Papers presented at the fall 2020 Pediatric Urologic Oncology Work Group of the Societies of Pediatric Urology meetingNeonatal Serum Electrolyte and Proteinuria Screening on 46,XY Ambiguous Genitalia Patients May Allow Early Diagnosis of Denys-Drash Syndrome: A Case Report.

A term infant with prenatally noted ambiguous genitalia and nonpalpable gonads presented with life-threatening hyponatremia, hypertension, acidosis, and anuric renal failure requiring peritoneal dialysis at age 3 months.Sequencing confirmed 46, XY Denys-Drash syndrome (DDS) due to heterozygous Wilms tumor-1 exon 8 mutation encoding p.His445Arg. Renal US identified bilateral multifocal renal masses at age 8 months. Bilateral retroperitoneal nephrectomies found bilateral nephroblastomatosis without Wilms' tumor avoiding chemotherapy, followed by bilateral laparoscopic orchiopexies. We suggest monthly screening of 46, XY DSD cases for DDS by evaluating for proteinuria and electrolyte disarray starting at diagnosis of DSD to prevent acute life-threatening renal failure presentation.

Role of hypertension in progression of chronic kidney disease in children.

PURPOSE OF REVIEW: Hypertension is an independent risk factor for progression of chronic kidney disease (CKD) in children. Children with early CKD develop hypertension secondary to renal disease. This review aims to highlight recent advances that help us better understand the current role of hypertension in progression of CKD in children. RECENT FINDINGS: There is increasing evidence that children with CKD who have hypertension develop early atherosclerosis and cardiac adaptive changes. Emerging data from pediatric research in CKD show that elevated blood pressure is associated with the presence of abnormal subclinical markers of cardiovascular disease including increased carotid intima-media thickness, pulse wave velocity and left ventricular mass index. There is also some evidence that these early cardiovascular changes are reversible. Twenty-four hour ambulatory blood pressure monitoring (ABPM) is recommended in children with CKD by the American Academy of Pediatrics to diagnose hypertension. SUMMARY: Hypertension is associated with subclinical cardiovascular disease in children with CKD. Early diagnosis of hypertension by ABPM and identification of subclinical cardiovascular changes provide a window for intervention, which may reverse early cardiovascular disease, thereby delaying dialysis and improving cardiovascular morbidity and mortality.