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Challenges in Managing Portal Hypertension and Fibrosis in a Case of Biliary Atresia Post-Kasai Procedure—Implications for Early Detection and Long-Term Care Jasin, Yayu Dwinita; Prihaningtyas, Rendi Aji; Octariyandra, Syania Mega; Setyoboedi, Bagus; Arief, Sjamsul
Current Internal Medicine Research and Practice Surabaya Journal Vol. 6 No. 1 (2025): CURRENT INTERNAL MEDICINE RESEARCH AND PRACTICE SURABAYA JOURNAL
Publisher : Universitas Airlangga

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.20473/cimrj.v6i1.54321

Abstract

Biliary atresia frequently causes portal hypertension, resulting in significant morbidity and mortality. Elevated portal pressure can be detected as early as during a Kasai procedure. Pediatric portal hypertension is the primary cause of various complications, including variceal hemorrhage, ascites, and extra-hepatic processes. This paper aims to draw attention to the complications and limitations of the Kasai procedure by presenting a case of portal hypertension and fibrosis in an 8-month-old male infant with biliary atresia who underwent the procedure at 3 months. The patient was never completely free of jaundice post-surgery. The jaundice worsened, and the pale-colored stool reappeared two months later. The abdomen was distended, accompanied by ascites, hepatomegaly, splenomegaly, and dilated veins. Edema was present on the lower extremities and the scrotum. The patient was diagnosed with biliary atresia and portal hypertension. Supportive therapy was recommended as a preparatory measure before a liver transplant. However, the parents declined the procedure due to financial constraints. This case suggests that the Kasai procedure may not always be effective. Despite the timely execution of the procedure, liver fibrosis may persist and be associated with portal hypertension. Most patients develop significant fibrosis that progresses to cirrhosis, requiring a liver transplant. To date, biliary atresia remains the primary indication for liver transplant in children, with no alternative medical treatment recognized. This case report highlights the progression of portal hypertension and liver fibrosis following the Kasai procedure for biliary atresia, emphasizing the challenges in early detection, complications management, and the exploration of alternative therapeutic strategies.   Highlights: 1. This study critically reviews the limitations and complications of the Kasai procedure in treating biliary atresia, with particular focus on portal hypertension issues, for which case reports are limited. 2. This study highlights the important need for accessible and effective long-term treatment alternatives by presenting a pediatric case where financial constraints prevented a liver transplant. 3. The data from this study are anticipated to contribute to the advancement of healthcare equity and pediatric liver disease management.
The Correlation between Cardiac and Liver Function in Children with Heart Failure Utamayasa, I Ketut Alit; Wicaksono, Henry; Karyani, Enny; Nastiti, Prima Hari; Setyoboedi, Bagus; Rahman, Mahrus A.
INDONESIAN JOURNAL OF CLINICAL PATHOLOGY AND MEDICAL LABORATORY Vol. 31 No. 3 (2025)
Publisher : Indonesian Association of Clinical Pathologist and Medical laboratory

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.24293/ijcpml.v31i3.2478

Abstract

The liver has a complex dual blood supply, which makes it more resistant to hepatocyte necrosis due to hemodynamic disturbances. Prior hemodynamic data has indicated that an elevation in right atrial pressure in patients with heart failure may result in aberrant cholestatic markers of liver function. This cross-sectional study examined the correlation between ejection fraction and liver function in 85 children (1–18 years) with congenital or acquired heart disease. Echocardiography assessed TAPSE, Tricuspid Regurgitation (TR), E/A ratio, and Left Ventricular Ejection Fraction (LVEF), while liver function was evaluated using total bilirubin and SGPT. Results showed a strong negative correlation between TAPSE and total bilirubin (p=0.001, r= -0.725) and a moderate positive correlation between tricuspid regurgitation and total bilirubin (p=0.001, r=0.548). The E/A ratio, LVEF, and total bilirubin showed weak correlations, while TAPSE and SGPT had a weak negative correlation (p=0.025, r= -0.243). No correlation was found between TR, E/A ratio, LVEF, and SGPT. A strong correlation existed between heart failure severity and total bilirubin (p=0.001, r=0.603), but not with SGPT. These results highlight the correlation between the severity of heart failure and liver impairment by indicating that decreasing right ventricular function correlates to elevated total bilirubin.
Hepatitis B and Hepatocellular Carcinoma: A Case Report and Literature Review Rendi Aji Prihaningtyas; Muhammad Irawan; Qurrota Ayuni Novia Putri; Bagus Setyoboedi; Sjamsul Arief
MEDICINUS Vol. 38 No. 6 (2025): MEDICINUS
Publisher : PT Dexa Medica

