Prophylactic bilateral nephrectomy and preemptive kidney transplantation for Denys–Drash syndrome prior to development of kidney failure.

Authors: Hosokawa, Chika¹, Hotta, Kiyohiko¹ hotta1125@mac.com, Okamoto, Takayuki², Cho, Yuko², Hirose, Takayuki¹, Iwahara, Naoya¹, Manabe, Atsushi², Shinohara, Nobuo¹

Source: Pediatric Nephrology. Mar2024, Vol. 39 Issue 3, p905-909. 5p.

Subject Terms: *GENITAL abnormalities, *DENYS-Drash syndrome, *NEPHRECTOMY, *KIDNEY failure, *KIDNEY transplantation, *SURGERY, *TREATMENT effectiveness, *NEPHROBLASTOMA, *PROTEINURIA, *PREVENTIVE medicine

Genotype-Phenotype Correlations in Denys-Drash Syndrome in Children

Authors: Glénisson, Mathilde ^{1, 2, 24}, Grapin, Mathilde ^{3, 24}, Blanc, Thomas ^{1, 2}, Preka, Evgenia ³, Hogan, Julien ⁴, Aurelle, Manon ^{5, 6, 7}, Roussey, Gwenaëlle ⁸, Mouche, Antoine ⁹, Rousset-Rouviere, Caroline ¹⁰, Novo, Robert ¹¹, Faudeux, Camille ¹², Fila, Marc ¹³, Vrillon, Isabelle ¹⁴, Cloarec, Sylvie ¹⁵, Simon, Thomas ¹⁶, Harambat, Jérôme ¹⁷, Casado, Edouard Martinez ¹⁸, Rod, Julien ¹⁹, Lecoindre, Morgane Carre ²⁰, Heidet, Laurence ^{3, 21}, Boyer, Olivia ^{2, 3, 21}, Garcelon, Nicolas ^{22, 23}, Kachmar, Jessica ²⁰, Dorval, Guillaume ²⁰, Sarnacki, Sabine ^{1, 2, ∗}

Source: In Kidney International Reports April 2025 10(4):1205-1212

WT1-related disorders: more than Denys-Drash syndrome.

Authors: Lopez-Gonzalez, Mercedes¹ mercedes.lopgonz@gmail.com, Ariceta, Gema^1,2

Source: Pediatric Nephrology. Sep2024, Vol. 39 Issue 9, p2601-2609. 9p.

Subject Terms: *GENETICS of disease susceptibility, *SEX differentiation disorders, *STEROIDS, *PROTEINURIA, *RISK assessment, *CONSERVATIVE treatment, *KIDNEY transplantation, *DENYS-Drash syndrome, *GENITALIA tumors, *SEVERITY of illness index, *NEPHROTIC syndrome, *PARADIGMS (Social sciences), *WAGR syndrome, *GENETIC mutation, *NEPHROBLASTOMA, *INDIVIDUALIZED medicine, *PHENOTYPES, *GENOTYPES, *DRUG resistance, *HEALTH care teams, *INTEGRATED health care delivery, *DISEASE risk factors, *SYMPTOMS

Unusual Presentation of Denys-Drash Syndrome in a Girl with Undisclosed Consumption of Biotin.

Authors: Bizzarri, Carla¹, Giannone, Germana Antonella², Gervasoni, Jacopo³, Benedetti, Sabina², Albanese, Federica², Strologo, Luca Dello⁴, Guzzo, Isabella⁴, Mucciolo, Mafalda⁵, Camassei, Francesca Diomedi⁶, Emma, Francesco⁴, Cappa, Marco¹, Porzio, Ottavia^2,7 ottavia.porzio@opbg.net

Source: Journal of Clinical Research in Pediatric Endocrinology. Sep2021, Vol. 13 Issue 3, p347-352. 6p.

