Original articlePseudohermaphroditism, glomerulopathy, and Wilms tumor (Drash syndrome): Frequency in end-stage renal failure*
References (19)
- et al.
A syndrome of pseudohermaphroditism, Wilms' tumor, hypertension and degenerative renal disease
J Pediatr
(1970) - et al.
Pseudohermaphroditism, glomerulonephritis with the nephrotic syndrome and Wilms' tumor in infancy
J Pediatr
(1971) - et al.
A new hemodialysis access device for infants and small children
Kidney Int
(1983) - et al.
Gonadoblastoma associated with pure gonadal dysgenesis in monozygous twins
J Pediatr
(1964) - et al.
XY gonadal dysgenesis with gonadoblastoma discovered after kidney transplantation
Am J Obstet Gynecol
(1977) - et al.
Congenital nephrotic syndrome: Evolution of medical management and results of renal transplantation
J Pediatr
(1984) - et al.
Renal transplantation in patients with bilateral Wilms' tumors
J Pediatr Surg
(1979) - et al.
Association d'un syndrome anatomo-pathologique de pseudohermaphrodisme masculin, d'une tumeur de Wilms, d'une nephropathie parenchymateuse et d'un mosaicisme XX/XY
Arch Fr Pediatr
(1967) - et al.
Two children with kidney disease
Br Med J
(1978)
Cited by (75)
Genetic Testing Proves Crucial in Case of Ambiguous Genitalia and Renal Masses
2019, UrologyCitation Excerpt :The majority of patients have a normal male karyotype, although this is not universal. The relative paucity of cases of Denys-Drash with a female karyotype (XX sex chromosomes) may be secondary to under diagnosis of the syndrome in phenotypic females with nephropathy or as a result of under ascertainment secondary to previous poor survival of children with renal failure in infancy or early childhood.3-5 A similar nephropathy is seen in Frasier syndrome.
46-XY Denys-Drash Syndrome. Is There a Role for Nephron-sparing Modalities in Management of Renal Masses? A Report of 2 Cases
2018, UrologyCitation Excerpt :In the literature, 75% of patients (12/16) had ESRD at a mean age of 13.9 months (1-28).9-12 Simple nephrectomy for ESRD was done at 24.8 months (6-36).9-11 Regarding the ESRD onset, the mean age of our patients was much higher than the age of others in the literature.
Outcomes and Risk Factors for Graft Loss: Lessons Learned from 1,056 Pediatric Kidney Transplants at the University of Minnesota
2017, Journal of the American College of SurgeonsCitation Excerpt :Surgical techniques have evolved over the years. Currently, we perform unilateral or bilateral nephrectomies when indicated (eg reflux demonstrated on a voiding cystourethrogram, uncontrolled hypertension, Drash syndrome in which bilateral gonadectomy is also performed).7 Preoperatively, all recipients < 25 kg undergo dialysis catheter placement, facilitating perioperative fluid management, dialysis, or parenteral nutrition as needed.
Genetic Abnormalities in Glomerular Function
2013, Seldin and Geibisch's The KidneyGenetic Abnormalities in Glomerular Function
2012, Seldin and Giebisch's The Kidney: Physiology and PathophysiologyNon-neoplastic kidney
2009, Modern Surgical Pathology
- *
Supported by Grants AI-10704 and AM-13083 from the National Institutes of Health and by the Viking Children's Fund.
- 1
Dr. Eddy supported by the Medical Research Council of Canada.