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Published on: May 29, 2020
Autoimmune hepatitis and long-term disease course in children in Turkey, a single-center experience
Miray Karakoyun1, Cigdem O Ecevit, Erhan Kilicoglu
1aDepartment of Pediatric Gastroenterology, Hepatalogy and Nutrition, Gaziantep Children's Hospital, Gaziantep bDepartment of Pediatric Gastroenterology, Hepatology and Nutrition cDepartment of Pediatrics, Dr. Behcet Uz Children's Hospital dDepartment of Pediatric Gastroenterology, Hepatology and Nutrition, Ege University, Izmir, Turkey.
Insights
Autoimmune hepatitis (AIH) in Turkish children is predominantly type 1, with most responding well to prednisolone. Long-term treatment with low-dose corticosteroids and azathioprine shows a low relapse rate and manageable side effects.
Area of Science:
- Pediatric Hepatology
- Autoimmune Diseases
- Clinical Immunology
Background:
- Autoimmune hepatitis (AIH) is a chronic liver disease of unknown etiology.
- Pediatric AIH presents with diverse clinical and laboratory features.
- Understanding regional variations in AIH is crucial for effective management.
Purpose of the Study:
- To characterize clinic and laboratory features of pediatric AIH in Turkey.
- To evaluate treatment protocols and responses in children with AIH.
- To assess long-term follow-up, relapse rates, and side effects of AIH treatment.
Main Methods:
- Retrospective analysis of 47 children diagnosed with AIH between 1998 and 2012.
- Data collected included clinical presentation, autoantibody profiles, treatment regimens, and outcomes.
- Statistical analysis of clinical, laboratory, and treatment data.
Main Results:
- AIH type 1 was most prevalent (85.1%) in children, typically presenting in the second decade of life.
- Prednisolone induced complete remission in 86.4% of patients.
- Combination therapy with low-dose steroids and thiopurine resulted in a low relapse rate (9.4%) and minimal treatment side effects.
Conclusions:
- Autoimmune hepatitis type 1 is the predominant form in Turkish children.
- Low-dose corticosteroids combined with azathioprine represent an effective maintenance therapy for pediatric AIH.
- Long-term management can achieve sustained remission with acceptable safety profiles.
Introduction:
The aim of this study is to determine clinic and laboratory features, treatment protocols, treatment responses, and long term follow-up of children with autoimmune hepatitis (AIH) in a region of Turkey followed at Ege University.
Materials And Methods:
The records of 47 children with AIH between 1998 and 2012 were retrospectively analyzed for clinical profiles, treatment response, relapse rate, and long-term side effects.
Results:
The median age of the children was 10±4.1 years (55.3% females). A total of 29 patients presented with chronic hepatitis (61.7%). According to the autoantibody profiles, 40 (85.1%) and seven (14.9%) cases were classified as type 1 and type 2, respectively. Presentation with acute hepatitis and chronic hepatitis was significantly higher in type 1 disease. Laboratory findings at presentation was found similar among races as well as AIH types (P>0.05). The prednisolone was used for remission induction in 37 patients; 86.4% (n: 32) achieved a complete response, 2.7% (n: 1) achieved a partial response, and four patients (10.8%) showed no response. Maintenance was attained by low-dose steroid plus thiopurine and relapse in steroid responders (n: 32) was 9.4% (n: 3) at 8, 12, and 48 months. A total of 36% (n: 24) had neither acute nor chronic treatment side effects. Bone marrow suppression was observed in five patients and hyperglycemia was observed in one patient (10.6 and 2.1%), respectively.
Conclusion:
AIH type 1 prevails in children in a region of Turkey during the second decade of life. Low-dose corticosteroids combined with azathioprine are found.
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