Registry for Patients With Wilms' Tumor Suppressor Gene 1 (WT1) Mutation Associated Diseases

NCT ID: NCT01252901

Last Updated: 2015-05-28

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

52 participants

Study Classification

OBSERVATIONAL

Study Start Date

2010-10-31

Study Completion Date

2014-06-30

Brief Summary

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The Wilms' tumor suppressor gene 1 (WT1) encodes for a transcription factor which plays an important role during urogenital development. Patients carrying a WT1 germline mutation show symptoms like proteinuria, Wilms tumors, genital malformations and kidney failure. Milder variants are possible and classification is not always possible. In this registry we are collecting detailed clinical data of affected individuals to establish genotype-phenotype correlations with the greater goal to optimize patient care.

Detailed Description

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The Wilms' tumor suppressor gene 1 (WT1) encodes for a transcription factor which plays an important role during urogenital development. Patients carrying a WT1 germline mutation show symptoms like proteinuria, Wilms tumors, genital malformations and kidney failure, which usually occur in early childhood. Milder variants are possible and classification as one of the three rare syndromes associated with WT1 mutations (Denys-Drash syndrome, Frasier syndrome or WAGR syndrome) is not always possible. In this registry we are collecting detailed clinical data of affected individuals to establish genotype-phenotype correlations with the greater goal to optimize patient care.

Conditions

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Denys-Drash Syndrome Frasier Syndrome Nephrotic Syndrome Wilms Tumor

Study Design

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Observational Model Type

COHORT

Study Time Perspective

RETROSPECTIVE

Eligibility Criteria

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Inclusion Criteria

* Germline mutation in WT1 gene

Exclusion Criteria

* none
Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Universitätsklinikum Hamburg-Eppendorf

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Anja Lehnhardt, MD

Role: PRINCIPAL_INVESTIGATOR

Universitätsklinikum Hamburg-Eppendorf

Locations

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Universitätskrankenhaus Hamburg-Eppendorf

Hamburg, , Germany

Site Status

Countries

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Germany

References

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Royer-Pokora B, Beier M, Henzler M, Alam R, Schumacher V, Weirich A, Huff V. Twenty-four new cases of WT1 germline mutations and review of the literature: genotype/phenotype correlations for Wilms tumor development. Am J Med Genet A. 2004 Jun 15;127A(3):249-57. doi: 10.1002/ajmg.a.30015.

Reference Type BACKGROUND
PMID: 15150775 (View on PubMed)

Mucha B, Ozaltin F, Hinkes BG, Hasselbacher K, Ruf RG, Schultheiss M, Hangan D, Hoskins BE, Everding AS, Bogdanovic R, Seeman T, Hoppe B, Hildebrandt F; Members of the APN Study Group. Mutations in the Wilms' tumor 1 gene cause isolated steroid resistant nephrotic syndrome and occur in exons 8 and 9. Pediatr Res. 2006 Feb;59(2):325-31. doi: 10.1203/01.pdr.0000196717.94518.f0.

Reference Type BACKGROUND
PMID: 16439601 (View on PubMed)

Ruf RG, Schultheiss M, Lichtenberger A, Karle SM, Zalewski I, Mucha B, Everding AS, Neuhaus T, Patzer L, Plank C, Haas JP, Ozaltin F, Imm A, Fuchshuber A, Bakkaloglu A, Hildebrandt F; APN Study Group. Prevalence of WT1 mutations in a large cohort of patients with steroid-resistant and steroid-sensitive nephrotic syndrome. Kidney Int. 2004 Aug;66(2):564-70. doi: 10.1111/j.1523-1755.2004.00775.x.

Reference Type BACKGROUND
PMID: 15253707 (View on PubMed)

Kist-van Holthe JE, Ho PL, Stablein D, Harmon WE, Baum MA. Outcome of renal transplantation for Wilms' tumor and Denys-Drash syndrome: a report of the North American Pediatric Renal Transplant Cooperative Study. Pediatr Transplant. 2005 Jun;9(3):305-10. doi: 10.1111/j.1399-3046.2005.00311.x.

Reference Type BACKGROUND
PMID: 15910385 (View on PubMed)

Aucella F, Bisceglia L, De Bonis P, Gigante M, Caridi G, Barbano G, Mattioli G, Perfumo F, Gesualdo L, Ghiggeri GM. WT1 mutations in nephrotic syndrome revisited. High prevalence in young girls, associations and renal phenotypes. Pediatr Nephrol. 2006 Oct;21(10):1393-8. doi: 10.1007/s00467-006-0225-0. Epub 2006 Aug 15.

Reference Type BACKGROUND
PMID: 16909243 (View on PubMed)

Chesney RW. Why is the oncogene WT1 in the developing kidney and what is it doing there? Pediatr Nephrol. 2002 Dec;17(12):990-2. doi: 10.1007/s00467-002-0927-x. Epub 2002 Oct 1. No abstract available.

Reference Type BACKGROUND
PMID: 12520325 (View on PubMed)

Klamt B, Koziell A, Poulat F, Wieacker P, Scambler P, Berta P, Gessler M. Frasier syndrome is caused by defective alternative splicing of WT1 leading to an altered ratio of WT1 +/-KTS splice isoforms. Hum Mol Genet. 1998 Apr;7(4):709-14. doi: 10.1093/hmg/7.4.709.

Reference Type BACKGROUND
PMID: 9499425 (View on PubMed)

Pelletier J, Bruening W, Kashtan CE, Mauer SM, Manivel JC, Striegel JE, Houghton DC, Junien C, Habib R, Fouser L, et al. Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome. Cell. 1991 Oct 18;67(2):437-47. doi: 10.1016/0092-8674(91)90194-4.

Reference Type BACKGROUND
PMID: 1655284 (View on PubMed)

Related Links

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http://www.uke.de

University Medical Center Hamburg-Eppendorf

http://www.gp-nephrologie.de

Gesellschaft für Pädiatrische Nephrologie

http://www.netzwerk-is.de/

DSD Network for Disorders of Sex Development

http://www.kinderkrebsinfo.de

Das Informationsportal der Gesellschaft für Pädiatrische Onkologie und Hämatologie

Other Identifiers

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WT1 Registry

Identifier Type: -

Identifier Source: org_study_id

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