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.56951/e6bqh839

Abstract

Introduction: Current data estimates that over 90% of primary liver tumors are hepatocellular carcinomas (HCC), a type of malignant hepatocyte tumor. Viral hepatitis B is particularly associated with HCC. Early detection of chronic hepatitis B is crucial to prevent complications. Case Presentation: A 17-year-old girl presented with a one-month history of abdominal enlargement, jaundice, dark urine, and pale-colored stool. She experienced weight loss and a lack of appetite. Anthropometric assessment indicated severe malnutrition. The abdomen appeared distended with ascites. There was a palpable liver, with a firm consistency and a lumpy surface up to the epigastrium, with a size of 8 x 7 x 6 cm, and accompanied by splenomegaly. Laboratory examination indicated anemia (hemoglobin 9.5 g/dl), white blood cells 12.38 x 10³/μl, platelets 859 x 10³/μl, AST 1,095 U/l, ALT 104 U/l, cholestasis (total bilirubin 66.5 mg/dl and direct bilirubin 4.14 mg/dl), hypoalbuminemia (albumin 2.81 g/dl), LDH 3146 U/l, reactive HBsAg >1,000, AFP >1,000 ng/ml. An abdominal ultrasound revealed hepatomegaly with multiple nodules distributed across nearly all liver segments, and some of which formed a mass measuring 7.9 x 6.4 x 5.8 cm in the right lobe. Additionally, splenomegaly and ascites were observed. CT scan of the abdomen showed masses distributed throughout the hepatic lobe with contrast enhancement in the arterial phase and feeding from hepatic artery, in the venous phase, contrast washout with ascites. These findings support the diagnosis of hepatocellular carcinoma. Conclusion: Children with HBV may have had prior medical visits without being accurately diagnosed, representing missed opportunities for timely HBV detection. There is a critical need for increased awareness and early screening for hepatitis B virus (HBV) infection in children, especially in regions where HBV prevalence is high.
LIVER CANCER ASSOCIATED WITH HEPATITIS B VIRUS INFECTION IN A CHILD: A CASE REPORT Karina Pharamita Dewi; Rendi Aji Prihaningtyas; Bagus Setyoboedi; Sjamsul Arief
Journal of Vocational Health Studies Vol. 9 No. 1 (2025): July 2025 | JOURNAL OF VOCATIONAL HEALTH STUDIES
Publisher : Faculty of Vocational Studies, Universitas Airlangga

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.20473/jvhs.V9.I1.2025.64-69

Abstract

Background: Two-thirds of primary liver tumors in children are malignant. Hepatoblastoma (HBL) accounts for 40-60 percent of pediatric liver tumors and is the most common malignant liver tumor. Hepatocellular Carcinoma (HCC) is the second most common malignancy in children, accounting for approximately 20% of cases. Both malignant liver tumors have similar clinical and imaging features, but different prognosis and treatment. Purpose: This report aims a case of liver cancer related to hepatitis B virus infection in a child. Case analysis: A 15-year-old girl presented with a two-month history of right upper quadrant pain along with abdominal distension, weight loss, back pain, anorexia, nausea, weakness and fatigue for 5 months prior to admission. Physical examination revealed a hard, firm mass with a bumpy surface in the right upper quadrant of the abdomen and splenomegaly. Laboratory test showed an increase in Lactate Dehydrogenase (LDH), Gamma-Glutamyl Transpeptidase (GGT), Alpha Feto Protein (AFP), quantitative Hepatitis B Virus (HBV) DNA, and HBsAg reactive. Abdominal Multi-Slice Computed Tomography (MSCT) revealed malignant solid mass with necrotic component inside, indistinct borders, irregular margins and kissing spleen. Result: The diagnosis of liver cancer in this patient suggested HCC associated with chronic hepatitis B infection. Conclusion: Routine follow-up of all children with chronic hepatitis B infection is crucial because of the risk of developing liver cancer in adolescence and adulthood.
Steroid Therapy on Cholestasis: A Case Report Rendi Aji Prihaningtyas; Bagus Setyoboedi; Sjamsul Arief; Prihaningtyas, Rendi Aji
MEDICINUS Vol. 38 No. 11 (2025): MEDICINUS
Publisher : PT Dexa Medica

