II. Epidemiology
-
Cholelithiasis or biliary sludge Prevalence
- Age <18 years: 30 to 50%
- Adults: 75 to 85%
III. Pathophysiology
- Intrahepatic cholestasis
- RBC sickling within liver sinusoids results in hepatic ischemia and Unconjugated Hyperbilirubinemia accumulation
- Progressive Intrahepatic cholestasis
- Life threatening condition associated with a high mortality rate
- Presents with RUQ Abdominal Pain, fever, Vomiting and Jaundice
- Risk of Hemorrhage (due to Coagulopathy), encephalopathy and Renal Failure
- Contrast with benign cholestasis (benign Hyperbilirubinemia)
-
Cholelithiasis or biliary sludge
- Hemolysis episodes generate higher Red Blood Cell turn-over, resulting in higher Unconjugated Bilirubin
- Increased Incidence of Gallstones, sludging, Cholecystitis, Cholelithiasis, Choledocholithiasis, Gallstone Pancreatitis
- Asymptomatic Cholelithiasis may be observed as with non-Sickle Cell Anemia patients
- Onset in childhood (by age 4 years) and affects up to 75 to 85% of adults
- Acute hepatic sequestration
- Abnormal Red Blood Cells may be sequestered within the liver
IV. Symptoms
- Diffuse Abdominal Pain and tenderness
V. Signs
- Hepatomegaly (two thirds of SCA patients)
VI. Differential Diagnosis: Abdominal Pain in Sickle Cell Anemia
- Biliary disorders (see above)
- Intrahepatic cholestasis
- Cholelithiasis
- Choledocholithiasis
- Ascending Cholangitis
- Acute Pancreatitis
- Vaso-occlusive crisis
- Splenic Sequestration (life threatening)
- Intrahepatic sickling (first year of life)
- Consider common other intraabdominal conditions
VII. Labs
- Elevated Liver Function Tests
- Coagulation studies (in Sickle Cell Intrahepatic Cholestasis)
VIII. Management
-
Acute Cholecystitis or Choledocholithiasis
- Emergent Antibiotics and surgical management
- Consult hematology regarding preoperative transfusion
IX. References
- Dwyer, Kleinmann, Goswami and Lopez (2025) Crit Dec Emerg Med 39(1): 26-35
