The frequency of cholelithiasis in the study population was 45%. One-third of the patients were diagnosed before 10 years of age. Patients with the SS homozygous or Sb heterozygous phenotype were at a higher risk for the development of cholelithiasis than patients with sickle cell disease. About 50% of patients with gallstones were asymptomatic, the most of them did not undergo surgery and did not present complications during a 7-year follow-up period. Cholecystectomy must be considered in symptomatic patients. In asymptomatic patients, conservative management seems to be the better choice.

Background-Sickle cell disease (SCD) causes chronic and recurrent hemolysis which is a recognized risk factor for cholelithiasis. This complication occurs in 50% of adults with SCD. Surgery is the consensual therapy in symptomatic patients, but the surgical approach is still controversial in asymptomatic individuals.Aims-To describe the frequency and the outcome of children with SCD complicated with gallstones followed up in a tertiary pediatric hematology center. Patients and methods-In a retrospective and descriptive study, 225 charts were reviewed and data regarding patient's outcome were recorded.Results-The prevalence of cholelithiasis was 44,9% and half of the patients had no symptom. The mean age diagnosis of cholelithiasis and surgical treatment were 12.5 years (SD= 5) and 14 years (SD= 5.4), respectively. The prevalence of cholelithiasis was higher in patients with SS and Sβ thalassemia when compared to patients with SC disease (χ 2 = 0.001). In 50% of symptomatic patients, recurrent abdominal pain was the single or predominant symptom. Thirty-nine of 44 operated patients reported symptom relieve after surgical procedure. Asymptomatic non-operated individuals were followed up for 7 years (SD = 4.8), and none of them presented complications related to cholelithiasis during this period.Conclusions-Cholelithiasis frequency in studied children was 44,9%. One-third of patients were diagnosed before 10 years of age. Patients with the SS or Sß phenotype showed higher frequency of cholelithiasis. About 50% of patients with gallstones had no symptom and most of them did not undergo surgery and did not present complications during a 7-year follow-up period.