, Tanu, Baylor Scott and White Central Texas, Temple, Texas, United States
Introduction
Donors with horseshoe kidney present unique challenges. A pediatric donor with possible undiagnosed syndrome can further compromise outcomes
Case Description
37yr old Male(M) with ESRD from autosomal dominant polycystic kidney disease had deceased donor transplant of horseshoe kidney from a 2yr old M. Donor had medical history of hypertension, bicuspid aortic valve, patent ductus arteriosus & leukocoria requiring corneal transplants.
Donation was post brain death due to cerebral anoxia & Creatinine(Cr) was 0.4mg/dL. Immunosuppression was Tacrolimus, Mycophenolate mofetil and Prednisone. Recipient's Cr was 2mg/dL, 3 month(mo) Post Transplant (PTx).
By 6mo, nephrotic-range proteinuria developed, Cr rose to 3.4 mg/dL. Allograft biopsy showed 2 sclerosed glomeruli & 2 with collapsing glomerulopathy(CG). Trial of angiotensin receptor blockade was complicated by rise in Cr to 5.6 mg/dL. Renal ultrasonography did not show stenosis. Second biopsy, 8mo PTx showed thin glomerular basement membranes(TBM), mean thickness 260nm (normal 370 ± 45nm adult M) & focal thinning as low as 217nm with focal disruption.
Third biopsy, 9 mo PTx revealed 2 glomeruli with FSGS. Distinction between donor-derived and de novo FSGS could not be made. An attempt at transitioning from Tacrolimus to Belatacept was undertaken but did not halt allograft deterioration and recipient became dialysis dependent 10 mo PTx.
Discussion
This case shows early allograft loss in a recipient of pediatric horseshoe kidney from donor with an undiagnosed syndrome.
CG is classically associated with APOL1 high-risk variants, and viral infections; however, it has also been described in context of ischemia-reperfusion injury & donor-derived nephropathies. This donor's presentation raises possibility of an undiagnosed gn. Significance of TBM is also speculative.Failure of all interventions underscores the limited options available once progressive glomerular injury is established.
This case raises important questions about pre-transplant risk stratification.Risks and benefits of accepting donors with undiagnosed syndromes must be carefully weighed.