Kidney Week

Abstract: TH-PO1220

Native Nephrectomy and Early Kidney Transplant Outcomes in ARPKD

Session Information

• Pediatric Nephrology: CV Health, CKD, AKI, Dialysis, Transplantation, and Health Services Research
  October 22, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

• 1800 Pediatric Nephrology

Authors

• Choudhry, Aksa, University of Pennsylvania, Philadelphia, Pennsylvania, United States

Aksa Choudhry

• Amaral, Sandra, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States

Sandra Amaral, MD, MHS

• Hartung, Erum Aftab, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States

Erum Aftab Hartung, MD, MS

Background

ARPKD is an important cause of childhood kidney failure and causes diffusely microcystic, enlarged kidneys. Some patients undergo native nephrectomy (NN), either in infancy to try to improve respiration or nutrition, or later in preparation for kidney transplant (KT). We aimed to examine associations between NN and KT outcomes in ARPKD and examine clinical differences between patients who did vs. did not undergo NN and those who underwent NN prior to vs. at the same time as KT.

Methods

Retrospective study of children with ARPKD who underwent KT at a single center from 2006-2020. Outcomes included post-KT hospital length of stay (LOS) and eGFR at 1 mo, 1 yr, and 2 yrs. Clinical variables included age at KT, living (LD) vs. deceased (DD) donor, age at NN, and height-adjusted average native kidney length on ultrasound closest to NN or KT.

Results

17 patients with ARPKD underwent KT at a median (IQR) age of 6.9 (3.2, 13.4) yrs, with 47% LD, 53% DD, 94% KT-only, and 6% combined liver-kidney transplant (CKLT). NN was performed in 10 (59%) patients (1 unilateral, 1 staged bilateral, 8 simultaneous bilateral) at a median (IQR) age of 2.5 (1.0, 6.5) yrs. Children who had NN (vs. those who did not) were significantly younger at KT (3.2 vs. 13.4 yrs, p=0.006), had higher height-adjusted kidney length (median 18.8 cm/m vs. 8.2 cm/m, p=0.001), higher rates of LDKT (70% vs. 14%, p=0.05), similar post-KT hospital LOS (11.5 vs. 12.5 days, p=0.9), higher eGFR at 1 mo (117 vs. 63, p=0.008), and similar eGFR at 1 and 2 yrs (Table). Of the 8 patients with simultaneous bilateral NN, 5 were prior to KT and 3 were on the same day as KT/CKLT. Median age at KT (2.7 vs. 3.2 years), height-adjusted kidney length (20.2 vs. 19.2 cm/m), post-KT hospital LOS (11 vs. 15 days), and eGFR at 1 mo (102 vs. 102 mL/min/1.73m²) were similar between these two groups (all p=NS).

Conclusion

Children with ARPKD who underwent NN were significantly younger at KT and had larger native kidneys. Outcomes were similar between patients with vs. without NN and between those with NN prior to vs. at the same time as KT.

Table

Funding

• Private Foundation Support