Emirzad DEMİREL, Tuğçin Bora POLAT, Seda Geylani GÜLEÇ, Ela ERDEM, Nafiye URGANCI

Otoimmün hepatitli olgularımızın irdelenmesi: 11 yıllık deneyimimiz

Amaç: Otoimmün hepatit tanılı olgularımızın klinik ve laboratuvar verilerini, tedavi sonuçlarını ve tedavi yan etkilerini irdelemeyi amaçladık. Gereç ve Yöntem: Otoimmün hepatit tanılı olguların dosyaları geriye dönük olarak incelendi. Tüm olgularda viral ve metabolik nedenler dışlandı. Olgular tip 1, tip 2, tanımlanamayan tip ve ayrıca akut, kronik olarak gruplandırıldı. Olguların tedavi yanıtı ve tedavi sonlandırılma bilgileri değerlendirildi. Bulgular: Olgular 4-17 yaş (12±2,68 yıl) arasında olup, 20’si kız 11’i erkekti. Olguların 17’si akut, 14’ü kronik; 18’i tip-1, altısı tip-2 ve yedisi tanımlanamayan tip idi. Tüm olgulara deflazakort başlandı. Geç yanıt alınan 10 olguya üçüncü ayın sonunda azatiyopirin (2 mg/kg/gün) eklendi. Deflazakort 6-8 haftada bir azaltılarak 5 mg/gün dozuyla devam edildi. İki yılın sonunda 24 olgu tam yanıtlı, beş olgu kısmi yanıtlı, iki olgu ise yanıtsız olarak değerlendirildi. Dokuz yıllık izlem süresince altı olgunun tedavisi sonlandırıldı. Bir olguda altı ay sonra tekrarlama oldu. Tedavisi kesilen beş olgu hala sorunsuz olarak izlenmektedir. Tedaviye rağmen beş olguda portal hipertansiyon bulguları gelişti. Çıkarımlar: Çocukluk yaşı otoimmün hepatitinin erken tanı ve tedavisi siroza ilerleyiş hızını azaltmakta ve yaşam süresini uzatmaktadır. Deflazakortun tedavideki etkinliği ve yan etkilerinin azlığı göz önüne alındığında otoimmün hepatit tanılı çocuklarda kullanılabileceği görüşündeyiz.

Evaluation of patients with autoimmune hepatitis: eleven years of experience

Aim: This study aimed to review a series of patients with autoimmune hepatitis in terms of disease pattern, laboratory results, treatment outcomes and adverse effects of treatment. Material and Method: Children with autoimmune hepatitis were retrospectively reviewed. In all patients, viral and metobolic eitologies were excluded. Patients were classified as type-1, type-2 and non-classified type, as well as acute and chronic groups. Treatment response and cessation of treatment were evaluated. Results: Patients were beetween 4 and 17 years old (12±2.68 years). Twenty patients were female, 11 patients were male. Seventeen patients were in acute and 14 patients were in chronic group; 18 of them were in type-1, 6 of them in type-2 and 7 of them were in non-classified type. Deflazacort was started in all patients. Azathioprine (2 mg/kg/day) was added to 10 patients with late response at the end of the third month. Deflazacort dosage was decreased at 6-8 weeks intervals and continued at a maintanance dosage of 5 mg/day. After a two-year period complete response was obtained in 24, partial response was obtained in 5 patients. No response was obtained in 2 patients. In the nine-year follow-up period, treatment was ended in 6 patients. In one patient, there was a recurrance in 6 months; the remaining 5 patients are still being followed up without a problem. Despite treatment portal hypertension was observed in 5 patients. Conclusions: Early diagnosis and treatment of childhood autoimmune hepatitis can decrease the risk of progression to cirrhosis and can increase the survival. Deflazocort can be a choice instead of prednisolone because of lesser side effects and efficiancy in treatment.

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