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Year Number of Results
1990 1
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1997 1
2000 2
2001 1
2002 2
2003 1
2004 2
2007 1
2009 1
2010 1
2011 3
2012 2
2013 2
2017 1
2018 2
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2020 3
2021 2
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2023 2
2024 1

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28 results

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Page 1
Management of bilateral Wilms tumours.
Millar AJ, Cox S, Davidson A. Millar AJ, et al. Pediatr Surg Int. 2017 Apr;33(4):461-469. doi: 10.1007/s00383-016-4047-2. Epub 2017 Jan 4. Pediatr Surg Int. 2017. PMID: 28054101 Review.
At least 10% of synchronous BWTs have unfavourable histology, and up to 22% are associated with genitourinary abnormalities, aniridia, WAGR syndrome, Denys-Drash Syndrome, hemihypertrophy, or one of the other overgrowth syndromes. ...There is potential for a reduced …
At least 10% of synchronous BWTs have unfavourable histology, and up to 22% are associated with genitourinary abnormalities, aniridia, WAGR …
Challenges to congenital genetic disorders with "RNA-targeting" chemical compounds.
Ogawa Y, Hagiwara M. Ogawa Y, et al. Pharmacol Ther. 2012 Jun;134(3):298-305. doi: 10.1016/j.pharmthera.2012.02.001. Epub 2012 Feb 9. Pharmacol Ther. 2012. PMID: 22342810 Free article. Review.
Based on this simple idea, we have looked for chemical compounds which can be applicable for congenital diseases and found that protein kinase inhibitors such as INDY, TG003, and SRPIN340 are promising as clinical drugs for DS, DMD, and DDS, respectively....
Based on this simple idea, we have looked for chemical compounds which can be applicable for congenital diseases and found that protein kina …
Unusual Presentation of Denys-Drash Syndrome in a Girl with Undisclosed Consumption of Biotin.
Bizzarri C, Antonella Giannone G, Gervasoni J, Benedetti S, Albanese F, Dello Strologo L, Guzzo I, Mucciolo M, Diomedi Camassei F, Emma F, Cappa M, Porzio O. Bizzarri C, et al. J Clin Res Pediatr Endocrinol. 2021 Aug 23;13(3):347-352. doi: 10.4274/jcrpe.galenos.2020.2020.0064. Epub 2020 Aug 25. J Clin Res Pediatr Endocrinol. 2021. PMID: 32840097 Free PMC article.
We describe a 46,XX girl with Denys-Drash syndrome, showing both kidney disease and genital abnormalities, in whom a misdiagnosis of hyperandrogenism was made. ...Similar testosterone levels were detected on repeat immunoassay two weeks after biotin uptake interrupt …
We describe a 46,XX girl with Denys-Drash syndrome, showing both kidney disease and genital abnormalities, in whom a misdiagno …
A cross-sectional nationwide survey of congenital and infantile nephrotic syndrome in Japan.
Hamasaki Y, Hamada R, Muramatsu M, Matsumoto S, Aya K, Ishikura K, Kaneko T, Iijima K. Hamasaki Y, et al. BMC Nephrol. 2020 Aug 24;21(1):363. doi: 10.1186/s12882-020-02010-5. BMC Nephrol. 2020. PMID: 32838745 Free PMC article.
Among those with non-Finnish-type disease, 26 had no syndrome and 24 had a syndrome, of which the most frequent was Denys-Drash syndrome (70.8%). Patients with non-Finnish-type disease with syndrome showed the earliest progression to end-stage kidney disease compare …
Among those with non-Finnish-type disease, 26 had no syndrome and 24 had a syndrome, of which the most frequent was Denys-Drash sy …
Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms' tumor.
Beckwith JB, Kiviat NB, Bonadio JF. Beckwith JB, et al. Pediatr Pathol. 1990;10(1-2):1-36. doi: 10.3109/15513819009067094. Pediatr Pathol. 1990. PMID: 2156243 Review.
PLNRs were strongly associated with synchronous bilateral WTs, and ILNRs with metachronous contralateral WTs. ILNRs were associated with aniridia and Drash syndrome, whereas PLNRs were more commonly found with hemihypertrophy and/or Beckwith-Wiedemann syndrome. The …
PLNRs were strongly associated with synchronous bilateral WTs, and ILNRs with metachronous contralateral WTs. ILNRs were associated with ani …
Risk factors for post-nephrectomy hypotension in pediatric patients.
