The Denys-Drash syndrome (Q28247154)

Journal of Medical Genetics

Europe PubMed Central

title

The Denys-Drash syndrome (English)

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R F Mueller

1

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language of work or name

English

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publication date

June 1994

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published in

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volume

31

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issue

6

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page(s)

471-7

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cites work

A syndrome of pseudohermaphroditism, Wilms' tumor, hypertension, and degenerative renal disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Association of an anatomo-pathological syndrome of male pseudohermaphroditism, Wilms' tumor, parenchymatous nephropathy and XX/XY mosaicism

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Homozygous deletion in Wilms tumours of a zinc-finger gene identified by chromosome jumping

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Zinc finger point mutations within the WT1 gene in Wilms tumor patients

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

GONADOBLASTOMA ASSOCIATED WITH PURE GONADAL DYSGENESIS IN MONOZYGOUS TWINS.

20 March 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Mutational screening of the Wilms's tumour gene, WT1, in males with genital abnormalities

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Nephrotic syndrome in the 1st year of life

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Chronic renal disease, myotonic dystrophy, and gonadoblastoma in XY gonadal dysgenesis.

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Wilms' tumor and glomerular disease. Occurrence with features of membranoproliferative glomerulonephritis and secondary focal, segmental glomerulosclerosis

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Mutation and cancer: a model for Wilms' tumor of the kidney

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Chronic renal failure and XY gonadal dysgenesis: "Frasier" syndrome--a commentary on reported cases

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Genetic mechanisms of tumor-specific loss of 11p DNA sequences in Wilms tumor

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Pseudohermaphroditism, glomerulopathy, and Wilms tumor (Drash syndrome): frequency in end-stage renal failure

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

The Drash syndrome revisited: diagnosis and follow-up

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

The association of Wilms' tumor, male pseudohermaphroditism and diffuse glomerular disease (Drash syndrome): report of eight cases with clinical and morphologic findings and review of the literature

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

A report of 4 patients with the Drash syndrome and a review of the literature

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Binding of the Wilms' tumor locus zinc finger protein to the EGR-1 consensus sequence

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Clinicopathologic review of twelve children with nephropathy, Wilms tumor, and genital abnormalities (Drash syndrome).

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

The candidate Wilms' tumour gene is involved in genitourinary development

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

An internal deletion within an 11p13 zinc finger gene contributes to the development of Wilms' tumor

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Identification and characterization of the Egr-1 gene product, a DNA-binding zinc finger protein induced by differentiation and growth signals

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Evidence for WT1 as a Wilms tumor (WT) gene: intragenic germinal deletion in bilateral WT

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Alternative splicing and genomic structure of the Wilms tumor gene WT1

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

End-stage renal disease and primary hypogonadism associated with a 46,XX karyotype

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

XY gonadal dysgenesis with gonadoblastoma discovered after kidney transplantation

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Association of Wilms' tumor, pseudohermaphroditism and glomerular nephropathy. Partial and complete forms of the syndrome

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Congenital anomalies in children with Wilms' Tumor. A new survey

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Pseudohermaphroditism, glomerulonephritis with the nephrotic syndrome, and Wilms' tumor in infancy

29 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Complete and incomplete Drash syndrome: a clinicopathologic study of five cases of a dysontogenetic-neoplastic complex.

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

MR imaging in Drash syndrome

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Loss of heterozygosity in Wilms' tumors, studied for six putative tumor suppressor regions, is limited to chromosome 11.

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Nephrogenic Rests in Wilms Tumor Patients with the Drash Syndrome

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Germline mutations in the Wilms' tumor suppressor gene are associated with abnormal urogenital development in Denys-Drash syndrome

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

WT1 mutations contribute to abnormal genital system development and hereditary Wilms' tumour

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Renal tumors with pseudohermaphroditism and glomerular disease

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Inherited WT1 mutation in Denys-Drash syndrome

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Germline intronic and exonic mutations in the Wilms' tumour gene (WT1) affecting urogenital development

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Infantile nephrotic syndrome due to glomerulonephritis in a male pseudohermaphrodite

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Membranous nephropathy. Long-term follow-up and association with neoplasia

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Somatic deletion and duplication of genes on chromosome 11 in Wilms' tumours.

2 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

Nomenclature of "Drash syndrome".

