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Abstrak :
he fourth edition of this authoritative text covers every aspect of liver disease affecting infants, children, and adolescents. As in the previous editions, it offers an integrative approach to the science and clinical practice of pediatric hepatology and charts the substantial progress in understanding and treating these diseases. All of the chapters are written by international experts and address the unique pathophysiology, manifestations, and management of these disorders. This edition of the landmark text features extended coverage of viral hepatitis, metabolic liver disease, fatty liver disease, and liver transplantation, including a new chapter on post-transplant care and outcomes. All of the chapters have been updated to reflect changing epidemiology and recent advances in molecular medicine and genomics. With the continued evolution of pediatric hepatology as a discipline, this text remains an essential reference for all physicians involved in the care of children with liver disease.

Abstrak :
Latar Belakang: Penolakan cangkok akut pascatransplantasi hati anak dapat berakibat cangkok tidak berfungsi. Angka kejadian yang mencapai 31% di Rumah Sakit Cipto Mangunkusumo (RSCM) membutuhkan evaluasi faktor risiko untuk mengurangi morbiditas dan mortalitas. Metode: Rekam medis 44 resipien anak pascatransplantasi hati donor hidup dari tahun 2010-Januari 2020 dievaluasi, dan dianalisa menggunakan fisher test. Hasil: Sebelas subjek (25%) mengalami penolakan cangkok akut pascatransplantasi dengan median waktu 12 hari (jarak waktu 6-70 hari) pascatransplantasi. Total 44 subjek, 29 (65,9%) berusia > 1 tahun dan 30 (68,1%) bergizi kurang. Kejadian penolakan cangkok akut pada kelompok usia ≤1 tahun, adalah 5 (33%) dan pada usia >1 tahun, 6 (20%). Penolakan cangkok akut terjadi pada 6 subjek (20%) dengan gizi kurang, dan 5 subjek (35,7%) dengan gizi baik. Hasil analisa menunjukkan tidak ada hubungan antara usia (p= 0,468; 95% CI 0,47-0,77; OR 1,917) dan status gizi (p=0,287; 95% CI 0,11- 1,85; OR 0,450) terhadap reaksi penolakan cangkok akut pascatransplantasi hati donor hidup anak di RSCM. Hasil observasi tiga bulan pertama memperlihatkan rerata kadar tacrolimus darah 6-8 ng/mL pada hari 12-15, tidak mencapai target untuk mendapatkan efek imunosupresi yang adekuat. Kesimpulan: Pada penelitian ini status gizi kurang dan usia resipien saat transplantasi hati tidak signifikan sebagai faktor risiko independen reaksi penolakan cangkok akut, tetapi dapat dipikirkan bahwa kedua faktor ini mempengaruhi imunitas resipien, yang selanjutnya berperan dalam reaksi penolakan cangkok akut. Penggunaan imunosupresan yang adekuat juga harus diperhatikan dalam menekan reaksi penolakan cangkok pascatransplantasi hati. ......Background: Acute rejection post-liver transplant in children may result in graft failure. The incidence rate of up to 31% at Cipto Mangunkusumo Hospital (RSCM) needs further evaluation of risk factors to lower morbidity and mortality. Methods: 44 medical records of post living donor liver transplant pediatric recipients between 2010 until January 2020 were evaluated and analyzed using Fisher’s test. Results: Eleven subjects (25%) were found to experience acute rejection post-transplant with a median time of 12 days (range 6-70 days) after surgery. Of the 44 recipients, 29 subjects (65,9%) were >1 year old and 30 subjects (68,1%) were undernourished. Acute rejection occurred in 5 subjects (33%) ≤1 year-old and in 6 subjects (20%) that were >1 year old. Acute rejection of the transplant occurred in 6 subjects (20%) that were undernourished and in 5 subjects (35,7%) with good nutritional status. Analysis of the data found no relationship between age (p= 0,468; 95% CI 0,47-0,77; OR 1,917) and nutritional status (p=0,287; 95% CI 0,11-1,85; OR 0,450 to acute rejection in pediatric living donor liver transplant at RSCM. Observation in the first three months post-transplant reveal that mean levels of tacrolimus in the blood were 6-8 ng/mL on days 12-15, insufficient of reaching the target of obtaining an adequate immunosuppressive effect. Conclusion: In this study, age and nutritional status of recipients during the time of transplant were found to be insignificant independent risk factors of liver transplant acute rejection. However, these two factors can be thought to effect recipients’ immune status, which plays a role in acute rejection post-transplant. The use of adequate immunosuppressant needs to be carefully monitored in suppressing rejection reactions post-liver transplant.

