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.
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
History of lymphoproliferative disorder prior to transplantation
Other hematopoietic cancer
Other genetic disease with a proven increased risk of lymphoproliferative disorder
In the French cohort of patients followed for Denys Drash, between 2000 and 2022, 36 patients underwent kidney transplants.
Case-control
Retrospective
Collection of data from the patient's medical file
Other interventionCollection of data from the patient's medical file. The data will be collected until 2024.
Occurrence of lymphoproliferative disorder
Occurrence of lymphoproliferative disorder, confirmed by anatomopathological analyses of lymph node biopsies or of another affected site.
Description of risk factors of post-transplant lymphoproliferative disorder following kidney transplantation
Investigate risk factors for post-transplant lymphoproliferative disorder following kidney transplantation in patients with Denys Drash syndrome. Description of the following factors: increase or secondary positivity of Epstein-Barr virus PCR, donor and recipient Epstein-Barr virus serology before transplantation, patient age at the time of transplantation.
Comparison of the time to onset of post-transplant lymphoproliferative disorder following kidney transplantation
Time to onset of post-transplant lymphoproliferative disorder following kidney transplantation in patients with Denys-Drash syndrome compared to patients without this syndrome.
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Assistance Publique - Hôpitaux de Paris
Lead sponsor
URC-CIC Paris Descartes Necker Cochin
Collaborator
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