Denys-Drash Syndrome and Risk of Post-transplant Lymphoproliferative Disorder

ClinicalTrials.gov ID

NCT07605884

Lead Sponsor

Assistance Publique - Hôpitaux de ParisOTHER

Overall Status

Not yet recruiting

Study Type

Observational

Enrollment

108Estimated

Last Update Posted

May 26, 2026Actual

Start Date

Jun 2026Estimated

Primary Completion Date

Jun 2027Estimated

Completion Date

Jun 2027Estimated

Official Title

Post-transplant Lymphoproliferative Disorder Following Kidney Transplantation in Denys-Drash Syndrome: a Case-control Study

Brief Summary

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.

Detailed Description

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.

The study hypothesizes that there is an increased risk of lymphoproliferative disorder in children with Denys-Drash syndrome. If this hypothesis is confirmed, it would allow for the development of active treatment methods to combat post-transplant lymphoproliferative disorder in this population.

• Denys-Drash Syndrome

• Collection of data from the patient's medical file

Ages Eligible for Study

Child (0-17)Adult (18-64)Older Adult (65+)

Sexes Eligible for Study

All

Accepts Healthy Volunteers

No

Eligibility Criteria

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

Observational Model

Case-Control

Time Perspective

Retrospective

Lead Sponsor

Assistance Publique - Hôpitaux de ParisOTHER

Collaborators

URC-CIC Paris Descartes Necker CochinOTHER

robert.novo@chu-lille.fr

gwenaelle.roussey@chu-nantes.fr

mathilde.grapin@aphp.fr0033142192726

julien.hogan@aphp.fr

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