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"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."

"Liver disease has been identified as a leading cause of death in HIV-infected patients since the introduction of highly active antiretroviral therapy (HAART) in 1996. The HIV treatment community has been caught largely unaware of this emerging dilemma. Many HIV care providers are ill-equipped to understand and interpret liver injury patterns, or to provide comprehensive care and management for viral coinfections which they are not familiar with.HIV and liver disease provides a comprehensive update of the field covering the epidemiology, pathogenesis, management and treatment of liver disease in patients with HIV infection. The volume will help HIV care providers understand and interpret liver injury patterns, and/or provide comprehensive care and management for viral coinfections. Gastroenterologists and hepatologists will gain an understanding of complex drug regimens that are used to treat HIV and which may impact HCV and HBV treatment. Written by expert clinicians and researchers across multiple disciplines, HIV and Liver Disease will be of great value to gastroenterologists, hepatologists, infectious disease practitioners, as well as other health care providers who provide care or participate in research in the field of HIV."

"Latar belakang: Pilihan terapi terbaik untuk gagal hati terminal adalah transplantasi hati (TH). Setelah transplantasi seorang anak akan mempunyai hati dengan fungsi normal, namun tidak berarti menjadi anak yang sehat. Pasca TH anak akan berada dalam kondisi kronis dengan morbiditas tersendiri.Tujuan: Mengetahui perbedaan Quality of Life anak dengan penyakit hati kronis (PHK) yang dilakukan TH dengan yang tidak secara kuantitatif.Metode: Penelitian ini merupakan studi potong lintang terhadap anak pasca TH minimal selama 1 tahun dan anak PHK yang merupakan kandidat TH berusia 2-18 tahun. Subjek pasca TH diambil secara konsekutif, kelompok PHK minimal berjumlah setara dengan dengan kelompok pasca TH. Kedua kelompok dilakukan penilaian Quality of Life (QoL) menggunakan kuesioner PedsQLTM4.0 dalam Bahasa Indonesia yang telah divalidasi. Rerata nilai PedsQLTM4.0 pada anak sehat adalah 82,92+15,55 dan 83,91+12,47 masing-masing untuk proksi orangtua (OT) dan penilaian anak (A). Pada kondisi kronis rerata PedsQLTM,4.0 untuk proksi OT dan A masing-masing adalah 73,14+16,46 dan 74,16+15,38. Nilai yang lebih tinggi menunjukkan QoL yang lebih baik. Nilai <1 SD adalah batas anak memerlukan intervensi terkait QoL nya.Hasil: Kesintasan 1 dan 5 tahun pasca TH anak di RSCM adalah 85,4% dan 79,3%. Subjek pasca TH proporsi kelompok usia terbanyak adalah 5-7 tahun (66,7%), median usia 6 tahun 7,5 bulan; diagnosis dasar terbanyak adalah atresia bilier (84,6%). Pada subjek PHK kelompok usia terbanyak adalah 2-4 tahun (46,3%), median usia 9 tahun, penyebab terbanyak adalah kelainan vaskular (29,3%). Rerata total QoL subjek pasca TH 1 tahun lebih tinggi dibandingkan dengan subjek PHK.Kesimpulan: Rerata nilai total QoL 39 subjek pasca TH lebih baik dibandingkan 41 subjek PHK kandidat TH baik berdasarkan proksi OT maupun A secara bermakna. Penilaian QoL proksi OT dan anak menyimpulkan dimensi terbaik adalah fungsi sosial dan terendah fungsi sekolah. Pada penelitian ini QoL tidak dipengaruhi oleh status gizi, infeksi CMV/ EBV, ataupun rejeksi.

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Background: Liver transplantation (LT) is the best-known treatment for terminal chronic liver disease (CLD). Following a LT procedure, the patient will have a functional liver but is not considered as healthy child. Post LT, the patient will remain in a chronic condition with its own morbidity.

Objective: To distinguish Quality of Life (QoL) distinction between terminal CLD patient who underwent LT and not.

