Clinical trial · Observational
Denys-Drash Syndrome and Risk of Post-transplant Lymphoproliferative Disorder
Post-transplant Lymphoproliferative Disorder Following Kidney Transplantation in Denys-Drash Syndrome: a Case-control Study
- Source
- ClinicalTrials.gov
- Retrieved
- Sep 8, 2026
- Layer
- normalized (units and labels harmonized; values unchanged)
- Run
- ING-CLINICALTRIALS-20260908-000001
Summary
Brief summary (as posted)
Denys-Drash syndrome is a rare genetic disorder of childhood characterized by nephrotic syndrome, nephroblastomas, and genital developmental abnormalities. These children present with rapidly progressive renal failure, leading to kidney transplantation at a median age of 3.6 years. In a study of the French cohort of patients with Denys-Drash syndrome, a high risk of lymphoproliferative syndrome was observed (20%). This frequency is significantly higher than in the general transplant population (4%). The aim of the study is to evaluate the risk of post-transplant lymphoproliferative disorder following kidney transplantation in patients with Denys-Drash syndrome compared to patients with kidney transplant patients without Denys-Drash syndrome.
Conditions
Conditions (1)
Free-text conditions as registered, with the CancerIndex entity they were reconciled to and the match type.
| Condition (as posted) | Mapped entity | Match | Confidence |
|---|---|---|---|
| Denys-Drash Syndrome | — | UNRESOLVED | — |
Interventions
Interventions (1)
| Intervention | Type | Mapped drug | Match |
|---|---|---|---|
| Collection of data from the patient's medical file | Other | — | UNRESOLVED |
Design
Arms and outcomes
Arms (2)
- label
- Patients with Denys-Drash syndrome
- description
- Patients who were minors at the time of kidney transplantation between 2000 and 2022.
- interventionNames
- Other: Collection of data from the patient's medical file
- label
- Control patients
- description
- The controls will be matched in chronological order of transplantation with patients with Denys-Drash syndrome: a control before and a control after, matched according to the Epstein-Barr virus status of the pretransplant recipient, the Epstein-Barr virus status of the donor and the age of transplantation.
- interventionNames
- Other: Collection of data from the patient's medical file
Primary outcomes (1)
- measure
- Occurrence of lymphoproliferative disorder
- timeFrame
- Up to 24 years
Eligibility
Eligibility (as posted)
- Sex
- All
Show eligibility criteria text
Inclusion Criteria: * Patient must be a minor at the time of kidney transplantation * Diagnosis of Denys-Drash syndrome (WT1 pathogenic variants in exons 8 or 9) for cases * Controls: minor patient, kidney transplanted at the same center as the case, immediately before and immediately after the case * Kidney transplant recipient on immunosuppressants * Hospital follow-up in a participating center in France * Regardless of their Epstein-Barr virus status before transplantation * Between 2000 and 2022 * Holders of parental authority or adult patients informed of the study and not objecting to the processing of medical data for the study Exclusion Criteria: * History of lymphoproliferative disorder prior to transplantation * Other hematopoietic cancer * Other genetic disease with a proven increased risk of lymphoproliferative disorder
References
Publications (4)
- BACKGROUNDGlenisson M, Grapin M, Blanc T, Preka E, Hogan J, Aurelle M, Roussey G, Mouche A, Rousset-Rouviere C, Novo R, Faudeux C, Fila M, Vrillon I, Cloarec S, Simon T, Harambat J, Casado EM, Rod J, Lecoindre MC, Heidet L, Boyer O, Garcelon N, Kachmar J, Dorval G, Sarnacki S. Genotype-Phenotype Correlations in Denys-Drash Syndrome in Children. Kidney Int Rep. 2025 Jan 16;10(4):1205-1212. doi: 10.1016/j.ekir.2025.01.014. eCollection 2025 Apr. PMID 40303223
- BACKGROUNDLopez-Gonzalez M, Ariceta G. WT1-related disorders: more than Denys-Drash syndrome. Pediatr Nephrol. 2024 Sep;39(9):2601-2609. doi: 10.1007/s00467-024-06302-y. Epub 2024 Feb 7. PMID 38326647
- BACKGROUNDFulchiero R, Amaral S. Post-transplant lymphoproliferative disease after pediatric kidney transplant. Front Pediatr. 2022 Dec 7;10:1087864. doi: 10.3389/fped.2022.1087864. eCollection 2022. PMID 36568415
- BACKGROUNDMynarek M, Hussein K, Kreipe HH, Maecker-Kolhoff B. Malignancies after pediatric kidney transplantation: more than PTLD? Pediatr Nephrol. 2014 Sep;29(9):1517-28. doi: 10.1007/s00467-013-2622-5. Epub 2013 Sep 24. PMID 24061645