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51 results

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Page 1
Gonadal tumor in Frasier syndrome: a review and classification.
Ezaki J, Hashimoto K, Asano T, Kanda S, Akioka Y, Hattori M, Yamamoto T, Shibata N. Ezaki J, et al. Cancer Prev Res (Phila). 2015 Apr;8(4):271-6. doi: 10.1158/1940-6207.CAPR-14-0415. Epub 2015 Jan 26. Cancer Prev Res (Phila). 2015. PMID: 25623218 Review.
Several unusual Frasier syndrome cases have been reported in which male pseudohermaphroditism was absent. ...On the basis of our findings, we propose a new diagnostic algorithm for Frasier syndrome....
Several unusual Frasier syndrome cases have been reported in which male pseudohermaphroditism was absent. ...On the basis of o …
Proteinuria in Frasier syndrome.
Peco-Antić A, Ozaltin F, Parezanović V, Milosevski-Lomić G, Zdravković V. Peco-Antić A, et al. Srp Arh Celok Lek. 2013 Sep-Oct;141(9-10):685-8. doi: 10.2298/sarh1310685p. Srp Arh Celok Lek. 2013. PMID: 24364235 Free article.
INTRODUCTION: Frasier syndrome (FS) is a genetic form of glomerulopathy, which results from mutations in the Wilms'tumour suppressor gene (WT1). ...
INTRODUCTION: Frasier syndrome (FS) is a genetic form of glomerulopathy, which results from mutations in the Wilms'tumour supp …
Chronic renal failure and XY gonadal dysgenesis: "Frasier" syndrome--a commentary on reported cases.
Moorthy AV, Chesney RW, Lubinsky M. Moorthy AV, et al. Am J Med Genet Suppl. 1987;3:297-302. doi: 10.1002/ajmg.1320280535. Am J Med Genet Suppl. 1987. PMID: 3130865 Review.
Gonadoblastoma arising from the streak gonad was noted in five of the six patients. "Frasier" syndrome would be a suitable term to denote this association after Frasier et al, who described two patients in 1964. ...
Gonadoblastoma arising from the streak gonad was noted in five of the six patients. "Frasier" syndrome would be a suitable ter …
Immune-complex glomerulonephritis with a membranoproliferative pattern in Frasier syndrome: a case report and review of the literature.
Matsuoka D, Noda S, Kamiya M, Hidaka Y, Shimojo H, Yamada Y, Miyamoto T, Nozu K, Iijima K, Tsukaguchi H. Matsuoka D, et al. BMC Nephrol. 2020 Aug 24;21(1):362. doi: 10.1186/s12882-020-02007-0. BMC Nephrol. 2020. PMID: 32838737 Free PMC article. Review.
Identification of a de novo Wilms tumor 1 splice donor-site mutation in intron 9 (NM_024426.6:c.1447 + 4C > T) and 46,XY-gonadal dysgenesis led to the diagnosis of Frasier syndrome. CONCLUSIONS: Our findings, together with those of others, point to the importance …
Identification of a de novo Wilms tumor 1 splice donor-site mutation in intron 9 (NM_024426.6:c.1447 + 4C > T) and 46,XY-gonadal dysgenes …
Frasier syndrome, part of the Denys Drash continuum or simply a WT1 gene associated disorder of intersex and nephropathy?
Koziell A, Charmandari E, Hindmarsh PC, Rees L, Scambler P, Brook CG. Koziell A, et al. Clin Endocrinol (Oxf). 2000 Apr;52(4):519-24. doi: 10.1046/j.1365-2265.2000.00980.x. Clin Endocrinol (Oxf). 2000. PMID: 10762296 Review.
More recently WT1 mutations have also been described in a related condition, Frasier syndrome. We report a case where genetic analysis showed a WT1 mutation typically associated with Frasier syndrome: a 1228 + 5 guanine to adenine substitution at the 3 …
More recently WT1 mutations have also been described in a related condition, Frasier syndrome. We report a case where genetic …
A Unique Presentation of XY Gonadal Dysgenesis in Frasier Syndrome due to WT1 Mutation and a Literature Review.
