Single Center Experience of Demographic, Clinical, Laboratory, Radiological, Histopathological Findings and Treatment at the Time of Diagnosis in Children with Autoimmune Hepatitis Diagnosis

Öz

Objective: It was aimed to evaluate the demographic, clinical, laboratory and histopathological data at the time of diagnosis, treatment and response rates of pediatric patients with autoimmune hepatitis (AIH). Material and Methods: The data of patients diagnosed with AIH in our center in the last 10 years were analyzed retrospectively. Results: Of the 27 patients included in the study, 19 were female and 8 were male. The mean age at diagnosis was 10.89 ± 4.5 years, and 3 of them were Syrian ethnicity. Nine patients had extrahepatic diseases. The clinical presentation was most frequently acute hepatitis; 24 patients diagnosed with Type 1 AIH and 3 patients with seronegative AIH. The most common reason for admission was jaundice and fatigue. In physical examination, hepatomegaly and palmar erythema were observed most frequently. At the time of diagnosis, 23 patients' Ig G was more than 2 times higher and their ANA was positive. AMA in 2 patients and anti-SMA in 1 patient were positive. Liver biopsy revealed interface hepatitis in 23 patients and lymphoplasmacytic infiltration in 20 patients most frequently. Sclerosing cholangitis findings were observed in the magnetic resonance cholangiopancreatography of two patients. Complete clinical response was observed in 23 patients who were started on the first-line treatment regimen; There was partial response in 1 patient and no response in 3 patients. Treatment changes were made in seven patients due to treatment unresponsiveness and/or post-drug side effects. While corticosteroid monotherapy was preferred in two patients, tacrolimus was preferred in five patients. Conclusion: Due to the variability of the presentation type and duration of AIH in childhood, AIH should also be suspected in children presenting with liver disease findings. Studies with larger numbers of patients are needed on the diagnostic criteria, treatment regimens and second-line treatment agents of this rare disease.

Anahtar Kelimeler

• Epidemiology
• clinic
• laboratory autoimmune hepatitis
• treatment

OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ

Öz

Amaç: Otoimmün hepatit (OİH) tanılı çocuk hastaların tanı sırasındaki demografik, klinik, laboratuvar ve histopatolojik verilerini, tedavi ve tedaviye yanıt oranlarını değerlendirme amaçlanmıştır. Gereç ve Yöntemler: Merkezimizde son 10 yılda OİH tanısı konulan hastaların verileri retrospektif olarak incelendi. Bulgular: Çalışmaya alınan 27 hastanın 19’u kız, 8’i erkekti. Ortalama tanı yaşı 10.89 ± 4.5 yıldı ve 3’ü Suriye uyrukluydu. Dokuz hastada ekstrahepatik hastalıklar mevcuttu. Klinik prezentasyon en sık akut hepatit olup, 24 hastaya Tip 1 OİH ve 3 hastaya seronegatif OİH tanısı konuldu. En fazla başvuru sebebi sarılık ve halsizlikti. Fizik muayenelerinde en sık hepatomegali ve palmar eritem gözlendi. Tanı anında 23 hastanın Ig G’si 2 katından yüksek ve ANA pozitifti. İki hastanın AMA ve 1 hastanın anti-SMA pozitifti. Karaciğer biyopsisinde en sık olarak 23 hastada arayüz hepatiti ve 20 hastada lenfoplazmositik infiltrasyon saptandı. İki hastanın manyetik rezonans kolanjiopankreatografisinde sklerozan kolanjit bulguları gözlendi. Birinci basamak tedavi rejimi başlanan 23 hastada tam klinik yanıt görülürken; 1 hastada kısmı yanıt ve 3 hasta yanıtsızdı. Yedi hastada tedavi yanıtsızlığı ve/veya ilaç sonrası yan etki nedeniyle tedavi değişikliği yapıldı. İki hastada kortikosteroid monoterapisi tercih edilir iken beş hastada takrolimus kullanıldı. Sonuç: OİH’nin çocukluk çağındaki başvuru şekli ve süresinin değişkenliği sebebiyle karaciğer hastalığı bulguları ile başvuran çocuklarda OİH’den de şüphelenilmelidir. Çocuklarda nadir görülen bu hastalığın tanı kriterleri, tedavi rejimleri ve ikinci basamak tedavi ajanları konusunda daha fazla sayıda hasta içeren çalışmalara ihtiyaç vardır.

Anahtar Kelimeler

• Epidemiyoloji
• klinik
• laboratuvar
• otoimmün hepatit
• tedavi

Kaynakça

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