Original Article

Prevalence, Treatment, and Outcomes of Renal Conditions in Pediatric Sickle Cell Disease

Authors: James R. Stallworth, MD, Avnish Tripathi, MD, MPH, Jeanette M. Jerrell, PhD

Abstract

Objectives: Vaso-occlusive events in pediatric sickle cell disease (SCD) may cause various renal complications and lead to renal failure. We describe the renal conditions that develop among young patients with SCD and the factors associated with the prevalence of these nephropathies.


Materials and Methods: Medicaid medical and pharmacy claims for an 11-year period were used to identify 2194 pediatric patients with SCD (HbSS homozygous). Survival analysis identified the most significant predictors of acute kidney injury and chronic renal failure, using demographics, SCD severity and pain medication, comorbid hypertension, hematuria, and proteinuria as the initial covariates.


Results: Prevalence of renal complications in our cohort was found to be relatively low, predominantly hematuria (6.3%) and proteinuria (3.2%). The multivariable analysis indicated that earlier development of acute kidney injury was significantly associated with older age (adjusted hazard ratio [aHR] 1.16, confidence interval [CI] 1.06-1.27), preexisting hypertension (aHR 3.05, CI 1.09-8.60), and preexisting hematuria (aHR 2.87, CI 1.05-7.93). Earlier development of chronic renal failure was significantly associated with older age (aHR 1.20, CI 1.08-1.32), preexisting hematuria (aHR 4.67, CI 1.57-13.94), and preexisting proteinuria (aHR 8.25, CI 2.12-10.38).


Conclusions: These prevalence findings are novel in the US SCD pediatric population. The predictors of nephropathies identified in these children confirm clinical expectations. In addition, they suggest not only that pediatric nephrologists should be consulted earlier in the treatment of patients with SCD who are diagnosed as having comorbid hypertension or who develop hematuria or proteinuria during the course of their SCD treatment but also that both hydroxyurea and angiotensin-converting enzyme inhibitor therapies may be better used in these cases.

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