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.56951/mp7wkf03

Abstract

Introduction: Cholestasis is a pathological condition associated with immune-mediated processes that interfere with bile flow, thus requiring early diagnosis and prompt treatment to ensure optimal outcomes. The use of steroid therapy in the treatment of cholestasis has not been previously reported. Case: A 2-month and 9-day-old male infant presented with jaundice beginning at one week of age, followed by pale stools and tea-colored urine. Physical examination revealed jaundice, hepatomegaly, and splenomegaly. Laboratory investigations showed elevated liver enzymes: aspartate aminotransferase (AST) 292 U/l, alanine transaminase (ALT) 187 U/l, gamma-glutamyl transferase (GGT) 312.7 U/l, and alkaline phosphatase (ALP) 882 U/l), along with elevated bilirubin level (total bilirubin 7.9 mg/dl and direct bilirubin 6 mg/dl). Cytomegalovirus (CMV) IgG was reactive. A two-phase abdominal ultrasound examination revealed a preprandial gallbladder size of 0.65 x 0.33 x 1.91 cm, a postprandial size of 0.37 x 0.29 x 0.73 cm. Liver biopsy revealed chronic hepatitis and mild fibrosis (F1). The patient was treated with methylprednisolone, which was gradually tapered off. Following steroid therapy, liver enzyme and bilirubin levels normalized, gallbladder size increased, and stool color darkened. Posttreatment gallbladder size showed an increase (preprandial 3.05 x 0.96 x 0.72 cm, postprandial 2.45 x 0.72 x 0.82 cm). Conclusions: Steroids may improve bile flow and prevent further liver damage. Targeting immune mechanisms could represent a promising approach for future cholestasis treatments.
Successful Steroid Treatment of Extrahepatic Cholestasis: A Case Report Setyoboedi, Bagus; Prihaningtyas, Rendi Aji; Irawan, Muhammad; Octariyandra, Syania Mega; Arief, Sjamsul
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 8 No. 4 (2024): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v8i4.963

Abstract

Background: Biliary atresia is the most common cause of cholestasis. However, not all healthcare facilities are capable of performing Kasai surgery and liver transplantation. The involvement of inflammatory processes in the bile ducts triggered by viral infections has been suggested in several theories of pathogenesis. This case report describes the successful steroid treatment of an infant with extrahepatic cholestasis. Case presentation: A girl aged 2 months and 20 days presented with complaints of jaundice since the age of 2 weeks, with no improvement and worsening of the jaundice, with the color of the stools becoming paler than before. The nutritional status is normal. The physical examination revealed icteric sclera, hepatomegaly, and splenomegaly. There was cholestasis (total bilirubin 7.30 mg/dL and direct bilirubin 5.75 mg/dL), as well as elevated levels of AST (249 U/L), ALT (251 U/L), GGT (995.7 U/L) and increased to 1529.6 U/L, CMV reactive IgG 28.9, and Rubella reactive IgG 6.90. A two-phase ultrasound of the abdomen showed a thickening of the gallbladder wall. A liver biopsy showed mild portal fibrosis (F1). Steroids at a dose of 2 mg/kg/day in combination with ursodeoxycholic acid were administered. At follow-up one month later, the jaundice had improved. Stools were yellow-brown, and liver function tests and bilirubin were normal. Conclusion: Adjunctive steroid therapy to suppress the inflammatory process in biliary obstruction may be beneficial in the early phase of the disease, especially in limited surgical and transplant settings.
Steroid therapy in extrahepatic cholestasis suggestive of biliary atresia: A case report Syahbani, Primadita; Prihaningtyas, Rendi Aji; Setyoboedi, Bagus; Arief, Sjamsul
Bioscientia Medicina : Journal of Biomedicine and Translational Research Vol. 8 No. 9 (2024): Bioscientia Medicina: Journal of Biomedicine & Translational Research
Publisher : HM Publisher