Methods: A cross-sectional study was conducted on 39 subjects consisting of 1 year LT survivor patient and 41 LT candidate children, aged 2-18 years. Liver recipient subjects were taken consecutively, equal amount CLD subjects were collected. Both groups were assessed for Quality of Life (QoL) using the validated PedsQLTM4.0 questionnaire in Indonesian. PedsQLTM4.0 cut-off point average scores in healthy children (population) are 82.92+15.55 and 83.91+12.47 each representing parent proxy (P) and child self-assessment (C). In chronic conditions children, cut off point average score of PedsQLTM4.0 for P and C were 73.14+16.46 and 74.16+15.38 respectively. Higher values ​​indicate better QoL. One standard deviation below the population mean was explored as a cut-off point score for an at-risk status of impaired QoL.

Results: The 1 and 5 years-survival rate of LT children in RSCM were respectively 85.4% and 79.3%. Liver recipient subjects mostly consist of 5-7 years (66.7%) age group, median age was 6 years and 7.5 months; and the most prevalent diagnosis was biliary atresia (84.6%). In CLD, a portion of 2-4 years old age group (46.3%) was the dominant, the median was 9 years, and the most common diagnosis was vascular disorders (29.3%). Higher QoL score in post-transplant subjects was observed.

Conclusion: LT children’s QoL was significantly higher than children who were candidate for LT, according to parent proxy and child self-assessment. Based on both parent proxy and child-assessment, social function was observed to have the best the QoL function and school function scored the lowest. In this study, QoL of liver recipient children were not affected by nutritional status, CMV or EBV infection, nor rejection."

"Obesity is performed by genetic, environmental, and complex interaction between genetic and environmental factors.Fatness levels in obese children are indicative of increased risk for elevated blood pressure, cholesterol, and lipoproteinratios children and adolescents. Serum cholesterol and blood pressure are related to raise atherosclerotic lesion. Certaincardiovascular disease risk factors in obese children are related to the earliest stages of atherosclerosis disease. Obesechildren have the high risk factors of certain cardiovascular disease. Genetic factors affecting metabolic rate can besuccessfully managed by the introduction of environmental factors such as decrease caloric intake and increase physicalactivity. The treatment of obesity and of atherosclerotic patients should include dietary restriction (protein sparingmodified fast and hypocaloric balanced diet), nutrition education, increased physical activity, behavior modification, andfamilial support. Success fully management of obesity can improve the quality of life and reduce the risk of morbidityand mortality associated with obesity."

"Latar Belakang. Lean NAFLD lebih sering ditemukan di negara Asia dan prevalensinya di Indonesia masih belum diketahui. Tingginya prevalensi, asimptomatik dan baru bergejala setelah timbul komplikasi, dan tingginya mortalitas lean NAFLD menjadikan perlunya deteksi dini pada populasi dewasa dengan IMT <23 kg/m2. Skrining pada populasi umum tidak direkomendasikan karena meningkatkan biaya kesehatan.Tujuan. Membuat sistem skoring untuk penapisan lean NAFLD pada populasi dewasa di Jakarta.Metode. Studi ini menggunakan desain potong lintang dari laporan pemeriksaan kesehatan individu dewasa >18 tahun dengan IMT <23 kg/m2 yang melakukan pemeriksaan kesehatan di klinik. Parameter yang dianalisis antara lain usia, jenis kelamin, lingkar pinggang, kadar GDP, kolesterol total, HDL, LDL, trigliserida, AST, ALT, dan asam urat. Variabel dengan nilai p <0,25 dilanjutkan ke analisis multivariat untuk pembuatan sistem skoring.Hasil. Sebanyak 276 individu diikutsertakan pada penelitian ini dan didapatkan prevalensi lean NAFLD sebesar 9,8%. Lean NAFLD lebih banyak ditemukan pada laki-laki dan memiliki karakteristik usia lebih tua, IMT, lingkar pinggang, kadar GDP, ALT, dan trigliserida lebih tinggi dibanding lean tanpa NAFLD. Analisis bivariat mendapatkan jenis kelamin laki-laki, usia ≥45 tahun, kadar GDP ≥100 mg/dL, ALT ≥35 U/L, dan trigliserida ≥150 mg/dL berhubungan dengan lean NAFLD. Sistem skoring melibatkan 4 parameter yaitu laki-laki, kadar GDP ≥100 mg/dL, ALT ≥35 U/L, dan trigliserida ≥150 mg/dL dengan masing-masing bernilai 1 poin. Model skoring ini memiliki sensitivitas 44,4%, spesifisitas 84,3%, dan AUROC 0,74.Kesimpulan. Parameter jenis kelamin, kadar GDP, ALT, dan trigliserida dapat digunakan sebagai sistem skoring dengan performans menengah untuk penapisan lean NAFLD dewasa......Background. Lean NAFLD is commonly found in Asian countries and its prevalence in Indonesia is still unknown. The high prevalence, asymptomatic until complications occur, and the high mortality of lean NAFLD makes it necessary for early detection in adult with BMI <23 kg/m2. Screening in general population is not recommended due to the high cost burden.Aim. To develop a scoring system for screening lean NAFLD in adults in Jakarta Methods. A cross-sectional study design was conducted from medical examination reports from individual >18 years old and BMI <23 kg/m2 who performed medical check up at the clinic. Several parameters including age, gender, waist circumference (WC), fasting blood glucose (FBG), total cholesterol (TC), HDL, LDL, triglycerides (TG), AST, ALT, and uric acid (UA) were analyzed in this study. Variabels with p-value <0.25 were included in multivariate analysis for the development of scoring systems.Results. A total of 276 people were enrolled in this study. Prevalence of lean NAFLD is 9.8%. Lean NAFLD are more commonly found in men and have older age, higher BMI, WC, GDP, ALT, and TG levels than lean non-NAFLD. In bivariate analysis, male sex, age ≥ 45 years, FBG ≥100 mg/dL, ALT ≥35 U/L, and TG ≥150 mg/dL are associated with lean NAFLD. The scoring system involves four parameters including male, FBG ≥100 mg/dL, ALT ≥35 U/L, and TG ≥150 mg/dL, worth 1 point each. This model has sensitivity 44.4%, specificity 84.3%, and AUROC 0.74.Conclusion. Parameters including gender, FBG, ALT, and TG levels can be used as a scoring system with moderate performance for screening lean NAFLD in adults."