Subject Terms: *DENYS-Drash syndrome, *CHRONIC kidney failure, *ULTRASONIC imaging, *NEPHROTIC syndrome, *TESTOSTERONE, *LIQUID chromatography, *MAGNETIC resonance imaging, *BIOTIN, *MASS spectrometry, *HYPERANDROGENISM, *DIAGNOSTIC errors

Genotype-Phenotype Correlation Analysis of WT1 Gene Variants in Denys-Drash Syndrome and Frasier Syndrome

Authors: CAO Yaqing, GUO Baocheng, NIE Min

Source: 罕见病研究, Vol 3, Iss 1, Pp 63-76 (2024)

Subject Terms: wt1 gene, denys-drash syndrome, frasier syndrome, 46, xy disorders of sex development, isolated nephrotic syndrome, wilms tumor, Medicine

File Description: electronic resource

Relation: https://doaj.org/toc/2097-0501

Access URL: https://doaj.org/article/58f5dcd5368f4c4d8fb6a3e7f2d12f3a

Refining the Diagnosis of Congenital Nephrotic Syndrome on Long-term Stored Tissue: c.1097G>A (p.(Arg366His)) WT1 Mutation Causing Denys Drash Syndrome.

Authors: Hillen, Lisa Maria¹, Kamsteeg, Erik Jan², Schoots, Jeroen², Tiebosch, Anton Tom³, Speel, Ernst Jan¹, Roemen, Guido M.¹, Peutz-Koostra, Carine J.¹, Stumpel, Constance T.R.M.⁴

Source: Fetal & Pediatric Pathology. Apr2017, Vol. 36 Issue 2, p112-119. 8p.

Subject Terms: *NEPHROTIC syndrome, *DENYS-Drash syndrome, *NEPHROBLASTOMA, *PERINATAL death, *GENETICS, *THERAPEUTICS

Focal Segmental Membranoproliferative Glomerulonephritis: A Histological Variant of Denys-Drash Syndrome.

Authors: Karmila, A. B.¹ (AUTHOR), Yap, Y. C.² (AUTHOR), Appadurai, M.² (AUTHOR), Oh, L.¹ (AUTHOR), Fazarina, M.³ (AUTHOR), Abd Ghani, F.⁴ (AUTHOR), Ariffin, H.¹ (AUTHOR)

Source: Fetal & Pediatric Pathology. Apr2021, Vol. 40 Issue 2, p113-120. 8p.

Subject Terms: *GLOMERULONEPHRITIS, *KIDNEY failure, *SYNDROMES, *MALE reproductive organs, *NEPHROBLASTOMA, *FOCAL segmental glomerulosclerosis

A patient with Denys-Drash syndrome(DDS) underwent renal allotransplantation with preserved autologous kidney

Authors: Yu Mao, Shi-jian Feng, Xi Jin, Kun-jie Wang

Source: Asian Journal of Surgery, Vol 46, Iss 3, Pp 1313-1314 (2023)

Subject Terms: Denys-Drash syndrome(DDS), Renal allotransplantation, WT1 gene, Surgery, RD1-811

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S1015958422012040; https://doaj.org/toc/1015-9584

Access URL: https://doaj.org/article/6dee023145874fc4ab698cf946dc62c8

A familial WT1 mutation associated with incomplete Denys-Drash syndrome.

Authors: Zhu, Chunhua, Zhao, Fei¹, Zhang, Weizhen¹, Wu, Hongmei¹, Chen, Ying¹, Ding, Guixia¹, Zhang, Aihua, Huang, Songming smhuang@njmu.edu.cn

Source: European Journal of Pediatrics. Oct2013, Vol. 172 Issue 10, p1357-1362. 6p.

Subject Terms: *DENYS-Drash syndrome, *INTERSEXUALITY, *PROTEINURIA, *GENITAL abnormalities, *ARTERIOSCLEROSIS, *INGUINAL hernia, *DIAGNOSIS, *THERAPEUTICS

Atypical severe early-onset nephrotic syndrome: Answers.

Authors: Berthaud, Romain¹ romain.berthaud@aphp.fr, Heidet, Laurence², Oualha, Mehdi³, Brat, Roselyne⁴, Talmud, Déborah⁵, Garaix, Florentine⁶, Rabant, Marion⁷, Frémeaux-Bacchi, Véronique⁸, Antignac, Corinne⁹, Boyer, Olivia¹⁰, Dorval, Guillaume⁹

Source: Pediatric Nephrology. Nov2022, Vol. 37 Issue 11, p2637-2642. 6p. 1 Color Photograph, 1 Chart.

