Denys-Drash Syndrome and Risk of Post-transplant Lymphoproliferative Disorder
DRASH-PTLD
Post-transplant Lymphoproliferative Disorder Following Kidney Transplantation in Denys-Drash Syndrome: a Case-control Study
DRASH-PTLD
Post-transplant Lymphoproliferative Disorder Following Kidney Transplantation in Denys-Drash Syndrome: a Case-control Study
Sponsor study identifier
observational
108
1 country
7
Study Start
June 2026
Estimated (date passed)This estimate has passed. The registry has not confirmed an actual date for this milestone.
Primary Completion
June 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
Study Completion
June 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
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.
Registry-reported information. Elapsed time and enrollment totals alone do not measure study progress or recruitment pace.
June 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
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Dates and qualifiers reported by the registry. Estimated dates do not confirm that a milestone has occurred.
April 20, 2026
Actual (reported)May 26, 2026
Actual (reported)June 2026
Estimated (date passed)This estimate has passed. The registry has not confirmed an actual date for this milestone.
June 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
June 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
Occurrence of lymphoproliferative disorder
Occurrence of lymphoproliferative disorder, confirmed by anatomopathological analyses of lymph node biopsies or of another affected site.
Up to 24 years
Description of risk factors of post-transplant lymphoproliferative disorder following kidney transplantation
Up to 24 years
Comparison of the time to onset of post-transplant lymphoproliferative disorder following kidney transplantation
Up to 24 years
Patients who were minors at the time of kidney transplantation between 2000 and 2022.
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.
Collection of data from the patient's medical file. The data will be collected until 2024.
Registry-reported names are shown in full. Names without a link have not yet been matched to a Syfrah profile.
In the French cohort of patients followed for Denys Drash, between 2000 and 2022, 36 patients underwent kidney transplants.
Hôpital Femme Mère Enfant, Hospices Civils de Lyon
Bron, 69500, France
CHU de Lille
Lille, 59000, France
CHU Montpellier
Montpellier, 34000, France
Glenisson 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.
Lopez-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.
Mathilde M.D. Grapin
Assistance Publique - Hôpitaux de Paris
First Submitted
April 20, 2026
First Posted
May 26, 2026
Study Start
June 2026
Estimated (date passed)This estimate has passed. The registry has not confirmed an actual date for this milestone.
Primary Completion
June 2027
EstimatedHaemophilus Influenzae Pneumonia in Adults
Contact the study team to confirm eligibility.
CHU de Nancy - Hôpitaux de Brabois
Nancy, 54500, France
Hôpital enfants et adolescents, CHU Nantes
Nantes, 44093, France
Hôpital Necker-Enfants Malades
Paris, 75015, France
Hôpital Robert Debré
Paris, 75019, France
Fulchiero 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.
Mynarek 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.
Planned date reported by the registry; this milestone is not confirmed as achieved.
Study Completion
June 2027
EstimatedPlanned date reported by the registry; this milestone is not confirmed as achieved.
Last Updated
May 26, 2026
Record last verified: 2026-04
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Source: ClinicalTrials.gov.
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