Cholelithiasis in children with sickle cell disease.

Area of Science:

• Pediatrics
• Gastroenterology
• Hematology

Background:

• Sickle cell disease (SCD) is a genetic blood disorder associated with various complications.
• Gallstone formation is a known, though not fully understood, complication in SCD patients.

Purpose of the Study:

• To determine the prevalence of gallstones in children with homozygous sickle cell disease.
• To evaluate the clinical presentation and outcomes of gallstone disease in this pediatric population.

Main Methods:

• A cohort of 47 children (ages 2-18) with homozygous sickle cell disease was assessed.
• Diagnostic imaging included oral cholecystography and cholecystosonography.
• Symptomatic patients underwent cholecystectomy and were followed post-operatively.

Main Results:

• Gallstones were identified in 8 out of 47 patients (17%) via both imaging modalities.
• These patients frequently experienced recurrent right upper quadrant abdominal pain, sickle cell abdominal crises, and hepatic crises.
• All eight patients who underwent cholecystectomy were found to have gallstones and remained asymptomatic during 7-17 months of follow-up.

Conclusions:

• Gallstones represent a significant complication in children with homozygous sickle cell disease.
• Cholecystectomy effectively manages symptomatic gallstone disease, resolving abdominal pain and crises in this population.