Children presenting with end-stage renal disease of unexplained etiology: implications for disease recurrence after transplantation.

With longer graft and patient survival, recurrent disease is becoming recognized as an increasingly important contributor to long-term graft loss in renal transplant recipients. However, patients may present for the first time in end-stage renal disease (ESRD) leading to uncertainty as to their underlying diagnosis and the risk of recurrence. The purpose of this study was to describe the features of children who presented for the first time in ESRD and to determine the predictive value of investigations in differentiating diseases with and without a recurrence risk. From 7/99 to 11/04, 13 children presented to our center in ESRD. Their median age was 13.3 yr; 77% were male. The majority were hypertensive (77%) and oligoanuric (69%). All had proteinuria (median urine protein to creatinine ratio [Up/c] 7.0), and 92% had microhematuria. Only seven had small kidneys on ultrasound. All children underwent a serologic work-up and six (46%) were biopsied. Of the 13 children, seven had a glomerular disease; in five the diagnosis was made on biopsy, in one on serologic testing and one by family history. Of the remaining six children, three had non-glomerular diseases: obstructive uropathy in one and primary hyperoxaluria type 1 in two, and 3 had an unknown disease. When patients with glomerular diseases were compared with those with non-glomerular diseases, the two predictors for glomerular disease were a lower serum albumin (p = 0.004) and a higher serum bicarbonate level (p = 0.01). Comparing patients with and without a risk of recurrence, there were no differences between the two groups in any of their demographic, clinical, or biochemical parameters by analysis of variance (including serum albumin or proteinuria). In summary, the vast majority of children presenting in ESRD have hematuria and proteinuria, even with non-glomerular diseases. The significant overlap in clinical features between patients with and without a risk of recurrence emphasizes the need for all children presenting in ESRD to be evaluated extensively so that disease recurrence after transplantation can be anticipated or even prevented.