27 November 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1049926

A syndrome of chronic renal failure and XY gonadal dysgenesis in young phenotypic females without genital ambiguity

27 November 2018

1 reference

12 December 2020

Pseudohermaphroditism, glomerulopathy, and Wilms tumor (Drash syndrome)

1 reference

12 December 2020

Chronic renal failure in an obese adolescent

1 reference

12 December 2020

The association of aniridia, Wilms' tumor, and genital abnormalities

1 reference

12 December 2020

Nephrotic syndrome and nephroblastoma. Report of a case

1 reference

12 December 2020

Pseudohermaphroditism, nephron disorder and wilms' tumor: a unifying concept

1 reference

12 December 2020

[A case of nephroblastoma associated with the nephrotic syndrome]

1 reference

12 December 2020

Dysgenetic male pseudohermaphroditism

1 reference

12 December 2020

Two children with kidney disease. Demonstrated at the Royal College of Physicians of London

1 reference

12 December 2020

The nephropathy associated with male pseudohermaphroditism and Wilms' tumor (Drash syndrome): a distinctive glomerular lesion--report of 10 cases

1 reference

12 December 2020

Focal glomerulosclerosis as a late sequela of Wilms tumor

1 reference

12 December 2020

Partial androgen receptor deficiency and mixed gonadal dysgenesis in Drash syndrome

1 reference

12 December 2020

Nephropathy associated with Wilms' tumor. A case of a 13-year-old girl

1 reference

12 December 2020

Nephropathy-gonadal dysgenesis, type 2: renal failure in three siblings with XY dysgenesis in one

1 reference

12 December 2020

Subclinical Wilms' tumor in incomplete Drash syndrome

1 reference

12 December 2020

1 reference

12 December 2020

Renal failure wit XY gonadal dysgenesis: report of the second case

1 reference

12 December 2020

Association Between Wilms Tumor and Gonadal Dysgenesis

1 reference

12 December 2020

The Drash syndrome: male pseudohermaphroditism, nephritis, and Wilms tumor

1 reference

12 December 2020

Nephroblastoma associated with mesangioproliferative glomerulonephritis

1 reference

12 December 2020

Epidemiological features of Wilms' tumor: results of the National Wilms' Tumor Study

1 reference

12 December 2020

Glomerulonephritis associated with male pseudohermaphroditism and nephroblastoma

1 reference

12 December 2020

Wilms' tumor, nephron disorder and ambiguous genitalia

1 reference

12 December 2020

Loss of alleles at loci on human chromosome 11 during genesis of Wilms' tumour

1 reference

12 December 2020

Pseudohermaphroditism, glomerulopathy and Wilms' tumour (Drash syndrome): a case report

1 reference

12 December 2020

Smallest region of overlap in Wilms tumor deletions uniquely implicates an 11p13 zinc finger gene as the disease locus

1 reference

12 December 2020

A case report of Drash syndrome in a 46,XX female

1 reference

12 December 2020

Intussusception nephrosis and Drash syndrome

1 reference

12 December 2020

Nephropathy gonadal dysgenesis--or incomplete Drash syndrome?

1 reference

12 December 2020

Should bilateral nephrectomy be carried out in all children with diffuse mesangial sclerosis prior to renal transplantation in view of the connection with Drash syndrome and therefore the risk of a Wilms' tumour developing?

1 reference

12 December 2020

A point mutation found in the WT1 gene in a sporadic Wilms' tumor without genitourinary abnormalities is identical with the most frequent point mutation in Denys-Drash syndrome

1 reference

12 December 2020

Distinct molecular origins for Denys-Drash and Frasier syndromes

1 reference

12 December 2020

Constitutional mutations in the WT1 gene in patients with Denys-Drash syndrome

1 reference

12 December 2020

A novel insertional mutation at the third zinc finger coding region of the WT1 gene in Denys-Drash syndrome

1 reference

12 December 2020

Evidence that WT1 mutations in Denys — Drash syndrome patients may act in a dominant-negative fashion

1 reference

12 December 2020

Further evidence that imbalance of WT1 isoforms may be involved in Denys – Drash syndrome

1 reference

12 December 2020

A novel mutation of the WT1 gene (a tumor suppressor gene for Wilms' tumor) in a patient with Denys-Drash syndrome

1 reference

12 December 2020

[Space perception and psychomotricity in infants with motor deficiency]

1 reference

12 December 2020

Bilateral Wilms's tumor in a male pseudohermaphrodite

1 reference

12 December 2020

ASSOCIATION OF NEPHROTIC SYNDROME AND NEPHROBLASTOMA IN SIBLINGS

1 reference

12 December 2020

External genital abnormalities associated with wilms tumor

1 reference

12 December 2020

Wilms tumor and nephrotic syndrome in male pseudohermaphroditism

1 reference

12 December 2020

Wilms' tumor and glomerulopathy

1 reference

12 December 2020