Subject Terms: *HEMOLYTIC-uremic syndrome diagnosis, *DENYS-Drash syndrome, *NEPHROTIC syndrome, *DIFFERENTIAL diagnosis, *SEVERITY of illness index, *RARE diseases

A Zinc Finger Truncation of Murine WT1 Results in the Characteristic Urogenital Abnormalities of Denys-Drash Syndrome

Authors: Patek, Charles E., Little, Melissa H., Fleming, Stewart, Miles, Colin, Charlieu, Jean-Paul, Clarke, Alan R., Miyagawa, Kiyoshi, Christie, Sheila, Doig, Jennifer, Harrison, David J., Porteous, David J., Brookes, Anthony J., Hooper, Martin L., Hastie, Nicholas D.

Source: Proceedings of the National Academy of Sciences of the United States of America, 1999 Mar . 96(6), 2931-2936.

Access URL: https://www.jstor.org/stable/47466

Single-cell transcriptomes of kidneys in a 6-month-old boy with Denys-Drash syndrome reveal stromal cell heterogeneity in the tumor microenvironment.

Authors: Li, Tao¹ (AUTHOR), Zhou, Jiangfeng¹ (AUTHOR), Wu, Haiyan² (AUTHOR), Gao, Xiucheng³ (AUTHOR), Shen, Qiyang⁴ (AUTHOR), Cheng, Rui⁵ (AUTHOR) chengrui350@163.com, Zhang, Mingshun⁶ (AUTHOR) mingshunzhang@njmu.edu.cn

Source: Clinical Kidney Journal. Jan2024, Vol. 17 Issue 1, p1-14. 14p.

Subject Terms: *STROMAL cells, *TUMOR microenvironment, *NEPHROBLASTOMA, *CELL anatomy, *MACROPHAGES

Case Report: Denys–Drash Syndrome With WT1 Causative Variant Presenting as Atypical Hemolytic Uremic Syndrome

Authors: Cheng Cheng, Lizhi Chen, Sijia Wen, Zhilang Lin, Xiaoyun Jiang

Source: Frontiers in Pediatrics, Vol 8 (2020)

Subject Terms: denys-drash syndrome (DDS), pseudohermaphroditism, atypical hemolytic uremic syndrome (aHUS), case report [publication type], wilms tumor 1 gene (WT1), Pediatrics, RJ1-570

File Description: electronic resource

Relation: https://www.frontiersin.org/articles/10.3389/fped.2020.605889/full; https://doaj.org/toc/2296-2360

Access URL: https://doaj.org/article/a619143d2c3f43b7bf7871f8b5a6e571

Hemolytic uremic syndrome as the presenting manifestation of WT1 mutation and Denys-Drash syndrome: a case report

Authors: Joseph L. Alge, Scott E. Wenderfer, John Hicks, Mir Reza Bekheirnia, Deborah A. Schady, Jamey S. Kain, Michael C. Braun

Source: BMC Nephrology, Vol 18, Iss 1, Pp 1-6 (2017)

Subject Terms: Hemolytic uremic syndrome, HUS, Wilms tumor 1, WT1, Denys-Drash syndrome, Diffuse mesangial sclerosis, Diseases of the genitourinary system. Urology, RC870-923

File Description: electronic resource

Relation: http://link.springer.com/article/10.1186/s12882-017-0643-1; https://doaj.org/toc/1471-2369

Access URL: https://doaj.org/article/c71b081b283d41ce807f5937d08126e3

P067: Identification of novel variant in WT1 gene in African-American girl with Denys-Drash syndrome

Authors: Jasmine Lee, Meghan McGath, Berrin Monteleone

Source: Genetics in Medicine Open, Vol 1, Iss 1, Pp 100086- (2023)

Subject Terms: Genetics, QH426-470, Medicine

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S2949774423000869; https://doaj.org/toc/2949-7744

