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Correlation of Serum Cholyiglycine Level with Hepatic Dysfunction in Children with Sickle Cell Anemia

Roula A. Farah, MD

Zora R. Rogers, MD

Department of Pediatrics, University of Texas Southwestern Medical Center and Children's Medical Center of Dallas

James E. Heubi, MD

Department of Pediatrics, University of Texas Southwestern Medical Center and Children's Medical Center of Dallas; Children's Hospital Medical Center, University of Cincinnati

George R. Buchanan, MD

Department of Pediatrics, University of Texas Southwestern Medical Center and Children's Medical Center of Dallas

Robert H. Squires, Jr, MD

Department of Pediatrics, University of Texas Southwestern Medical Center and Children's Medical Center of Dallas; Department of Pediatrics, Division of Gastroenterology and Nutrition, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd., Dallas, Texas 75235-9063

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 ensuing 2 years.

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