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.37275/bsm.v8i9.1042

Abstract

Background: Biliary atresia is a fibroinflammatory disease obstructing the extrahepatic biliary system. Biliary atresia is the leading cause of cholestasis in infants and the cause of end-stage liver disease in the first two years of life. Surgical treatment with Kasai portoenterostomy has been performed but has not eliminated the need for liver transplantation. The consideration of adjuvant steroid therapy for suppressing the fibro-inflammatory process in the bile ducts may improve the outcome of extrahepatic cholestasis. Case Presentation: A case of a 2-month 7-day-old boy with a chief complaint of jaundice with suspicion of biliary atresia. Jaundice started 1 week after birth, followed by acholic stools, yellow-brown urine, distended abdomen, hepatomegaly, and visible abdominal veins. Laboratory examination revealed an elevated level of direct bilirubin (cholestasis) in combination with elevated levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), and Gamma-glutamyl transferase (GGT), hypoalbuminemia, and reactive of IgG and IgM CMV. A percutaneous liver biopsy was performed and showed extrahepatic cholestasis with mild fibrosis consistent with biliary atresia. The patient was treated with oral methylprednisolone and ursodeoxycholic acid (UDCA). After 12 weeks of therapy, the patient was free of jaundice and darkening of stool color, followed by a normal bilirubin level. Conclusion: In young infants with extrahepatic cholestasis suggestive of biliary atresia, steroid administration resulted in clinical and laboratory improvement. The involvement of the immune response in the pathogenesis of biliary atresia may suggest new therapeutic targets for biliary atresia, such as steroids for improving the outcome of biliary atresia in young infants.
A CASE REPORT OF SUCCESSFUL STEROID TREATMENT IN INFANT WITH EXTRAHEPATIC CHOLESTASIS Bagus Setyoboedi; Rendi Aji Prihaningtyas; Agrasenfani Hadi; Muhammad Nur Alpi Apriansyah; Sjamsul Arief
Journal of Vocational Health Studies Vol. 9 No. 2 (2025): November 2025 | JOURNAL OF VOCATIONAL HEALTH STUDIES
Publisher : Faculty of Vocational Studies, Universitas Airlangga

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.20473/jvhs.V9.I2.2025.139-145

Abstract

Background: Cholestatic jaundice in infants is a significant healthcare challenge, particularly in regions where access to surgical intervention and liver transplantation is limited. An immunologic mechanism underlies the pathogenesis of biliary atresia leading to fibro-obliteration of the bile ducts. However, the successful management of biliary atresia is often difficult because treatment typically occurs at an advanced stage. Therefore, alternative therapies that can suppress bile duct inflammation are urgently needed. Administering anti-inflammatory drugs such as methylprednisolone to infants in the early stages of cholestasis may provide opportunities to improve outcomes in the limited capacity to perform Kasai surgery and liver transplantation. Purpose: This case report describes the clinical improvement of extrahepatic cholestasis following steroid administration. Case analysis: We report a case of a 24-day-old male infant presenting with clinical symptoms of jaundice and pale stool. Liver biopsy revealed features consistent with extrahepatic obstructive cholestasis characteristic of biliary atresia. The patient was treated with methylprednisolone (a corticosteroid) and ursodeoxycholic acid without surgical intervention. Result: The combination of methylprednisolone and ursodeoxycholic acid normalized liver function tests and led to significant clinical improvement. Both jaundice and pale stools completely resolved within two months of treatment. Conclusion: Steroid therapy may provide clinical benefits for infants with extrahepatic cholestasis, particularly in settings with limited healthcare resources. Steroid administration may play a role in the suppression of the inflammatory process that causes fibrosis and bile duct obliteration in the early stages of the disease.
Outcome and Treatment of Hepatic Epithelioid Hemangioendotheliomas in Child: A Rare Case Report Setyoboedi, Bagus; Prihaningtyas, Rendi Aji; Arief, Sjamsul
Journal of Community Medicine and Public Health Research Vol. 6 No. 2 (2025): Journal Community Medicine and Public Health Research
Publisher : Universitas Airlangga

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.20473/jcmphr.v6i2.47474

Abstract

Hepatic tumors in children are relatively rare, accounting for 0.5- 2% of all childhood tumors. Hepatic epithelioid haemangioendothelioma (HEH) is a rare vascular tumour. HEH may appear clinically with nonspecific abdominal symptoms. A 9- year-9-month-old boy presented to the paediatric outpatient clinic with sudden right upper abdominal pain, weight loss, loss of appetite, and weakness. Initial physical examination revealed hepatomegaly. Laboratory tests showed anemia and thrombocytopenia. Meanwhile, liver function tests, serum bilirubin, and serum alpha-fetoprotein (AFP) were within normal limits. Abdominal ultrasound showed hepatomegaly with multiple liver nodules involving both lobes, with a maximum diameter of 4.3 cm and hypoechoic lesions. An MSCT scan of the abdomen revealed numerous solid lesions, and the largest of which measured approximately 3 x 4 x 3.5 cm. These lesions were spread across both lobes and appeared hypodense. These findings are consistent with the hypothesis that HEH of the liver originates from both lobes. Supportive therapy with packed red cell transfusion and supplemental oxygen was started. Steroid therapy using prednisolone orally was started. The patient was following up after 2 weeks of treatment and revealed improvement in clinical condition and laboratory. Hepatic epithelioid hemangioendothelioma should be considered when round, multifocal lesions infiltrating the liver are present, and confirmed by a physician and radiologist. Long-term outcomes are uncertain; therefore, early detection, therapy, and appropriate follow-up are needed.
Maternal Mid-Upper Arm Circumference as a Screening Tool to Predict Infant Birth Weight Fathurrachman, Muhammad Rais; Umijati, Sri; Kurniawati, Eighty Mardiyan; Setyoboedi, Bagus
Folia Medica Indonesiana Vol. 59, No. 4
Publisher : Folia Medica Indonesiana

