FAKTOR PROGNOSIS TEMUAN HISTOPATOLOGI HATI DAN RASIO BILIRUBIN TOTAL 30/0 HARI TERHADAP KESINTASAN PASIEN ATRESIA BILIER PASCA OPERASI KASAI DI RSUP DR SARDJITO

DIAN NIRMALA SIRAIT, DR. dr. Akhmad Makhmudi, Sp.B, Sp.BA (K).; dr. Gunadi, PhD, Sp.BA

2021 | Tesis-Spesialis | BEDAH ANAK

Latar Belakang: Tanpa pengenalan dini dan prosedur Kasai, atresia bilier (BA) menyebabkan sirosis hati dan menyebabkan transplantasi atau kematian pada usia muda. Kami bertujuan untuk mengkarakterisasi temuan histopatologi hati untuk prediksi sirosis dan kelangsungan hidup pada pasien BA setelah operasi Kasai. Metode: Kami meninjau secara retrospektif semua hasil histopatologi untuk pasien BA yang menjalani biopsi hati selama operasi Kasai dari Agustus 2012 hingga Desember 2018 di Rumah Sakit Dr. Sardjito, Yogyakarta, Indonesia. Hasil: Lima puluh bayi dengan BA dipastikan dalam penelitian kami, 27 di antaranya laki-laki dan 23 perempuan. Usia rata-rata prosedur Kasai adalah 102,5 hari (kisaran interkuartil (IQR), 75,75-142,25 hari). Ada 33 (66%) dan 17 (34%) pasien BA dengan dan tanpa sirosis hati, sedangkan kelangsungan hidup secara keseluruhan adalah 52%. Pasien dengan proliferasi duktus biliaris berat, kolestasis berat, dan inflamasi portal berat memiliki risiko masing-masing 27, 22, dan 19,3 kali lipat untuk berkembang menjadi sirosis hati dibandingkan dengan pasien dengan proliferasi duktus biliaris sedang/ringan. sedang/ringan/tanpa kolestasis, dan inflamasi portal sedang/ringan (p = 3, masing-masing), sedangkan transformasi sel raksasa tidak terkait dengan perkembangan sirosis hati (p = 0,77). Proliferasi saluran empedu sangat berkorelasi dengan kolestasis dan peradangan portal (p = 3.6 - 10(-6) , 5.6 - 10(-4) and 1.6 - 10(-3), 4, masing-masing), dan kolestasis juga berkorelasi signifikan dengan peradangan portal (p = 0,016). Menariknya, usia pada prosedur Kasai sangat terkait dengan perkembangan sirosis hati (p = 0,02), tetapi tidak dengan kelangsungan hidup pasien (p = 0,33), sedangkan derajat fibrosis dan kolestasis secara signifikan berkorelasi dengan kelangsungan hidup pasien. , dengan HR masing-masing 33,9 (95% CI = 1,7-9,0; p = 0,017) dan 3,1 (95% CI = 1,4-7,0; p = 0,016). Kesimpulan: Temuan histopatologi proliferasi saluran empedu, kolestasis, dan peradangan portal dapat memprediksi perkembangan sirosis hati pada pasien dengan BA. Selanjutnya, derajat fibrosis dan kolestasis mempengaruhi kelangsungan hidup pasien setelah operasi Kasaisai

Background Without early recognition and Kasai procedure, biliary atresia (BA) results in liver cirrhosis and leads to either transplantation or death at a young age. Aim We aimed to characterize the liver histopathological findings for prediction of cirrhosis and survival in BA patients after Kasai surgery. Methods We retrospectively reviewed all histopathological results for BA patients who underwent liver biopsy during Kasai surgery from August 2012 to December 2018 in Dr. Sardjito Hospital, Yogyakarta, Indonesia. Results Fifty infants with BA were ascertained in our study, of whom 27 were males and 23 were females. The median age of Kasai procedure was 102.5 days (interquartile range (IQR), 75.75-142.25 days). There were 33 (66%) and 17 (34%) BA patients with and without liver cirrhosis, respectively, while the overall survival was 52%. The patients with a severe bile duct proliferation, severe cholestasis, and severe portal inflammation have a higher risk by 27-, 22-, and 19.3-fold, respectively, to develop liver cirrhosis compared with patients with a moderate/mild bile duct proliferation, moderate/mild/without cholestasis, and moderate/mild portal inflammation, respectively (p = 3.6- 10(-6) , 5.6- 10(-4) and 1.6- 10(-3), respectively), while the giant cell transformation was not associate with the development of liver cirrhosis (p = 0.77). The bile duct proliferation was strongly correlated with cholestasis and portal inflammation (p = 7.3 - 10(-5) and 2- 10(-4), respectively), and cholestasis was also significantly correlated with portal inflammation (p = 0.016). Interestingly, the age at Kasai procedure was strongly associated with the development of liver cirrhosis (p = 0.02), but not with the patients survival (p = 0.33), while the degree of fibrosis and cholestasis were significantly correlated with the patients survival, with HR of 3.9 (95% CI = 1.7-9.0; p = 0.017) and 3.1 (95% CI = 1.4-7.0; p = 0.016), respectively. Conclusions Histopathological findings of bile duct proliferation, cholestasis, and portal inflammation can predict the liver cirrhosis development in patients with BA. Furthermore, degree of fibrosis and cholestasis affect the patients survival following the Kasai operation.

Kata Kunci : Biliary atresia, Histopathological findings, Liver cirrhosis, Kasai procedure, Prognosis, Patient survival,