Pediatric Hepatobiliary Lithiasis in Homozygous Sickle Cell Disease: Laboratory Insights Into Cholestasis and Hemolysis

TL;DR

A 13-year-old girl with sickle cell disease developed liver and gallbladder issues, showing how hemolysis and cholestasis markers can help diagnose these complications early.

Contribution

Highlights the diagnostic utility of combining hemolysis and cholestasis markers in pediatric sickle cell disease patients.

Findings

Severe anemia and reticulocytosis indicated active hemolysis.

Mixed hyperbilirubinemia and elevated CRP suggested cholestasis and inflammation.

Imaging confirmed microlithiasis without bile duct obstruction.

Abstract

Chronic hemolysis in sickle cell disease (SCD) predisposes patients to hepatobiliary complications, including pigment gallstones. We report a 13-year-old girl with hemoglobin SS SCD who presented with right upper quadrant pain, progressive jaundice, and fever. Laboratory evaluation revealed severe anemia with reticulocytosis, mixed hyperbilirubinemia, elevated lactate dehydrogenase, normal cholestatic enzymes, and marked C-reactive protein elevation. Imaging confirmed microlithiasis without significant bile duct obstruction. Supportive care included transfusion, hydration, antibiotics, and pain control. Elective cholecystectomy was planned after stabilization. This case underscores the diagnostic value of combined hemolysis and cholestasis markers for early recognition of hepatobiliary complications in pediatric patients with SCD.

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