Abstrak :
Latar Belakang. Infeksi sitomegalovirus (CMV) merupakan infeksi oportunistik tersering yang menjadi penyulit pascatransplantasi hati anak. Infeksi CMV aktif pascatransplantasi hati diperkirakan terjadi tidak hanya karena faktor serologi donor dan resipien, tetapi juga karena nilai Pediatric End-stage Liver Disease (PELD) atau Model for End-stage Liver Disease (MELD), penggunaan kombinasi imunosupresan, dan klinis rejeksi yang pernah dialami. Metode. Penelitian ini menggunakan desain kasus kontrol pada pasien anak yang telah menjalani transplantasi hati selama satu tahun atau lebih sejak tahun 2010 hingga tahun 2022di RSCM dengan stratifikasi risiko menengah. Sumber data diperoleh dari rekam medis dan hasil pemeriksaan polymerase chain reaction (PCR) CMV di Laboratorium Mikrobiologi Klinik FKUI. Hubungan antarvariabel dianalisis dengan menggunakan Fisher’s exact test dan t-test untuk dua populasi independen. Nilai p < 0,05 dianggap bermakna secara statistik pada analisis bivariat. Hasil. Insidens CMV aktif pascatransplantasi hati pada periode satu tahun pertama adalah 26,7%. Kesintasan kumulatif bebas infeksi CMV aktif pada bulan ke-12 adalah 73,3% dan incidence rate tertinggi pada bulan ke-3. Tidak ditemukan perbedaan yang bermakna secara statistik pada nilai PELD/MELD antara kelompok CMV aktif dan kelompok non-CMV aktif (IK 95% -5,24-3,14, p = 0,61). Penggunaan kombinasi imunosupresan dan klinis rejeksi yang pernah dialami sebelumnya tidak berhubungan dengan terjadinya infeksi CMV aktif (RO 1,50, IK 95% 0,33-6,68, p = 0,72; dan RO 1,36, IK 95% 0,36-5,10, p = 0,65). Post-hoc power dari masing-masing uji hipotesis yang digunakan adalah 50-76%. Kesimpulan. Insidens infeksi CMV aktif pascatransplantasi hati anak pada periode satu tahun pertama pada subjek dengan stratifikasi risiko menengah adalah 26,7%. Nilai PELD/MELD, penggunaan kombinasi imunosupresan, dan klinis rejeksi yang pernah dialami tidak berhubungan dengan risiko infeksi CMV aktif pascatransplantasi hati. Namun, power penelitian ini rendah sehingga dibutuhkan penelitian lanjutan dengan desain prospektif dan mengikutsertakan subjek yang lebih besar. ......Background. Cytomegalovirus (CMV) infection is the most common opportunistic infection after pediatric liver transplantation. Active CMV infection after liver transplantation was considered to be affected not only by serology status of recipient and donor, but also by Pediatric End-stage Liver Disease (PELD) score or Model for End-stage Liver Disease (MELD) score, combination of immunosuppressive agents, and previous clinically rejection. Method. Case control study was designed in this study, and children who experienced liver transplantation beyond one year from 2010 to 2022 at RSCM with moderate risk stratification were included in this study. Source data was obtained from medical record and polymerase chain reaction (PCR) CMV in Clinical Microbiology Laboratory Faculty of Medicine University of Indonesia. Analysis of two variables was explained using Fisher’s exact test and t-test for two independent population. A p value < 0.05 was considered to be statistically significant in bivariate analysis. Result. Incidence of active CMV infection after liver transplantation within first one year was 26.7%. Cumulative survival of free CMV infection at 12th month was 73.3% and the highest incidence rate was in the first 3 months. There was no statistically significant difference between PELD/MELD score in active CMV group and not-active-CMV group (CI 95% -5.24-3.14, p = 0.61). Combination of immunosuppressive agents and previous cellular rejection were not associated with active CMV infection (OR 1.50, CI 95% 0.33-6.68, p = 0.72; and OR 1.36, CI 95% 0.36-5.10, p = 0.65). Post-hoc power of each hypothesis test was 50-76%. Conclusion. Incidence of active CMV infection within first year after pediatric liver transplantation among moderate risk stratification group was 26.7%. PELD/MELD score, combination of immunosuppressive agents, and previous clinically rejection were not associated with risk of active CMV infection. However, this study was underpower that further studies need to be conducted with a prospective design and enrolled more participants.

