Related Experiment Videos
Correlation of serum cholylglycine level with hepatic dysfunction in children with sickle cell anemia
A E Sayad1, R A Farah, Z R Rogers
1Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas 75235-9063, USA.
Insights
In children with sickle cell disease (SCD), elevated serum cholylglycine (CG) levels were common but did not predict future liver dysfunction or the need for gallbladder removal over two years.
Area of Science:
- Pediatric Hematology
- Hepatology
- Biochemistry
Background:
- Hepatic dysfunction is frequent in pediatric sickle cell disease (SCD).
- Cholestasis is a common manifestation of liver issues in SCD.
- Serum cholylglycine (CG) is a sensitive biomarker for cholestasis.
Purpose of the Study:
- To investigate elevated serum cholylglycine (CG) levels in children with SCD.
- To determine if CG levels predict hepatic dysfunction in these patients.
Main Methods:
- Blood samples collected from 97 children with SCD.
- Assessed liver function tests and measured serum CG concentrations.
- Followed patients for 2 years to monitor liver health outcomes.
Main Results:
- 38% of children with SCD exhibited elevated CG levels.
- No significant difference in abnormal liver function tests or cholecystectomy rates between elevated and normal CG groups over 2 years (16% vs. 13%, p=0.92).
Conclusions:
- Elevated serum cholylglycine (CG) is prevalent in children with SCD.
- Serum CG levels did not demonstrate predictive value for liver dysfunction in this cohort over a 2-year period.
Abstract:
Hepatic dysfunction occurs commonly in children with sickle cell disease (SCD). Although the etiology is multifactorial, cholestasis is a prominent feature. Serum cholylglycine (CG) has been found to be a very sensitive indicator of cholestasis. Our objective was to determine whether CG levels are elevated in children with SCD and whether they are predictive of hepatic dysfunction. Blood samples were obtained from 97 children with SCD. Liver function tests were done and serum CG concentrations were measured. Patients were followed up for 2 years. Thirty-eight percent of the patients had an elevated CG level. During the 2 years of follow-up, 16% of the children with a previously elevated CG level developed abnormal liver function test results or required a cholecystectomy as compared with 13% with a previously normal CG level (p = 0.92). We conclude that although CG level was elevated in 38% of the patients with SCD, it did not appear to predict liver dysfunction during the ensuring 2 years.