Nishi K, Kamei K, Ogura M, Sato M, Ishiwa S, Shioda Y, Kiyotani C, Matsumoto K, Nozu K, Ishikura K, Ito S. Nishi K, et al. Pediatr Nephrol. 2021 Nov;36(11):3699-3709. doi: 10.1007/s00467-021-05115-7. Epub 2021 May 14. Pediatr Nephrol. 2021. PMID: 33988732
METHODS: This retrospective observational study included the clinical data of children who underwent nephrectomy in our center between 2002 and 2020. ...The diagnoses were isolated Wilms tumor, neuroblastoma, congenital nephrotic syndrome, Denys-Drash syndrome …
METHODS: This retrospective observational study included the clinical data of children who underwent nephrectomy in our center betwee …
Results of Treatment for Patients With Multicentric or Bilaterally Predisposed Unilateral Wilms Tumor (AREN0534): A report from the Children's Oncology Group.
Ehrlich PF, Chi YY, Chintagumpala MM, Hoffer FA, Perlman EJ, Kalapurakal JA, Tornwall B, Warwick A, Shamberger RC, Khanna G, Hamilton TE, Gow KW, Paulino AC, Gratias EJ, Mullen EA, Geller JI, Grundy PE, Fernandez CV, Dome JS. Ehrlich PF, et al. Cancer. 2020 Aug 1;126(15):3516-3525. doi: 10.1002/cncr.32958. Epub 2020 May 27. Cancer. 2020. PMID: 32459384 Free PMC article. Clinical Trial.
The underlying diagnosis included Beckwith-Wiedemann syndrome in 9 patients, hemihypertrophy in 9 patients, multicentric tumors in 10 patients, WAGR syndrome in 2 patients, a solitary kidney in 2 patients, Denys-Drash syndrome in 1 patient, and Simpson-Golabi-Behmel …
The underlying diagnosis included Beckwith-Wiedemann syndrome in 9 patients, hemihypertrophy in 9 patients, multicentric tumors in 10 patien …
Pituitary-gonadal axis in male undermasculinisation.
Ng KL, Ahmed SF, Hughes IA. Ng KL, et al. Arch Dis Child. 2000 Jan;82(1):54-8. doi: 10.1136/adc.82.1.54. Arch Dis Child. 2000. PMID: 10630914 Free PMC article.
METHODS: A retrospective study of a register of cases of male undermasculinisation (20 with abnormal testes, eight with 5alpha-reductase deficiency, three with testosterone biosynthetic defects, seven with Drash syndrome, and 210 undiagnosed). RESULTS: A human chori …
METHODS: A retrospective study of a register of cases of male undermasculinisation (20 with abnormal testes, eight with 5alpha-reductase def …
The podocyte as a target: cyclosporin A in the management of the nephrotic syndrome caused by WT1 mutations.
Stefanidis CJ, Querfeld U. Stefanidis CJ, et al. Eur J Pediatr. 2011 Nov;170(11):1377-83. doi: 10.1007/s00431-011-1397-6. Epub 2011 Feb 8. Eur J Pediatr. 2011. PMID: 21298518 Review.
The patients of these studies had different types of WT1 mutations and varying clinical presentations. However, all of them were of young age and the favourable response to CsA might be the result of treatment at an early stage of the disease. ...
The patients of these studies had different types of WT1 mutations and varying clinical presentations. However, all of them were of y …
Prophylactic bilateral nephrectomy and preemptive kidney transplantation for Denys-Drash syndrome prior to development of kidney failure.
Hosokawa C, Hotta K, Okamoto T, Cho Y, Hirose T, Iwahara N, Manabe A, Shinohara N. Hosokawa C, et al. Pediatr Nephrol. 2024 Mar;39(3):905-909. doi: 10.1007/s00467-023-06113-7. Epub 2023 Aug 12. Pediatr Nephrol. 2024. PMID: 37572117
BACKGROUND : Nephropathy in Denys-Drash syndrome (DDS) develops within a few months of birth, often progressing to kidney failure. ...
BACKGROUND : Nephropathy in Denys-Drash syndrome (DDS) develops within a few months of birth, often progressing to kidney fail …
28 results