Comprehensive Exploration of Hepatobiliary Manifestations in Sickle Cell Disease: Hematological and Biochemical Profiles

Abstract

Introduction- Sickle Cell Disease (SCD) is a hereditary disorder with origins debated between migratory populations in India and equatorial Africa, spreading globally through trade routes. Liver involvement in SCD is frequent, marked by sickling, sinusoidal blockage, and hepatocyte abnormalities. Despite its significance, comprehensive data on liver function in SCD are lacking. This study conducted a one-year cross-sectional examination at MKCG Medical College and Hospital, focusing on pediatric patients with positive sickling tests. The study aimed to unravel hepatobiliary manifestations alongside hematological and biochemical profiles, addressing the current gap in comprehensive data. Results: A total of 210 cases showed a predominance in the 6-10 age group, with females outnumbering males. Chief complaints included pallor, weakness, jaundice, fever, and pain. Hepatic complications, notably benign hyperbilirubinemia, acute hepatic crisis, and cholelithiasis, were prevalent. Gastrointestinal system involvement was predominant, and ultrasonographic findings indicated splenomegaly and hepatosplenomegaly. Age-wise differences in complications and outcomes were observed, with 5.7% mortality, higher in SS than AS varieties. Discussion:The study highlighted the age distribution, clinical symptoms, and complications, emphasizing the need for early detection and comprehensive care. Complications were more prevalent in SS than AS groups, and mortality was higher in SS. Laboratory parameters were consistent with previous studies, underscoring the importance of a multidisciplinary approach for diagnosis and treatment.Conclusion: Complications in SCD increase with age, necessitating frequent hospital reporting. Hepatic complications, including benign hyperbilirubinemia, acute hepatic crisis, and cholelithiasis, were prevalent, stressing the importance of early detection and comprehensive care. Increased awareness and understanding of hepatobiliary complications, risk reduction, monitoring, and early intervention are crucial to decrease mortality in SCD.