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

Yıl 2023, , 85 - 92, 30.04.2023
https://doi.org/10.24938/kutfd.1200104

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.

Kaynakça

  • Aksoy B, Çağan Appak Y, Baran M. Autoimmune Hepatitis in Children. The Journal of Tepecik Education and Research Hospital.2021;31(1):1-8.
  • Alvarez F. Autoimmune hepatitis in children. J Pediatr (Rio J). 2019;95(4):382-384.
  • Jiménez-Rivera C, Ling SC, Ahmed N, Yap J, Aglipay M, Barrowman N, et al. Incidence and characteristics of autoimmune hepatitis. Pediatrics. 2015;136(5):e1237-e48.
  • Manns MP, Lohse AW, Vergani D. Autoimmune hepatitis–update 2015. Journal of hepatology. 2015;62(1):S100-S11.
  • Mieli-Vergani G, Vergani D, Baumann U, Czubkowski P, Debray D, Dezsofi A, et al. Diagnosis and management of pediatric autoimmune liver disease: ESPGHAN hepatology committee position statement. Journal of pediatric gastroenterology and nutrition. 2018;66(2):345-60.
  • Porta G, Carvalho Ed, Santos JL, Gama J, Borges CV, Seixas RB, et al. Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes. Jornal de pediatria. 2019;95(4):419-27.
  • Kerkar N, Annunziato RA, Foley L, Schmeidler J, Rumbo C, Emre S, et al. Prospective analysis of nonadherence in autoimmune hepatitis: a common problem. Journal of pediatric gastroenterology and nutrition. 2006;43(5):629-34.
  • Bose T. Bitter correlationship between autoimmune hepatitis and smoking. Medical hypotheses. 2015;84(2):118-21.
  • Mack CL, Adams D, Assis DN, Kerkar N, Manns MP, Mayo MJ, et al. Diagnosis and management of autoimmune hepatitis in adults and children: 2019 practice guidance and guidelines from the American Association for the study of liver diseases. Hepatology. 2020;72(2):671-722.
  • Villalta D, Girolami E, Alessio MG, Sorrentino MC, Tampoia M, Brusca I, et al. Autoantibody profiling in a cohort of pediatric and adult patients with autoimmune hepatitis. Journal of Clinical Laboratory Analysis. 2016;30(1):41-6.
  • Luca-Johnson D, Wangensteen KJ, Hanson J, Krawitt E, Wilcox R. Natural history of patients presenting with autoimmune hepatitis and coincident nonalcoholic fatty liver disease. Digestive diseases and sciences. 2016;61(9):2710-20.
  • Arcos-Machancoses JV, Busoms CM, Tatis EJ, Bovo MV, de Carpi JM. Accuracy of the simplified criteria for autoimmune hepatitis in children: systematic review and decision analysis. Journal of clinical and experimental hepatology. 2019;9(2):147-55.
  • Kerkar N, Chan A. Autoimmune hepatitis, sclerosing cholangitis, and autoimmune sclerosing cholangitis or overlap syndrome. Clinics in liver disease. 2018;22(4):689-702.
  • Gregorio GV, Portmann B, Karani J, Harrison P, Donaldson PT, Vergani D, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001;33(3):544-53.
  • Sheiko MA, Sundaram SS, Capocelli KE, Pan Z, McCoy AM, Mack CL. Outcomes in pediatric autoimmune hepatitis and significance of azathioprine metabolites. Journal of pediatric gastroenterology and nutrition. 2017;65(1):80-85
  • Sütçüoğlu O, Sargın ZG, Haghighi NA, Yüceldi SD, Karataş A, Önal İK, et al. Otoimmün Hepatit Hastalarında Tek Merkez Tedavi Deneyimi. Gazi Medical Journal. 2017;28(3):174-8
  • Zizzo AN, Valentino PL, Shah PS, Kamath BM. Second-line agents in pediatric patients with autoimmune hepatitis: a systematic review and meta-analysis. Journal of Pediatric Gastroenterology and Nutrition. 2017;65(1):6-15

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

Yıl 2023, , 85 - 92, 30.04.2023
https://doi.org/10.24938/kutfd.1200104

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.