Lavi E, Zighan M, Abu Libdeh A, Klopstock T, Weinberg-Shukron A, Renbaum P, Levy-Lahad E, Zangen D. Lavi E, et al. Pediatr Endocrinol Rev. 2020 Aug;17(4):302-307. doi: 10.17458/per.vol17.2020.lzz.xygonadalfrasiersyndromewt1mutation. Pediatr Endocrinol Rev. 2020. PMID: 32780953
Frasier syndrome (FS), a rare disease caused by inherited or de novo mutation in Wilm's Tumor suppressor gene 1 (WT1), is characterized by slow progressive nephropathy, XY gonadal dysgenesis (XY-DSD), and increased risk for gonadal tumors. ...
Frasier syndrome (FS), a rare disease caused by inherited or de novo mutation in Wilm's Tumor suppressor gene 1 (WT1), is char …
An unusual phenotype of Frasier syndrome due to IVS9 +4C>T mutation in the WT1 gene: predominantly male ambiguous genitalia and absence of gonadal dysgenesis.
Melo KF, Martin RM, Costa EM, Carvalho FM, Jorge AA, Arnhold IJ, Mendonca BB. Melo KF, et al. J Clin Endocrinol Metab. 2002 Jun;87(6):2500-5. doi: 10.1210/jcem.87.6.8521. J Clin Endocrinol Metab. 2002. PMID: 12050205 Review.
Mutations in the WT1 gene were identified in patients with WAGR (Wilms' tumor, aniridria, genitourinary abnormalities, and mental retardation), Denys-Drash syndrome, and Frasier syndrome (FS). Constitutional heterozygous mutations of the WT1 gene, almost all located …
Mutations in the WT1 gene were identified in patients with WAGR (Wilms' tumor, aniridria, genitourinary abnormalities, and mental retardatio …
WT1 Pathogenic Variants are Associated with a Broad Spectrum of Differences in Sex Development Phenotypes and Heterogeneous Progression of Renal Disease.
Ferrari MTM, Watanabe A, da Silva TE, Gomes NL, Batista RL, Nishi MY, de Paula LCP, Costa EC, Costa EMF, Cukier P, Onuchic LF, Mendonca BB, Domenice S. Ferrari MTM, et al. Sex Dev. 2022;16(1):46-54. doi: 10.1159/000517373. Epub 2021 Aug 13. Sex Dev. 2022. PMID: 34392242
Heterozygous germline pathogenic allelic variants of WT1 have been classically associated with Denys-Drash syndrome (DDS) and Frasier syndrome (FS). Usually, exonic pathogenic missense variants in the zinc finger region are the cause of DDS, whereas pathogenic varia …
Heterozygous germline pathogenic allelic variants of WT1 have been classically associated with Denys-Drash syndrome (DDS) and Frasier …
Frasier syndrome in 17-year-old girl--case report.
Matuszczak E, Debek W, Chomczyk I. Matuszczak E, et al. Ginekol Pol. 2011 Jun;82(6):468-70. Ginekol Pol. 2011. PMID: 21853939
The authors present a case of Frasier syndrome in a 17-year-old girl with nephrotic syndrome and male pseudohermaphroditism. ...Early prophylactic resection of dysgenetic gonads, such as was undertaken in this patient, is indicated in children with Frasier …
The authors present a case of Frasier syndrome in a 17-year-old girl with nephrotic syndrome and male pseudohermaphroditism. . …
Clinical heterogeneity in children with gonadal dysgenesis associated with non-mosaic 46,XY karyotype.
Wong YS, Tam YH, Pang KKY, To KF, Chan SSC, Chan KW, Lee KH. Wong YS, et al. J Pediatr Urol. 2017 Oct;13(5):508.e1-508.e6. doi: 10.1016/j.jpurol.2017.03.021. Epub 2017 Apr 10. J Pediatr Urol. 2017. PMID: 28434637
The three PGD patients presented with ambiguous genitalia at birth (n = 2), and isolated hypospadias (n = 1), which was associated with Frasier syndrome. Three patients had germ cell neoplasms: bilateral gonadoblastoma (n = 1), bilateral intratubular germ cell neopl …
The three PGD patients presented with ambiguous genitalia at birth (n = 2), and isolated hypospadias (n = 1), which was associated with F …
51 results