**5.3. Treatment of anemia**

The use of multiple blood transfusions demonstrated to restore the urinary concentrating ability in children with SCD [152, 153]. One study of 120 children with sickle hemoglobinopathies found that chronic red blood cell transfusions before the age of 9 years was protective against the onset of microalbuminuria [154]. Blood transfusion receives HbS, prevent direct sickling in the kidney and vaso-occulsion, reducing glomerular and tubular ischemia damage to the kidney. However, the benefits of transfusion therapy must be balanced against risks including infections, iron overload, acute or delayed hemolytic transfusion reactions [48, 155–158].

### **5.4. Treatment of end-stage renal disease**

Hemodialysis is reportedly the leading form of renal replacement therapy for SCD-ESRD patients, as well as peritoneal dialysis and kidney transplantation. Mortality in SCD patients is approximately 26% during the first year of therapy for ESRD, nearly threefold higher than in ESRD patients without SCD. However, SCD patients who received pre-dialysis nephrology care had a lower death rate than those who did not receive such care [159].

Kidney transplantation may offer survival advantage over dialysis in ESRD. As in the general population, allograft survival for patients with ESRD is greater in those with a living donor than in those with a deceased donor. The post-transplantation one-year graft survival exceeds 60–80% [160]. Complications specific to the SCD population include higher infection risk due to autosplenectomy and precipitation of sickle cell crises with anemia correction following a successful transplant. Kidney transplant may be also complicated by allograft venous thrombosis, deep vein thrombosis, and vaso-occlusive crises [63, 161, 162]. Suggested maneuvers to decrease the incidence of post-transplant complications in these patients include [63, 163] preoperative blood transfusions to decrease hemoglobin S levels, preoperative oxygen supplementation with 40% oxygen, pretransplantation warming of the kidney allograft using 37<sup>o</sup> C saline, intraoperative and postoperative dopamine infusion at 4 μg/kg/min stem cell transplantation remain as the only curative treatment with good result and survival rates around 90% in 4 years [164, 165].
