An Appraisal of Kidney Dysfunction and Its Risk Factors in Patients with Sickle Cell Disease

Abstract

Sickle cell disease (SCD), a genetically inherited disease of blacks, often presents with disabling acute complications which can occasionally be fatal. Its renal manifestations are increasingly being recognized as affected patients now survive to middle and rarely old age. We set out to determine the magnitude of kidney dysfunction in our SCD patient population and evaluate its predictive factors. We reviewed the available case records of SCD patients managed in our hospital. Information on socio-demographic, clinical and laboratory data were retrieved and collated. A total of 374 (99.46%) were reviewed with complete data; the median age was 23 years (range 7–62), while median age at diagnosis of SCD was 4 years (range 0.25–31). 235 patients (68.2%) had no kidney disease while the remaining 139 (37.2%) had proteinuria, hematuria or reduced glomerular filtration rate (GFR) <60 ml/min. The age of patients was a significant predictor of kidney disease (p = 0.002) and correlated with the level of serum creatinine (r = 0.188, p < 0.001), GFR (r = 0.245, p < 0.0001) and the degree of proteinuria (r = 0.174, p = 0.006). Patients with kidney disease had a significantly higher number of crises/hospitalizations (p < 0.001). Seven patients died in all and 4 (57%) of them had end-stage renal disease. We concluded that kidney disease is a common complication of SCD and significantly contributes to mortality. The age of the patients, duration of SCD and frequency of crises/hospitalizations are strong predictors of development of kidney disease.

Renal Tubular Dysfunction in Sickle Cell Disease

“…In the present study significant microalbuminuria, which is an early predictor of glomerular lesion, was observed in 27% of cases, which is lower than the described in previous studies [18,19,20,21,22,23,24]. This difference could be attributable to differences in genetics, includuing different haplotypes.…”

“…Urine endothelin-1 was also found to be associated with microalbuminuria in SCD patients, as well as with urinary concentration defect [26]. Albuminuria has been described in 16 to 60% of adults with SDC [5,18,20,21,22,27], and has significant correlation with age [11,19,24]. No patient in our cohort developed nephrotic range proteinuria, which is really rare in SCD [5].…”

Degrees of Kidney Disease in Nigerian Adults with Sickle-Cell Disease

“…Previous reports on Nigerian SCD patients identified age as a significant predictor of kidney disease and as correlating with GFR [12]. Indeed, end-organ damage in this group of patients becomes more pronounced with advancing age [24].…”

“…The burden of SCN has been carefully studied in the works by Aleem [11] and Arogundade et al [12], where up to 22.5% of SCD patients studied in Saudi Arabia and Nigeria, respectively, had subnormal creatinine clearance. Creatinine clearance may thus provide a means to determine the severity of renal pathology and its progression, and therefore provides insight into the natural history of SCN.…”

Kidney Transplantation From a Donor With Sickle Cell Disease

“…The extent to which kidneys from donors with SCD can be safely transplanted with acceptable outcomes is unknown; however, this report should provide support for the careful expansion of kidneys from donors with SCD without evidence of renal dysfunction and with normal tissue architecture on preimplantation biopsies. Predictors of the development of renal disease in SCD patients, such as age, duration of disease, frequency of crises or hospitalizations, hypertension, proteinuria, and increasingly severe anemia, should be considered prior to biopsy (10,11). The sickle cell status of the donor should be communicated to the recipient, and perhaps initially, kidneys from patients with SCD may be reserved for recipients with a higher risk of waitlist mortality and those with shorter expected posttransplant survival until we learn more about the natural history of these transplanted organs.…”