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Acute cholestatic jaundice in children with sickle cell disease: hepatic crises or hepatitis?
1Department of Pediatrics, Dhahran Health Center, Saudi Arabia.
Insights
Differentiating jaundice in children with sickle cell disease is challenging. Both hepatic vasoocclusive crises and hepatitis A present similarly but have distinct recovery patterns, with both proving benign.
Area of Science:
- Pediatric Gastroenterology
- Hematology
- Hepatology
Background:
- Sickle cell disease (SCD) patients are susceptible to liver complications, including jaundice.
- Distinguishing between hepatic vasoocclusive crises (VOC) and viral hepatitis as causes of cholestatic jaundice in SCD is clinically challenging.
- Both conditions can present with hyperbilirubinemia and elevated transaminases.
Purpose of the Study:
- To compare the clinical presentation, course, and outcome of acute cholestatic jaundice in children with SCD.
- To differentiate jaundice caused by hepatic VOC from that caused by hepatitis A.
- To assess the prognosis of these conditions in SCD patients.
Main Methods:
- Retrospective analysis of five children with SCD, acute cholestatic jaundice, and negative hepatitis A and B serology.
- Comparison with five children with SCD and serologically confirmed hepatitis A infection.
- Evaluation of clinical condition, bilirubin levels, transaminase levels, and recovery time.
Main Results:
- Children with SCD and jaundice due to hepatic VOC (negative hepatitis serology) presented well, with modest transaminase elevation, and recovered quickly.
- Children with SCD and hepatitis A were sicker, with marked transaminase elevation, and had a slower recovery.
- The clinical course of hepatitis A in SCD patients was similar to that in non-SCD children.
Conclusions:
- Acute cholestatic jaundice in children with SCD, whether from hepatic VOC or hepatitis A, appears to have a benign course with uneventful recovery.
- Hepatitis A in SCD patients follows a similar clinical pattern to that in the general pediatric population.
- Early reports suggesting severe outcomes for cholestatic jaundice in SCD may not reflect the typical clinical course.
Abstract:
Differentiating acute cholestatic jaundice resulting from hepatic vasoocclusive crises and hepatitis in children with sickle cell disease can be difficult. Both conditions result in hyperbilirubinemia, mainly of the conjugated type, and in elevation of serum transaminases. Five children with sickle cell disease, acute severe cholestatic jaundice and negative serology for hepatitis A and B presented in good general condition, with modest elevation of serum transaminases, and had an early uneventful recovery. Five children with sickle cell disease and serologically proved hepatitis A infection were sicker, exhibited a similar elevation of bilirubin concentration with marked elevation of the serum transaminases and recovered more slowly. The clinical course and outcome of hepatitis A in children with sickle cell disease was similar to that of hepatitis A in normal children. Unlike early reports acute cholestatic jaundice in our patients with sickle cell disease, whether caused by hepatitis or by hepatic vasoocclusive crises, was found to be benign with an uneventful recovery.