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

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

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

Risk factors for post-nephrectomy hypotension in pediatric patients.

Authors: Nishi, Kentaro¹, Kamei, Koichi¹ kamei-k@ncchd.go.jp, Ogura, Masao¹, Sato, Mai¹, Ishiwa, Sho^1,2, Shioda, Yoko³, Kiyotani, Chikako³, Matsumoto, Kimikazu³, Nozu, Kandai⁴, Ishikura, Kenji^1,5, Ito, Shuichi^1,6

Source: Pediatric Nephrology. Nov2021, Vol. 36 Issue 11, p3699-3709. 11p. 1 Diagram, 6 Charts.

Subject Terms: *DENYS-Drash syndrome, *ANTIHYPERTENSIVE agents, *HYPERTENSION, *NEPHRECTOMY, *SCIENTIFIC observation, *NEUROBLASTOMA, *NEPHROTIC syndrome, *MULTIVARIATE analysis, *DISEASE incidence, *RETROSPECTIVE studies, *KIDNEY transplantation, *RENIN-angiotensin system, *RISK assessment, *HYPOTENSION, *LOGISTIC regression analysis, *WAGR syndrome, *DISEASE risk factors, *CHILDREN, SURGICAL complication risk factors

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

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

Authors: Lopez-Gonzalez, Mercedes, Ariceta, Gema

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

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

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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

Renal failure from birth-AKI or CKD? Answers.

Authors: Carter, Sean¹, Dixit, Abhijit², Lunn, Andrew², Deorukhkar, Anjum², Christian, Martin² martin.christian@nuh.nhs.uk

Source: Pediatric Nephrology. Dec2016, Vol. 31 Issue 12, p2259-2262. 4p. 1 Chart.

Subject Terms: *NEPHROTIC syndrome diagnosis, *DENYS-Drash syndrome, *CHRONIC kidney failure, *FETAL growth retardation, *CHILDREN, *DIAGNOSIS

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

Genotype-phenotype correlation of WT1 mutation-related nephropathy in Chinese children

Authors: Huanru Chen, Miao Zhang, Jinai Lin, Jieyi Lu, Fazhan Zhong, Fu Zhong, Xia Gao, Xin Liao

Source: Frontiers in Pediatrics, Vol 11 (2023)

Subject Terms: WT1 gene, hereditary nephropathy, end-stage renal disease, steroid-resistant nephrotic syndrome, Denys-Drash syndrome, Frasier syndrome, Pediatrics, RJ1-570

File Description: electronic resource

Relation: https://www.frontiersin.org/articles/10.3389/fped.2023.1192021/full; https://doaj.org/toc/2296-2360

Access URL: https://doaj.org/article/7774663ea7514194b6d4c74b5207373a

A child with isolated nephrotic syndrome and WT1 mutation presenting as a 46, XY phenotypic male.

Authors: Yang, Yonghui¹, Feng, Dongning¹, Huang, Jun¹, Nie, Xiaojing¹, Yu, Zihua zihuayu@vip.sina.com

Source: European Journal of Pediatrics. Jan2013, Vol. 172 Issue 1, p127-129. 3p.

Subject Terms: *DENYS-Drash syndrome, *NEPHROTIC syndrome in children, *GENETIC mutation, *CHRONIC kidney failure in children, *HEART failure in children, *RESPIRATORY insufficiency in children

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

46-XY Denys-Drash Syndrome. Is There a Role for Nephron-sparing Modalities in Management of Renal Masses? A Report of 2 Cases.

Authors: Hodhod, Amr¹ amr.hodhod@mail.mcgill.ca, El-Sherbiny, Mohamed¹

Source: Urology. Jul2018, Vol. 117, p153-155. 3p.

Subject Terms: *DENYS-Drash syndrome, *NEPHRONS, *URINARY organ diseases, *GENITAL abnormalities, *NEPHRECTOMY

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

WT1 Deletion Leading to Severe 46,XY Gonadal Dysgenesis, Wilms Tumor and Gonadoblastoma: Case Report.

Authors: Finken, Martijn J.J.¹ m.finken@vumc.nl, Hendriks, Yvonne M.C.¹, van der Voorn, J. Patrick¹, Veening, Margreet a.¹, Lombardi, M. Paola¹, Rotteveel, Joost¹

Source: Hormone Research in Paediatrics. Apr2015, Vol. 83 Issue 3, p211-216. 6p. 1 Color Photograph, 1 Graph.

Subject Terms: *MISSENSE mutation, *DENYS-Drash syndrome, *RENAL cancer, *GONADS, *CASTRATION

Denys-Drash syndrome, septated vagina and low level of anti-Mullerian hormone in male neonate.

Authors: Hong Jun Lee¹, Jung-Sook Yeom¹, Ji Sook Park¹, Eun Sil Park¹, Ji-Hyun Seo¹, Jae Young Lim¹ pedneu@gnu.ac.kr, Chan-Hoo Park¹, Hyang-Ok Woo¹, Hee-Shang Youn¹

Source: Annals of Pediatric Endocrinology & Metabolism. 2014, Vol. 19 Issue 2, p100-103. 4p.

Subject Terms: *DENYS-Drash syndrome, *ANTI-Mullerian hormone, *NEPHROBLASTOMA, *GENITALIA development, *MALE reproductive organ diseases, *GENETICS

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