End Stage Renal Disease Diagnosed after Tonsillo-Adenoidectomy: A Case Report.

Topic: Pediatric Nephrology. Background: We report a 7 year old girl that was previously healthy, with the exception of recurrent pharyngitis, who presented with weakness, respiratory distress, and edema approximately 2 weeks after tonsilloadenoidectomy. Methods: In the Emergency room, she appeared dehydrated; but, after fluid resuscitation, developed pulmonary edema requiring intubation. Initial labs included anemia (hgb 6 g/dl), high anion gap metabolic acidosis (CO2 11 mmol/l), elevated bun (132 mg/dl), elevated serum creatinine (15.5 mg/dl), severe hypocalcemia (<5 mg/dl), hypoalbuminemia (alb 1.56 g/dl) and nephrotic range proteinuria. Results: She required acute to chronic hemodialysis. Echocardiogram showed left ventricular hypertrophy and mild left ventricular systolic function. Kidney biopsy showed diffuse global glomerulosclerosis with interstitial fibrosis and tubular atrophy consistent with end stage kidney disease. A preliminary microarray to determine possible genetic etiology for the nephrotic syndrome showed an XY karyotype. Pelvic US showed no ovaries or uterus. MRI without contrast shows rudimentary uterus. The rest of the genetic studies are in process but there is a high suspicion for Frasier's/Denys-Drash Syndrome. Conclusions: This rare case is presented to highlight the importance of recognizing the early clinical manifestations of patients with chronic kidney disease and to consider sending the karyotype in a patient with early onset nephrotic syndrome and renal failure. [ABSTRACT FROM AUTHOR]