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Journal of Case Reports
Denys Drash Syndrome: A Rare Presentation
Sandeep Gupta, Arun Kumar Maurya, Kumar Rajiv Ranjan, Dilip Kumar Pal
Department of Urology, Institute of Post-Graduate Medical Education and Research and S.S.K.M. Hospital, Kolkata-700020, West-Bengal, India.
Corresponding Author:
Dr. Sandeep Gupta
Email: urologyipgmer@gmail.com
Received: 27-APR-2016 Accepted: 04-JUL-2016 Published Online: 15-SEP-2016
DOI: http://dx.doi.org/10.17659/01.2016.0105
A 30 month old phenotypically female child presented to our outdoor with painless progressive lump in left upper abdomen with ambiguous genitalia. Ultrasound and CT findings were suggestive of malignant left renal mass with right normal and left streak gonad in abdomen. Karyotyping shows 46XY. Patient had persistent proteinuria and hypertension. Histopathology report of left renal mass was biphasic Wilms tumor with favorable histology and mesangial sclerosis in the non-malignant part. Patient had a classic triad of Denys Drash syndrome. In most of the reported cases, patient presented with end stage renal disease but in our case overt feature of nephrotic syndrome or end stage renal disease were not associated, which is a rare phenomenon.
Keywords : Denys-Drash syndrome, Gonads, Hypertension, Karyotyping, Kidney Failure, Nephrotic Syndrome.
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