Abstrak :
Latar belakang: Komplikasi vena hepatika merupakan komplikasi vaskular pascatransplantasi hati yang penting karena dapat mengakibatkan kongesti hati, sirosis dan kegagalan cangkok, dengan insidens lebih tinggi pada Transplantasi Donor Hidup (LDLT) dibandingkan teknik transplantasi lainnya. Tujuan: Mengetahui parameter ultrasonografi (USG) Doppler vaskular vena hepatika periode awal pascatransplantasi yang dapat berperan sebagai prediktor komplikasi vena hepatika Metode: Penelitian dilakukan pada 44 pasien pediatrik yang menjalani LDLT di RSUPN Dr. Ciptomangunkusumo dari tahun 2010 hingga Juli 2022 yang memiliki imaging USG Doppler vaskular pada hari pertama hingga ketiga pascatransplantasi. Kecepatan dan pola gelombang vena hepatika dari USG Doppler dievaluasi pada kelompok dengan dan tanpa komplikasi vena hepatika pada tahun pertama pascatransplantasi. Nilai titik potong kecepatan vena hepatika ditentukan menggunakan receiver operating curve. Hasil: Kecepatan vena hepatika pada hari kedua pascaoperasi secara signifikan lebih rendah pada pasien dengan komplikasi vena hepatika, dengan nilai tengah 21,3 (16,6-23,3) cm/detik, dibandingkan 28,9 (10,7-75,0) cm/detik pada pasien tanpa komplikasi vena hepatika, (nilai p = 0,018). Nilai titik potong kecepatan vena hepatika hari kedua pascaoperasi dalam membedakan komplikasi dan tanpa komplikasi vena hepatika adalah 23,65 cm/detik, sensitivitas 100%, spesifisitas 76,3%, AUC 0,803 (IK95% 0,679-0,927), dan nilai p = 0,018. Tidak terdapat perbedaan proporsi pola gelombang monofasik vena hepatika antara pasien dengan komplikasi vena hepatika dibandingkan dengan pasien tanpa komplikasi vena hepatika. Kesimpulan: Kecepatan vena hepatika yang rendah pada USG Doppler vaskular periode awal pascaoperasi terutama di hari kedua dapat membantu memprediksi komplikasi vena hepatika sehingga meningkatkan kewaspadaan dini terhadap komplikasi vena hepatika pada tahun pertama pascatransplantasi. ......Background: Hepatic vein complication is an important postoperative complication in pediatric liver transplantation in which liver congestion may progress to cirrhosis and graft failure, with higher incidence in living donor liver transplantation (LDLT) compared to other liver transplantation technique. Objective: This study aims to identify the role of Doppler ultrasound parameters of hepatic vein in early postoperative period of pediatric LDLT as predictors of hepatic vein complications. Methods: From 2010 to July 2022, there were 44 pediatric LDLT patients in RSUPN Dr. Ciptomangunkusumo who had Doppler ultrasound imaging from first until third postoperative day. Hepatic vein velocity and waveform were compared in patients with and without hepatic vein complications in one year follow up. Cut off point of hepatic vein velocity is determined using receiver operating curve. Results: Hepatic vein velocity in second postoperative day is significantly lower in patients with hepatic vein complication, with median of 21,3 (16,6-23,3) cm/s compared to 28,9 (10,7-75,0) cm/s in patients without hepatic vein complication (p value = 0,018), with cut off value to differentiate both group is 23,65 cm/s, sensitivity 100%, specificity 76,3%, AUC 0,803 (CI95% 0,679-0,927), and p value = 0,018. There is no difference in monophasic waveform proportion between patients with and without hepatic vein complication. Conclusion: Lower hepatic vein velocity in early postoperative Doppler ultrasound of pediatric LDLT, especially in second postoperative day, may aid to predict hepatic vein complication in first year follow up. Such finding may increase awareness of hepatic vein complication in the first year after transplantation.