Abstract
Sickle cell disease (SCD), caused by a mutation in the β-globin gene, leads to acute hemolytic crises and chronic anemia, necessitating frequent blood transfusions. Renal involvement begins early in life and is multifactorial. Chronic kidney disease (CKD) develops in about 30% of SCD adults and is a major cause of death. This study aims to identify markers of glomerular and tubular damage, along with potential risk factors, in a pediatric SCD cohort. We conducted monthly blood and urine tests over 24 months on pediatric patients with SCD. Glomerular hyperfiltration was defined as an estimated glomerular filtration rate (eGFR) > 140 ml/min/1.73 m², while CKD was defined as either eGFR < 60 ml/min/1.73 m² or the presence of specific structural or functional abnormalities. Proteinuria was measured by urinary albumin–creatinine ratio (uACR) or urinary protein-creatinine ratio (UPCR). The study enrolled 46 pediatric SCD patients with a median age of 12 years (range 3–18 years). Glomerular hyperfiltration was observed in 82.6% of patients. uACR and elevated UPCR were found in 15.2% and 32.6% of patients, respectively. A high transfusion requirement correlated with glomerular hyperfiltration and UPCR, irrespective of age. A significant portion of the cohort exhibited glomerular hyperfiltration and proteinuria, particularly those with high transfusion needs. These changes were observed independently of age, more as a consequence of disease progression over time, suggesting the need for early evaluation and potential treatment to prevent disease progression even at an early stage.