Nephrological Considerations in Children with DSD

Disorders of Sex Development (DSD) refer to a group of disorders of sexual differentiation which lead to internal and/or external genital anomalies manifesting predominantly as ambiguous genitalia. While these disorders are associated with significant morbidity, some of these patients may have congenital anomalies of the kidneys and the urinary tract and may develop recurrent urinary tract infections, steroid-resistant nephrotic syndrome, malignancies, and/or kidney failure. Presence of any of these renal anomalies is associated with significant morbidity in the long term, hence careful evaluation for these nephrological issues is warranted in all children with DSD. Multi-disciplinary care with early referral to the nephrologist upon identification of renal anomalies, hypertension, proteinuria, or kidney dysfunction will improve the overall care offered to these patients.