Renal Complications of Sickle Cell Syndrome in Southern Iran

In spite of many reports on sickle cell disease, to our knowledge frequencies of nephrologic complications are not well defined especially in sickle thalassemia. The aim of this study was to assess the nephrologic complications of sickle cell syndrome.
This is a cross sectional study, including all of the patients with sickle cell syndromes (97 cases) in hematology-oncology clinics of Shiraz University of Medical Sciences. A complete history taken and physical examination was performed. Blood and urine samples were sent for different tests including micro albuminuria and Cystatin C to measure glomerular filtration rate.
Ninety seven patients with mean age of 8.5 ± 3.4 years (55.4% males) were divided into three groups; 30 cases with sickle cell disease, 31 patients with sickle cell trait, and 36 cases with sickle thalassemia. Enuresis was present in 20 (22.9%) patients > 5 years. Significant proteinuria was not observed, but micro albuminuria was found in 14 (14.4%) patients. GFR was in normal range when calculated with creatinine clearance, but glomerular hyper filtration was present in 5.2% by Cystatin C formula. Mean serum creatinine was in low normal range (0.5 ± 0.1) but mean serum Cystatin C (0.9 ± 0.1) was higher than the normal range especially in Sβ patients (P = 0.023).
In contrast to other reports, at least in the first two decades of life significant renal complications including renal failure and proteinuria were unusual in our patients with sickle cell syndrome.