Outcomes in Pediatric Autoimmune Hepatitis and Significance of Azathioprine Metabolites.

Sheiko MA, Sundaram SS, Capocelli KE et al.

*University of Colorado School of Medicine, Department of Pediatrics, Address: Digestive Health Institute, Children’s Hospital Colorado, 13123 E. 16th Ave, B290, Aurora, CO 80045 †University of Colorado School of Medicine, Department of Pediatrics, Address: Digestive Health Institute, Children’s Hospital Colorado, 13123 E. 16th Ave, B290, Aurora, CO 80045 ‡University of Colorado School of Medicine, Department of Pathology, Address: Department of Pathology, Children’s Hospital Colorado, 13123 E. 16th Ave, B120, Aurora, CO 80045 §University of Colorado School of Public Health, Department of Biostatistics and Informatics, Address: 13001 E. 17th Place, B119, Building 500, Room W3112, Aurora, CO 80045 ||University of Colorado School of Medicine, Department of Pediatrics, Address: Digestive Health Institute, Children’s Hospital Colorado, 13123 E. 16th Ave, B290, Aurora, CO 80045 ¶University of Colorado School of Medicine, Department of Pediatrics, Address: Digestive Health Institute, Children’s Hospital Colorado, 13123 E. 16th Ave, B290, Aurora, CO 80045.

Journal of pediatric gastroenterology and nutrition. Mar 2017.

Autoimmune hepatitis (AIH) is a common pediatric liver disease and long-term remission is usually maintained with azathioprine (AZA). There is no consensus on the target range for AZA active metabolite 6-thioguanine (6-TGN) levels in pediatric AIH. The aim of this study was to characterize the outcomes of pediatric patients with AIH and determine correlations between AZA dosing or 6-TGN metabolite levels and biochemical remission.A retrospective chart review was performed and data on presentation, laboratories including AZA metabolite levels, medication use and outcomes was collected.Between 2002-2013, 66 children with AIH were identified (mean age at diagnosis 9.6 ± 5.1 yrs.) with a mean follow-up period of 2.9 ± 3.2 yrs. Common presenting symptoms included jaundice, fatigue and abdominal pain. The majority of subjects received steroids for induction, and AZA for maintenance of remission. 79% achieved biochemical remission (mean time to remission 6.2 ± 9.2 mons.), 14% were in the induction phase of therapy, 6% required liver transplantation and 18% were weaned off of immunosuppression and remained in remission. 6-TGN levels ranging from 50-250 pmol/8 × 10 RBC were associated with biochemical remission (ALT levels of ≤ 50 U/L).The vast majority of children with AIH maintain a sustained remission with AZA monotherapy. Biochemical remission was maintained with 6-TGN levels much lower than that recommended for inflammatory bowel disease. These findings suggest that patients should be maintained at the lowest AZA dose possible that is associated with biochemical remission.

Pubmed

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