"Latar belakang: Indonesia merupakan negara maritim yang berhubungan erat dengan industri pengiriman dan kekayaan laut yang berdampak pada sosio-ekonomik negara. Kesehatan pelaut berperan penting dalam mempertahankan manajemen ini dan perlu mendapat perhatian khusus. Pelaut memiliki risiko mengalami MAFLD, kelelahan saat bertugas, atau kombinasi keduanya. Penelitian ini bertujuan untuk mengetahui hubungan antara MAFLD dengan kelelahan kerja pada Pelaut tugboat.

Metode: Penelitian ini menggunakan metode potong lintang dengan data sekunder hasil MCU karyawan Perusahaan X. MAFLD didefinisikan sebagai fatty liver berdasarkan hasil USG ditambah dengan adanya obesitas atau overweight. Kelelahan diukur menggunakan kuesioner SOFI yang telah divalidasi dalam bahasa Indonesia. Hubungan antara MAFLD dengan kelelahan dianalisis menggunakan uji regresi logistik untuk mendapatkan nilai signifikansi (P), odds ratio (OR) dan interval kepercayaan (IK) 95%.

Hasil: Prevalensi pekerja yang mengalami kelelahan sedang sebanyak 64 orang (23,5%). Kelelahan kerja secara statistik berhubungan signifikan dengan MAFLD (aOR 5,05; IK 95% 2,65-9,60; p <0,001) dan kurangnya aktivitas fisik/olahraga (aOR 2,79; IK 95% 1,17-6,68; p 0,021).

Kesimpulan: Hampir seperempat dari total jumlah pelaut tugboat mengalami kekelahan sedang saat bekerja. Kelelahan tersebut berhubungan signifikan dengan MAFLD dan kurangnya aktivitas fisik/olahraga

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Background: Indonesia is a maritime nation closely associated with shipping industry and oceanic wealth that impacts the country's socio-economic status. The health of sailors plays a crucial role in maintaining this management and requires specific attention. Sailors are at risk of experiencing MAFLD, on-duty fatigue, or a combination of both. This research aims to investigate the relationship between MAFLD and work-related fatigue among tugboat sailors.

Methods: This study used a cross-sectional method with secondary data from the Medical Check-Up (MCU) results of Company X's employees. MAFLD was defined as fatty liver based on ultrasound results combined with the presence of obesity or overweight. Fatigue was measured using the validated SOFI questionnaire in the Indonesian language. The association between MAFLD and fatigue was analyzed using logistic regression to obtain significance values (P), odds ratios (OR), and a 95% confidence interval (CI).

