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Çocuklarda Otoimmün Hepatit: Tek Merkez Deneyimi: Tanımlayıcı Araştırma

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dc.creator AKÇAM, Mustafa
dc.creator İSSİ, Fatma
dc.creator SALMAN, Hakan
dc.date 2023-01-27T00:00:00Z
dc.date.accessioned 2025-02-25T10:39:48Z
dc.date.available 2025-02-25T10:39:48Z
dc.identifier dec28006-eed9-4f82-9b96-ecc22eab436b
dc.identifier 10.5336/pediatr.2022-92883
dc.identifier https://avesis.sdu.edu.tr/publication/details/dec28006-eed9-4f82-9b96-ecc22eab436b/oai
dc.identifier.uri http://acikerisim.sdu.edu.tr/xmlui/handle/123456789/101626
dc.description Amaç: Çocukluk yaş grubunda değişik klinik bulgularla gele- bilen otoimmün hepatit, nedeni tam aç ıklanamayan bazen ciddi seyir göstererek ölümcül olabilen bir durumdur. Bu çalışmada, kliniğimizde takip ettiğimiz otoimmün hepatitli hastaların irdelenmesi amaçlanmış- tır. Gereç ve Yöntemler: Ocak 2010-Aralık 2020 tarihleri arasında ta- kipli tan ı anında 18 ya ş altı otoimmün hepatit (O İH) hastalar ının demografik özellikleri, klinik tabloları, laboratuvar ve görüntüleme bul- guları, histopatolojik bulguları, tedavi ve seyirleri dosyalarından geriye dönük olarak değerlendirildi. Viral etiyolojiler, Wilson hastalığı ve al- kole bağlı olmayan yağlı karaciğer hastalığı gibi kronik karaciğer has- talığı olası nedenleri d ışlandı. Tüm hastalara karaci ğer biyopsisi yapılarak, uyumlu histopatolojik bulgular ile tan ı desteklendi. Bulgu- lar: Değerlendirilen 18 hastan ın 11’i (%61,1) k ız, tanıda yaş ortala- ması 9,75±4,52 y ıl idi. En s ık görülen klinik bulgu kar ın a ğrısıydı (%66,7). Başlangıçta 10 (%55,6) hasta ikterikti, 7’sinde (%38,9) tan ı anında siroz gelişmişti. Otoantikorlara göre 10 (%55,6) hasta Tip 1, 2 (%11,1) hasta Tip 2 ve 6 (%33,3) hasta da seronegatif olarak kabul edildi. En sık görülen histopatolojik bulgu interface hepatitti (%83,3). Takipte 2 (%11,1) hastada varyant sendrom olarak primer sklerozan kolanjit, 4 (%22,2) hastada da ek otoimmün hastalık tespit edildi. Has- taların hepsine steroid ve azatiyoprin, ayrıca kolestatik bulguları olan 15 (%83,3) hastaya ursodeoksikolik asit tedavisi verildi. Tedaviye diren- çli 2 (%11,1) hastada tedavi değişikliği yapıldı. İlaç yan etkisi olarak 4 (%22,2) hastada sadece lökopeni, 2 (%11,1) hastada da hem lökopeni hem de osteopeni-osteoporoz geli şti. Sonuç: Pediatrik çağda sıklıkla kızlarda ve 10 ya ş civarında görülen O İH değişken klinik bulgularla seyredip, immünsupresif tedaviye s ıklıkla yanıt vermektedir. Ancak ideal tedavi için ileri araştırmalara ihtiyaç vardır.
dc.description Objective: Autoimmune hepatitis, which may present with different clinical findings in the childhood, is a condition that is not explained clearly, sometimes with a serious course and a mortal course. In this study, it was aimed to examine the patients with autoimmune hepatitis that we followed up in our clinic. Material and Methods: Demographic characteristics, clinical pictures, laboratory and imaging findings, hystopathological findings, treatment and course of patients with autoimmune hepatitis under 18 years of age followed up between January 2010 and December 2020 were evaluated, retrospectively. Pos- sible causes of chronic liver disease such as Wilson’s disease, viral eti- ologies, and non-alcoholic fatty liver disease were excluded. Liver biopsy was performed in all patients and the diagnosis was supported by histopathological findings. Results: Of the 18 patients evaluated, 11 (61.1%) were girls, and the mean age at diagnosis was 9.75±4.52 years. The most common clinical finding was abdominal pain (66.7%). At admission, 10 patients (55.6%) were icteric and 7 (38.9%) had cir- rhosis at the time of diagnosis. According to autoantibodies, 10 (55.6%) patients were considered Type 1, 2 patients (11.1%) Type 2 and 6 pa- tients (33.3%) were considered seronegative. The most common histopathological finding was interface hepatitis (83.3%). At follow- up, primary sclerosing cholangitis was detected as variant syndrome in two patients (11.1%), and additional autoimmune disease was detected in 4 (22.2%) patients. All patients received steroids and azathioprine, as well as ursodeoxycholic acid treatment in 15 (83.3%) patients with cholestatic findings. Treatment changes were made in 2 resistant pa- tients (11.1%). As a side effect of the drug, only leukopenia developed in 4 patients (22.2%) and both leukopenia and ostoepenia-osteoporosis in 2 patients (11.1%). Conclusion: Autoimmune hepatitis, which is fre- quently seen in girls and around 10 years of age in childhood, has with variable clinical findings and often responds to immunosuppressive therapy. However, further research is needed for the ideal treatment.
dc.language tur
dc.rights info:eu-repo/semantics/openAccess
dc.title Çocuklarda Otoimmün Hepatit: Tek Merkez Deneyimi: Tanımlayıcı Araştırma
dc.type info:eu-repo/semantics/article


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