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Denys-Drash Syndrome and Risk of Post-transplant Lymphoproliferative Disorder (DRASH-PTLD)

18. maj 2026 opdateret af: Assistance Publique - Hôpitaux de Paris

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

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.

Studieoversigt

Status

Ikke rekrutterer endnu

Betingelser

Intervention / Behandling

Detaljeret beskrivelse

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.

Undersøgelsestype

Observationel

Tilmelding (Anslået)

108

Kontakter og lokationer

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Studiekontakt

Undersøgelse Kontakt Backup

Studiesteder

Deltagelseskriterier

Forskere leder efter personer, der passer til en bestemt beskrivelse, kaldet berettigelseskriterier. Nogle eksempler på disse kriterier er en persons generelle helbredstilstand eller tidligere behandlinger.

Berettigelseskriterier

Aldre berettiget til at studere

  • Barn
  • Voksen
  • Ældre voksen

Tager imod sunde frivillige

Ingen

Prøveudtagningsmetode

Ikke-sandsynlighedsprøve

Studiebefolkning

In the French cohort of patients followed for Denys Drash, between 2000 and 2022, 36 patients underwent kidney transplants.

Beskrivelse

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

Studieplan

Dette afsnit indeholder detaljer om studieplanen, herunder hvordan undersøgelsen er designet, og hvad undersøgelsen måler.

Hvordan er undersøgelsen tilrettelagt?

Design detaljer

Kohorter og interventioner

Gruppe / kohorte
Intervention / Behandling
Patients with Denys-Drash syndrome
Patients who were minors at the time of kidney transplantation between 2000 and 2022.
Andet: Collection of data from the patient's medical file
Collection of data from the patient's medical file. The data will be collected until 2024.
Control patients
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.
Andet: Collection of data from the patient's medical file
Collection of data from the patient's medical file. The data will be collected until 2024.

Hvad måler undersøgelsen?

Primære resultatmål

Resultatmål
Foranstaltningsbeskrivelse
Tidsramme
Occurrence of lymphoproliferative disorder
Tidsramme: Up to 24 years
Occurrence of lymphoproliferative disorder, confirmed by anatomopathological analyses of lymph node biopsies or of another affected site.
Up to 24 years

Sekundære resultatmål

Resultatmål
Foranstaltningsbeskrivelse
Tidsramme
Description of risk factors of post-transplant lymphoproliferative disorder following kidney transplantation
Tidsramme: Up to 24 years

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.
Up to 24 years
Comparison of the time to onset of post-transplant lymphoproliferative disorder following kidney transplantation
Tidsramme: Up to 24 years
Time to onset of post-transplant lymphoproliferative disorder following kidney transplantation in patients with Denys-Drash syndrome compared to patients without this syndrome.
Up to 24 years

Samarbejdspartnere og efterforskere

Det er her, du vil finde personer og organisationer, der er involveret i denne undersøgelse.

Sponsor

Assistance Publique - Hôpitaux de Paris

Samarbejdspartnere

URC-CIC Paris Descartes Necker Cochin

Efterforskere

  • Ledende efterforsker: Mathilde M.D. Grapin, Assistance Publique - Hôpitaux de Paris

Publikationer og nyttige links

Den person, der er ansvarlig for at indtaste oplysninger om undersøgelsen, leverer frivilligt disse publikationer. Disse kan handle om alt relateret til undersøgelsen.

Generelle publikationer

Datoer for undersøgelser

Disse datoer sporer fremskridtene for indsendelser af undersøgelsesrekord og resumeresultater til ClinicalTrials.gov. Studieregistreringer og rapporterede resultater gennemgås af National Library of Medicine (NLM) for at sikre, at de opfylder specifikke kvalitetskontrolstandarder, før de offentliggøres på den offentlige hjemmeside.

Studer store datoer

Studiestart (Anslået)

1. juni 2026

Primær færdiggørelse (Anslået)

1. juni 2027

Studieafslutning (Anslået)

1. juni 2027

Datoer for studieregistrering

Først indsendt

20. april 2026

Først indsendt, der opfyldte QC-kriterier

18. maj 2026

Først opslået (Faktiske)

26. maj 2026

Opdateringer af undersøgelsesjournaler

Sidste opdatering sendt (Faktiske)

26. maj 2026

Sidste opdatering indsendt, der opfyldte kvalitetskontrolkriterier

18. maj 2026

Sidst verificeret

1. april 2026

Mere information

Begreber relateret til denne undersøgelse

Nøgleord

Yderligere relevante MeSH-vilkår

Andre undersøgelses-id-numre

  • APHP251805

Plan for individuelle deltagerdata (IPD)

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Kliniske forsøg med Denys-Drash syndrom

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