Kaynakça

  • Aksoy B, Çağan Appak Y, Baran M. Autoimmune Hepatitis in Children. The Journal of Tepecik Education and Research Hospital.2021;31(1):1-8.
  • Alvarez F. Autoimmune hepatitis in children. J Pediatr (Rio J). 2019;95(4):382-384.
  • Jiménez-Rivera C, Ling SC, Ahmed N, Yap J, Aglipay M, Barrowman N, et al. Incidence and characteristics of autoimmune hepatitis. Pediatrics. 2015;136(5):e1237-e48.
  • Manns MP, Lohse AW, Vergani D. Autoimmune hepatitis–update 2015. Journal of hepatology. 2015;62(1):S100-S11.
  • Mieli-Vergani G, Vergani D, Baumann U, Czubkowski P, Debray D, Dezsofi A, et al. Diagnosis and management of pediatric autoimmune liver disease: ESPGHAN hepatology committee position statement. Journal of pediatric gastroenterology and nutrition. 2018;66(2):345-60.
  • Porta G, Carvalho Ed, Santos JL, Gama J, Borges CV, Seixas RB, et al. Autoimmune hepatitis in 828 Brazilian children and adolescents: clinical and laboratory findings, histological profile, treatments, and outcomes. Jornal de pediatria. 2019;95(4):419-27.
  • Kerkar N, Annunziato RA, Foley L, Schmeidler J, Rumbo C, Emre S, et al. Prospective analysis of nonadherence in autoimmune hepatitis: a common problem. Journal of pediatric gastroenterology and nutrition. 2006;43(5):629-34.
  • Bose T. Bitter correlationship between autoimmune hepatitis and smoking. Medical hypotheses. 2015;84(2):118-21.
  • Mack CL, Adams D, Assis DN, Kerkar N, Manns MP, Mayo MJ, et al. Diagnosis and management of autoimmune hepatitis in adults and children: 2019 practice guidance and guidelines from the American Association for the study of liver diseases. Hepatology. 2020;72(2):671-722.
  • Villalta D, Girolami E, Alessio MG, Sorrentino MC, Tampoia M, Brusca I, et al. Autoantibody profiling in a cohort of pediatric and adult patients with autoimmune hepatitis. Journal of Clinical Laboratory Analysis. 2016;30(1):41-6.
  • Luca-Johnson D, Wangensteen KJ, Hanson J, Krawitt E, Wilcox R. Natural history of patients presenting with autoimmune hepatitis and coincident nonalcoholic fatty liver disease. Digestive diseases and sciences. 2016;61(9):2710-20.
  • Arcos-Machancoses JV, Busoms CM, Tatis EJ, Bovo MV, de Carpi JM. Accuracy of the simplified criteria for autoimmune hepatitis in children: systematic review and decision analysis. Journal of clinical and experimental hepatology. 2019;9(2):147-55.
  • Kerkar N, Chan A. Autoimmune hepatitis, sclerosing cholangitis, and autoimmune sclerosing cholangitis or overlap syndrome. Clinics in liver disease. 2018;22(4):689-702.
  • Gregorio GV, Portmann B, Karani J, Harrison P, Donaldson PT, Vergani D, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001;33(3):544-53.
  • Sheiko MA, Sundaram SS, Capocelli KE, Pan Z, McCoy AM, Mack CL. Outcomes in pediatric autoimmune hepatitis and significance of azathioprine metabolites. Journal of pediatric gastroenterology and nutrition. 2017;65(1):80-85
  • Sütçüoğlu O, Sargın ZG, Haghighi NA, Yüceldi SD, Karataş A, Önal İK, et al. Otoimmün Hepatit Hastalarında Tek Merkez Tedavi Deneyimi. Gazi Medical Journal. 2017;28(3):174-8
  • Zizzo AN, Valentino PL, Shah PS, Kamath BM. Second-line agents in pediatric patients with autoimmune hepatitis: a systematic review and meta-analysis. Journal of Pediatric Gastroenterology and Nutrition. 2017;65(1):6-15
Toplam 17 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular Sağlık Kurumları Yönetimi
Bölüm Özgün Araştırma
Yazarlar

Anna Carina Ergani 0000-0003-3494-1581

Meltem Gümüş 0000-0002-9257-6597

Muslu Kazım Körez 0000-0001-9524-6115

Halil Haldun Emiroğlu 0000-0002-1635-1150

Erken Görünüm Tarihi 30 Nisan 2023
Yayımlanma Tarihi 30 Nisan 2023
Gönderilme Tarihi 6 Kasım 2022
Yayımlandığı Sayı Yıl 2023

Kaynak Göster

APA Ergani, A. C., Gümüş, M., Körez, M. K., Emiroğlu, H. H. (2023). OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ. The Journal of Kırıkkale University Faculty of Medicine, 25(1), 85-92. https://doi.org/10.24938/kutfd.1200104
AMA Ergani AC, Gümüş M, Körez MK, Emiroğlu HH. OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ. Kırıkkale Üni Tıp Derg. Nisan 2023;25(1):85-92. doi:10.24938/kutfd.1200104
Chicago Ergani, Anna Carina, Meltem Gümüş, Muslu Kazım Körez, ve Halil Haldun Emiroğlu. “OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ”. The Journal of Kırıkkale University Faculty of Medicine 25, sy. 1 (Nisan 2023): 85-92. https://doi.org/10.24938/kutfd.1200104.
EndNote Ergani AC, Gümüş M, Körez MK, Emiroğlu HH (01 Nisan 2023) OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ. The Journal of Kırıkkale University Faculty of Medicine 25 1 85–92.
IEEE A. C. Ergani, M. Gümüş, M. K. Körez, ve H. H. Emiroğlu, “OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ”, Kırıkkale Üni Tıp Derg, c. 25, sy. 1, ss. 85–92, 2023, doi: 10.24938/kutfd.1200104.
ISNAD Ergani, Anna Carina vd. “OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ”. The Journal of Kırıkkale University Faculty of Medicine 25/1 (Nisan 2023), 85-92. https://doi.org/10.24938/kutfd.1200104.
JAMA Ergani AC, Gümüş M, Körez MK, Emiroğlu HH. OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ. Kırıkkale Üni Tıp Derg. 2023;25:85–92.
MLA Ergani, Anna Carina vd. “OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ”. The Journal of Kırıkkale University Faculty of Medicine, c. 25, sy. 1, 2023, ss. 85-92, doi:10.24938/kutfd.1200104.
Vancouver Ergani AC, Gümüş M, Körez MK, Emiroğlu HH. OTOİMMÜN HEPATİT TANISI KONULAN ÇOCUKLARDA TANI ANINDAKİ DEMOGRAFİK, KLİNİK, LABORATUVAR, RADYOLOJİK, HİSTOPATOLOJİK BULGULARIN VE TEDAVİNİN TEK MERKEZ DENEYİMİ. Kırıkkale Üni Tıp Derg. 2023;25(1):85-92.

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