OTOİMMÜN HEPATİT TANILI ÇOCUKLARDABAŞVURU ŞEKLİ VE EŞLİK EDEN OTOİMMÜN HASTALIK VARLIĞI

Giriş ve Amaç: Otoimmün hepatit (OİH)çocukluk çağında nadir görülen karaciğerin inflamatuar hastalığıdır. Klinik prezantasyon çok değişkendir. OİH olan hastaların yaklaşık %20-40’nda eşlik eden otoimmün hastalıklar gözlenebilir. Bu çalışmamızda OİH tanısı almış çocukların başvuru şekli, biyokimyasal parametreleri, eşlik eden otoimmün veya otoinflamatuar hastalık varlığının araştırılması amaçlanmıştır. Gereç ve Yöntem:Yaşları 6 ila 18 arasında değişen, OİH tanısı almış 17değerlendirildi.Başvuru şekli, laboratuvar bulguları,eşlik eden otoimmün veya otoinflamatuar hastalıklar, anne-babada otoimmün hastalık varlığı kayıt edildi. Biyokimyasal parametreler başvuru şekline ve eşlik eden otoimmün hastalık varlığına göre kıyaslandı. Bulgular:OİH olan çocukların ondördü ( 10 kız, 4 erkek) tip1 OİH, üçü (1 kız, 2 erkek) ise tip-2OİH tanısı almıştı. Hastaların %53 akut hepatit, % 47 ise insidental karaciğer enzim yüksekliği nedeniyle başvurmuştu. OİH olan çocukların %35’inde eşlik eden bir veya birden fazla otoimmün veya otoinflamatuar hastalık gözlendi. En sık vitiligo (%12), ikinci sıklıkta ise Çölyak hastalığı (% 6) juvenil idiopatik artrit (%6), Ailevi Akdeniz ateşi %6 oranında gözlendi. Tip-1 OİH nedeniyle takip edilen bir hastada hem tip 1 diabetes mellitus hem de Hashimoto tiroiditi (HT) vardı.Eşlik eden otoimmün hastalığı olan çocukların hepsinin cinsiyeti kız ve insidental olarak enzim yüksekliği nedeniyle başvurmuş olanlardı. Hasta ebeveynlerinin % 24 de otiommün hastalık (üç kişide HT, iki kişide vitiligo) vardı. Sonuç:Çocuklarda OİH, kronik karaciğer hastalığının nadir fakat önemli nedenleri arasındadır. OİH ile birlikte diğer otoimmün ve otoinflamatuar hastalıkların da eşlik edebileceği, erken tanının tedavinin gecikmemesi açısından önemliolduğu unutulmamalıdır.

Mode of Presentation and Associated Autoimmune Diseasesin Children with Autoimmune Hepatitis

Background and Aims:Autoimmune hepatiti (AIH) is an inflammatory disease of the liver with variable clinical presentations.20-40% of thepatients with AIHhad another associated autoimmune or autoinflammatory disease. This study aimed to assessmode of presentation, biochemical features and outcomes in children with AIH, as well as to evaluate the frequency of concomitant autoimmune diseases (CAIDs). Materials and Methods: 17 children, aged 6 to 18 years were enrolled.The mode ofpresentation and accompanying autoimmune diseases were recorded. Biochemical parameters as well as immunoglobulin G levels were evaluated either at time of submission and thereafter. Results:Fourteenpatients had type-1AIH (10 females, 4 males), and three (2 males, 1 female) had type-2AIH. The mode of presentation was acute in 53% and incidental enzyme elevation in 47% of them. There was an associated autoimmune or auto-inflammatory disease in 35% of the patients, 12% had vitiligo, 6% had celiac disease, 6% had juvenile idiopathic arthritis,6% had Familial Mediterranean Fever, and one patient had both type-1diabetes mellitus and Hashimoto thyroiditis (HT). The subjects with CAIDs were females (6 patients) with insidious type of presentation. Autoimmune diseases were observed in 24%of the parents (3 had HT, 2 had vitiligo). Conclusion: AIH is a rare but important cause of chronic liver disease in children. Frequent association with autoimmune diseases should be kept in mind as the clinical expression of the associated disease can be extremely variable therefore diagnosis and treatment delay may occur.

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