Access URL: https://doaj.org/article/1d17bbc941c1415b96b5a06c9cfbf8b4

Denys-Drash Syndrome: a case report

Authors: Ahmed AbuAlreesh, Zuhair A Rahbeeni, Rayah Asiri

Source: Journal of Biochemical and Clinical Genetics, Vol 1, Iss 2, Pp 84-86 (2018)

Subject Terms: denys-drash syndrome, dds, wt1 gene, wilm's tumor, nephropathy, diffuse mesangial sclerosis, dms, undescended testis, Genetics, QH426-470

File Description: electronic resource

Relation: http://www.ejmanager.com/fulltextpdf.php?mno=17515; https://doaj.org/toc/1658-807X

Access URL: https://doaj.org/article/fbdb7793701f43b389a97544af65cf68

Generation of a human iPSC line (MPIi008-A) from a patient with Denys-Drash syndrome

Authors: Markus C. Doeser, Julia Krygin, Albrecht Röpke, Dong Han, Roland Wedlich-Söldner, Hans R. Schöler, Hermann Pavenstädt, Kee-Pyo Kim

Source: Stem Cell Research, Vol 62, Iss , Pp 102826- (2022)

Subject Terms: Biology (General), QH301-705.5

File Description: electronic resource

Relation: http://www.sciencedirect.com/science/article/pii/S1873506122001751; https://doaj.org/toc/1873-5061

Access URL: https://doaj.org/article/d2bab3d16bad401b90b206bcc9b0d4ef

Outcome of renal transplantation for Wilms’ tumor and Denys–Drash syndrome: A report of the North American Pediatric Renal Transplant Cooperative Study.

Authors: Kist-van Holthe, Joana E.^1,2, Ho, Ping L.³, Stablein, Donald³, Harmon, William E.¹, Baum, Michelle A.¹ michelle.baum@childrens.harvard.edu

Source: Pediatric Transplantation. Jun2005, Vol. 9 Issue 3, p305-310. 6p.

Subject Terms: *KIDNEY transplantation, *TRANSPLANTATION of organs, tissues, etc. in children, *PEDIATRIC surgery, *KIDNEY diseases, *CHILDHOOD cancer, *TUMORS in children

Bilateral Wilms' tumor in a child with Denys-Drash syndrome: novel frameshift variant disrupts the WT1 nuclear location signaling region.

Authors: Guaragna, Mara Sanches, Ledesma, Felipe Lourenço, Manzano, Victoria Zavanelli, Maciel-Guerra, Andréa Trevas, Guerra-Júnior, Gil, Silva, Marcelo Milone, Luiz de Brito, Pedro, Palandi de Mello, Maricilda

Source: Journal of Pediatric Endocrinology & Metabolism; Jun2022, Vol. 35 Issue 6, p837-843, 7p

Long-term kidney function in children with Wilms tumour and constitutional WT1 pathogenic variant.

Authors: Falcone, Maria Pia^1,2, Pritchard-Jones, Kathryn¹, Brok, Jesper^1,3, Mifsud, William¹, Williams, Richard D.¹, Nakata, Kayo¹, Tugnait, Suzanne¹, Al-Saadi, Reem^1,4, Side, Lucy⁵, Anderson, John¹, Duncan, Catriona¹, Marks, Stephen D.^1,6, Bockenhauer, Detlef^6,7, Chowdhury, Tanzina¹ Tanzina.Chowdhury@gosh.nhs.uk

Source: Pediatric Nephrology. Apr2022, Vol. 37 Issue 4, p821-832. 12p. 6 Charts, 1 Graph.

Subject Terms: *DENYS-Drash syndrome, *BLOOD pressure, *GENETIC mutation, *NEPHRECTOMY, *CONFIDENCE intervals, *TIME, *RETROSPECTIVE studies, *ACE inhibitors, *NEPHROBLASTOMA, *KIDNEY tumors, *GENOTYPES, *SURVIVAL analysis (Biometry), *DESCRIPTIVE statistics, *PROTEINURIA, *HEMODIALYSIS, *DISEASE management, *PHENOTYPES, *ALBUMINURIA, *CHILDREN