Results: The prevalence of workers experiencing moderate fatigue was 64 individuals (23.5%). Statistically, work-related fatigue was significantly associated with MAFLD (aOR 5.05; 95% CI 2.65-9.60; p <0.001) and insufficient physical activity/exercise (aOR 2.79; 95% CI 1.17-6.68; p 0.021).

Conclusion: Nearly a quarter of the total number of tugboat sailors experience moderate fatigue while working. This fatigue is significantly associated with MAFLD and insufficient physical activity/exercise."

"Latar belakang: Kekurangan gizi merupakan penyebab umum morbiditas pada anak dengan penyakit jantung bawaan (PJB). Data dari negara berkembang memperlihatkan prevalensi malnutrisi penderita dengan PJB sebelum dioperasi mencapai 45%. Penelitian ini bertujuan untuk mengetahui profil anhropometrik dan prevalensi kekurangan gizi pada anak dengan PJB dengan melakukan pengukuran anthropometrik.Metode: Penelitian ini merupakan penelitian dengan rancang bangun cross sectional pada anak berusia 0-2 tahun dengan PJB di RSCM. Pengukuran antropometri (berat badan, panjang badan, lingkar kepala) dilakukan pada seluruh pasien. Kekurangan gizi, failure to thrive/FTT, perawakan pendek, mikrosefali dinilai dengan menggunakan rekomendasi WHO tahun 2006, berupa perhitungan z-skor BB/PB, BB/U di 2 titik, PB/U dan LK/U < -2 SD.Hasil: Total subyek dalam penelitian ini berjumlah 95 orang, 73 orang dengan asianotik dan 22 orang dengan PJB sianotik. Prevalensi kekurangan gizi sebesar 51,1% dengan 22,3% diantaranya adalah gizi buruk. FTT terdapat pada 64,9%, perawakan pendek pada 49,5% dan mikrosefali pada 37% pasien. FTT ditemukan lebih banyak pada pasien dengan lesi asianotik (72,2%) dibandingkan dengan lesi sianotik (42,9). Pada lesi asianotik, berat badan lebih dipengaruhi daripada panjang badan (72,2% dengan 49,3%). Pasien dengan lesi sianotik, berat dan panjang badan akan dipengaruhi secara seimbang (42,9% dengan 54.5%). Konsultasi diet diberikan kepada pasien dengan kekurangan gizi. Terapi obat-obatan, intervensi transkateter atau bedah diindikasikan pada pasien tertentu.Kesimpulan: Prevalensi FTT lebih tinggi dibandingkan dengan kekurangan gizi pada anak dengan kelainan jantung kongenital. FTT ditemukan lebih banyak pada pasien dengan lesi asianotik. Pada lesi asianotik, berat badan lebih dipengaruhi daripada panjang badan. Pada lesi asianotik, berat badan lebih dipengaruhi daripada panjang badan.

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AbstractBackground: Undernutrition is a common cause of morbidity in children with CHD. Previous data from developing country showed prevalence of preoperative undernutrition in children with CHD was up to 45%. The aim of this study are to determine the anthropometric profi les and prevalence of undernutrition in children with CHD by using the anthropometric measurement.Methods: A cross-sectional study was carried out in children aged 0-2 years old with CHD in Cipto Mangunkusumo hospital. All patients underwent an anthropometric evaluation (weight, length and head circumference) at presentation. Undernutrition, failure to thrive /FTT, short stature and microcephaly were determined according to WHO, weight-forlength, weight-for-age at 2 points, length-for-age, head circumference-for-age z-score < -2SD accordingly.Results: We had total of 95 patients, 73 patients with acyanotic and 22 patients with cyanotic lesions. Prevalence of undernutrition in CHD was 51.1%, with 22.3% severe undernutrition. FTT was found in 64.9%, short stature in 49.5% and microcephaly in 37% patients. FTT was found higher in acyanotic (72.2%) compared to cyanotic lesions (42.9%). In acyanotic, weight was affected more than length (72.2% vs 49.3%). In cyanotic, weight and length affected equally (42.9% vs 54.5%). Diet counseling were done in patients with undernutrition. Medicines, transcatheter or surgery intervention were indicated in selected patients.Conclusions: Prevalence of FTT was higher than undernutrition in children with CHD. FTT was found higher in acyanotic lesions. In acyanotic, weight was affected more than length. In cyanotic, weight and length affected equally. "