Abstract

Objective: This study aimed to determine how common hepatobiliary complications were in children with sickle cell disease (SCD).
Methods: This was a retrospective descriptive study conducted at a single tertiary health care center, King Saud Medical City, Pediatric Hospital, Riyadh, from April 2023 to April 2024. Patient recruitment was done by reviewing the medical records. All pediatric patient cases under 14 years, with SCD in both genders were included without specifying any race or ethnic characteristic.
Results: A total of 143 patient cases with SCD were included in the hospital database. More than half of them were males. Hepatobiliary complications were observed in 19.5% of patients with SCD, cholelithiasis occurred in 26 (18.2%), cholangiopathy (bile duct stenosis) occurred in 3 (2.1%), and only one patient had acute hepatic sequestration (AHS). Biliary complication (cholelithiasis) was significantly associated with age and raised alanine aminotransferase (ALT) (p-value 0.042). Raised ALT was associated significantly with age, hemoglobin (Hb) level, and AHS. Total bilirubin > 2 mg/dl (> 34 micro mol/l) and direct bilirubin > 1 mg/dl (> 17 micro mol/l) were observed in 26.9% and 61.5% of patients who had cholelithiasis, respectively, low Hb in 46.2% and low platelet in 30.8%.
Conclusion: Hepatobiliary complications were observed in less than one-fifth of patients with SCD. The most common complication observed was cholelithiasis, rare complications were cholangiopathy and AHS.

Key words: Sickle cell disease, hepatobiliary complications, pediatric, Saudi Arabia