Iothalamate versus estimated GFR in a Hispanic-dominant pediatric renal transplant population.

BACKGROUND: Accurate knowledge of glomerular filtration rate (GFR) is essential to the practice of nephrology. Routine surveillance of GFR is most commonly executed using estimated GFR (eGFR) calculations, most often from serum creatinine measurements. However, cystatin C-based equations have demonstrated earlier sensitivity to decline in renal function. The literature regarding eGFR from cystatin C has few references that include transplant recipients. Additionally, for most of the published eGFR equations, patients of Hispanic ethnicity have not been enrolled in sufficient numbers. METHODS: The applicability of several eGFR equations to the pediatric kidney transplant population at our center were compared in the context of determining whether Hispanic ethnicity was associated with equation performance. RESULTS: Updated Schwartz, CKiD, and Zappitelli eGFR estimation equations demonstrated the highest correlations. CONCLUSIONS: The authors recommend further prospective investigations to validate and identify factors contributing to these findings.

Recent advances in congenital nephrotic syndrome.

PURPOSE OF REVIEW: This review provides a concise update of the most recent literature related to the diagnosis and care of patients with congenital nephrotic syndrome. This topic is of particular interest in light of the rapidly growing body of literature regarding mutations of proteins such as nephrin and podocin that are expressed at or near the podocyte slit diaphragm. RECENT FINDINGS: The phenotypic variance of patients with congenital nephrotic syndrome with nephrin and podocin mutations resulting from triallelic mutations represents an important advance in our understanding of the effect of multiple genetic mutations on clinical disease expression. Clinically, the management of patients with unilateral nephrectomy, rather than the classic bilateral nephrectomy, represents an efficacious alternative management strategy and may impart better chances of graft survival by allowing later transplantation. Identification of a subset of patients with congenital nephrotic syndrome at increased risk of recurrence who also have antinephrin antibodies may enhance our understanding of recurrent disease in congenital nephrotic syndrome after transplantation. SUMMARY: Exciting recent findings in the genotypic/phenotypic correlations of patients with congenital nephrotic syndrome may not only modify our understanding of this disease but may also help to revolutionize our understanding of human genetics. Promising outcomes with unilateral nephrectomy in patients with congenital nephrotic syndrome have permitted transplantation to be delayed and may potentially decrease the risk of complications. New findings regarding recurrence of nephrotic syndrome in patients with congenital nephrotic syndrome after transplantation may lead to improved survival in future renal transplantations.