.: ORİJİNAL ARAŞTIRMA
Çocuklarda Otoimmün Hepatit: Tek Merkez Deneyimi: Tanımlayıcı Araştırma
Autoimmune Hepatitis in Children: A Single Center Experience: Descriptive Research
Turkiye Klinikleri J Pediatr. 2023;32(1):1-7
doi: 10.5336/pediatr.2022-92883
Makale Dili: TR
Tam Metin
Ücretsiz Erişim
ÖZET
Amaç: Çocukluk yaş grubunda değişik klinik bulgularla gelebilen otoimmün hepatit, nedeni tam açıklanamayan bazen ciddi seyir göstererek ölümcül olabilen bir durumdur. Bu çalışmada, kliniğimizde takip ettiğimiz otoimmün hepatitli hastaların irdelenmesi amaçlanmıştır. Gereç ve Yöntemler: Ocak 2010-Aralık 2020 tarihleri arasında takipli tanı anında 18 yaş altı otoimmün hepatit (OİH) hastalarının demografik özellikleri, klinik tabloları, laboratuvar ve görüntüleme bulguları, histopatolojik bulguları, tedavi ve seyirleri dosyalarından geriye dönük olarak değerlendirildi. Viral etiyolojiler, Wilson hastalığı ve alkole bağlı olmayan yağlı karaciğer hastalığı gibi kronik karaciğer hastalığı olası nedenleri dışlandı. Tüm hastalara karaciğer biyopsisi yapılarak, uyumlu histopatolojik bulgular ile tanı desteklendi. Bulgular: Değerlendirilen 18 hastanın 11'i (%61,1) kız, tanıda yaş ortalaması 9,75±4,52 yıl idi. En sık görülen klinik bulgu karın ağrısıydı (%66,7). Başlangıçta 10 (%55,6) hasta ikterikti, 7'sinde (%38,9) tanı 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. Hastaların hepsine steroid ve azatiyoprin, ayrıca kolestatik bulguları olan 15 (%83,3) hastaya ursodeoksikolik asit tedavisi verildi. Tedaviye dirençli 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 gelişti. Sonuç: Pediatrik çağda sıklıkla kızlarda ve 10 yaş civarında görülen OİH değişken klinik bulgularla seyredip, immünsupresif tedaviye sıklıkla yanıt vermektedir. Ancak ideal tedavi için ileri araştırmalara ihtiyaç vardır.
Anahtar Kelimeler: Çocuk; otoimmün hepatit; karaciğer sirozu; transaminazlar
Amaç: Çocukluk yaş grubunda değişik klinik bulgularla gelebilen otoimmün hepatit, nedeni tam açıklanamayan bazen ciddi seyir göstererek ölümcül olabilen bir durumdur. Bu çalışmada, kliniğimizde takip ettiğimiz otoimmün hepatitli hastaların irdelenmesi amaçlanmıştır. Gereç ve Yöntemler: Ocak 2010-Aralık 2020 tarihleri arasında takipli tanı anında 18 yaş altı otoimmün hepatit (OİH) hastalarının demografik özellikleri, klinik tabloları, laboratuvar ve görüntüleme bulguları, histopatolojik bulguları, tedavi ve seyirleri dosyalarından geriye dönük olarak değerlendirildi. Viral etiyolojiler, Wilson hastalığı ve alkole bağlı olmayan yağlı karaciğer hastalığı gibi kronik karaciğer hastalığı olası nedenleri dışlandı. Tüm hastalara karaciğer biyopsisi yapılarak, uyumlu histopatolojik bulgular ile tanı desteklendi. Bulgular: Değerlendirilen 18 hastanın 11'i (%61,1) kız, tanıda yaş ortalaması 9,75±4,52 yıl idi. En sık görülen klinik bulgu karın ağrısıydı (%66,7). Başlangıçta 10 (%55,6) hasta ikterikti, 7'sinde (%38,9) tanı 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. Hastaların hepsine steroid ve azatiyoprin, ayrıca kolestatik bulguları olan 15 (%83,3) hastaya ursodeoksikolik asit tedavisi verildi. Tedaviye dirençli 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 gelişti. Sonuç: Pediatrik çağda sıklıkla kızlarda ve 10 yaş civarında görülen OİH değişken klinik bulgularla seyredip, immünsupresif tedaviye sıklıkla yanıt vermektedir. Ancak ideal tedavi için ileri araştırmalara ihtiyaç vardır.
Anahtar Kelimeler: Çocuk; otoimmün hepatit; karaciğer sirozu; transaminazlar
ABSTRACT
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. Possible causes of chronic liver disease such as Wilson's disease, viral etiologies, 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 cirrhosis at the time of diagnosis. According to autoantibodies, 10 (55.6%) patients were considered Type 1, 2 patients (11.1%) Type 2 and 6 patients (33.3%) were considered seronegative. The most common histopathological finding was interface hepatitis (83.3%). At followup, 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 patients (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 frequently 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.
Keywords: Child; autoimmune hepatitis; liver cirrhosis; transaminasemias
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. Possible causes of chronic liver disease such as Wilson's disease, viral etiologies, 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 cirrhosis at the time of diagnosis. According to autoantibodies, 10 (55.6%) patients were considered Type 1, 2 patients (11.1%) Type 2 and 6 patients (33.3%) were considered seronegative. The most common histopathological finding was interface hepatitis (83.3%). At followup, 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 patients (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 frequently 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.
Keywords: Child; autoimmune hepatitis; liver cirrhosis; transaminasemias
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