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Updated: Aug 9, 2026
Robot-Assisted Kidney Transplantation
Published on: July 19, 2021
Kidney transplantation outcomes in children with WT1-associated kidney disease: a single-center cohort study
Reyila Abasi1, Lin Zhilang1, Jiang Mengjie1
1Department of Pediatric Nephrology and Rheumatology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
Insights
Kidney transplantation in children with WT1-associated kidney disease shows promising graft survival. Careful monitoring for tumors, gonadal issues, and viral infections is crucial post-transplant.
Area of Science:
- Pediatric Nephrology
- Transplantation Immunology
- Clinical Genetics
Background:
- WT1-associated kidney disease causes early-onset end-stage kidney disease (ESKD) in children.
- This condition presents challenges for kidney transplantation due to associated Wilms tumor, gonadal tumors, and disorders of sex development (DSD).
Purpose of the Study:
- To evaluate the outcomes of kidney transplantation in children with WT1-associated kidney disease.
- To identify key factors for post-transplant management in this cohort.
Main Methods:
- Retrospective analysis of 13 children with WT1-associated kidney disease who underwent kidney transplantation.
- Review of clinical characteristics, genetic findings, transplant outcomes, and post-transplant complications.
Main Results:
- Median follow-up was 32 months with all grafts functional (median eGFR 68.7 mL/min/1.73 m²).
- Three patients developed Wilms tumor; three phenotypic females had 46,XY karyotype with gonadal dysgenesis.
- Twelve WT1 variants were identified; Epstein-Barr virus viremia occurred in five patients, with two developing post-transplant lymphoproliferative disorder. No disease recurrence was observed.
Conclusions:
- Kidney transplantation offers favorable short- to mid-term graft outcomes for children with WT1-associated kidney disease.
- Multidisciplinary surveillance for tumors, gonadal abnormalities, and viral complications is essential for optimal post-transplant care.
Introduction:
WT1-associated kidney disease is an important cause of early-onset end-stage kidney disease (ESKD) in children and may be accompanied by Wilms tumor, gonadal tumors, and disorders of sex development (DSD), creating distinctive challenges for kidney transplantation and post-transplant management.
Methods:
We retrospectively analyzed 13 children with WT1-associated kidney disease who underwent kidney transplantation at a single center between February 2020 and October 2025. Clinical characteristics, genetic findings, transplant outcomes, and post-transplant complications were reviewed.
Results:
The median age at disease onset was 2.3 years, the median age at progression to ESKD was 4.1 years, and the median age at transplantation was 5.3 years. Three patients developed Wilms tumor, and three phenotypic females had a 46,XY karyotype with gonadal dysgenesis. Twelve distinct WT1 variants were identified, predominantly exon 9 missense variants and intron 9-10 splice-site variants. After a median follow-up of 32 months, all grafts remained functional, with a median estimated glomerular filtration rate of 68.7 mL/min/1.73 m². Epstein-Barr virus viremia occurred in five patients, and two developed post-transplant lymphoproliferative disorder. No disease recurrence was observed.
Discussion:
Kidney transplantation achieved favorable short- to mid-term graft outcomes in this cohort. Multidisciplinary surveillance of tumor history, gonadal abnormalities, and post-transplant viral complications remains essential.
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