Show Abstract | Download Original | Original Source | Check in Google Scholar

Abstract

Highlights: 1. This study investigated the correlation between maternal mid-upper arm circumference and infant birth weight, aiming to present a different outcome compared to previous research. 2. The study's findings offer data suggesting that mid-upper arm circumference can serve as a screening tool for predicting infant birth weight. Abstract Mid-upper arm circumference (MUAC) is commonly used for assessing chronic energy deficiency in women of childbearing age. The measurement of MUAC during pregnancy mainly serves as early detection of potential low birth weight. However, certain studies have indicated no significant correlation between maternal MUAC and birth weight. Therefore, this study aimed to determine the relationship between maternal nutritional status and infant birth weight. The study used an analytical observational method with a cross-sectional approach, involving a sample of 86 mothers who delivered at Jagir Primary Healthcare Center in Surabaya, Indonesia, between July and December 2019. The participants were selected based on certain inclusion and exclusion criteria. The data were obtained from secondary sources, specifically the medical records of Jagir Primary Healthcare Center. The data were analyzed using the Spearman test, with a 95% confidence interval and a 5% margin of error. Most mothers (86.05%) had good nutritional status, as indicated by a MUAC measurement of ≥23.5 cm. Only 3.49% of infants were born with a low birth weight (<2,500 g), while 1.16% of infants were considered macrosomia (>4,000 g). Although most mothers exhibiting low MUAC did not give birth to infants with low birth weights, the analysis revealed a significant relationship (p=0.035) between maternal MUAC and infant birth weight. In conclusion, maternal MUAC can be utilized as a screening tool to predict infant birth weight because it indicates the condition of muscle tissue and subcutaneous fat, which serve as the mother's energy reserves. However, several variables can also impact infant birth weight, including maternal nutrient intake.
Co-Authors Agrasenfani Hadi Akbas, Ahmad Maulana Ifan Amilia krisdiantini Anggi Sepfana Zizilia Anindya Kusuma Winahyu Annisya Dinda Paramitha Arvelina Novia Damayanti Budiono Budiono Cindy Wahyu Agustina Eighty Mardiyan K, Eighty Enny Karyani, Enny Evi Rokhayati Fadilla, Anisa Yuniar Farahdina Farahdina Farida Triani Fathurrachman, Muhammad Rais Firyal Nadiah Rahmah Gina Noor Djalilah Gondo Mastutik Henry Wicaksono, Henry IDG Ugrasena Ilya Krisnana, Ilya Irawan, Muhammad Irwanto Irwanto Irwanto, Irwanto Jasin, Yayu Dwinita Karina Pharamita Dewi Kuntoro Kuntoro Lasmauli Situmorang Linda Dewanti Mahrus A Rahman, Mahrus A Manika Putri Kunigara Maretha Sukmawardani, Maretha Martono Tri Utomo Martono Tri Utomo Masturina, Melinda Maytasya Dwinaqifah Melantika Nur Fitria Syahri Melinda Masturina Muhammad Irawan Muhammad Nur Alpi Apriansyah Muhammad Rais Fathurrachman Mukarromah, Nur Nabilah Khansa Nastiti, Prima Hari Nesa, Ni Nyoman Metriani Ni Nyoman Metriani Nesa Ningrum, Astika Gita Nisak Luvi Mega Irawati Nur Mukarromah Octariyandra, Syania Mega Paramitha, Annisya Dinda Pratiwi, Fauziah Prihaningtyas, Rendi Aji Puspa Wardhani Qurrota Ayuni Novia Putri Rachmat Hargono Rahma Fadila, Zakiya Nur Rani Sidaryanti Rendi Aji Prihaningtyas Rendi Aji Prihaningtyas Rendi Aji Prihaningtyas Rendi Aji Prihaningtyas Reny Widayanti Samisatun Maulina, Siti Samsriyaningsih Handayani Situmorang, Lasmauli Sjamsul Arief Sjamsul Arief Sjamsul Arief Sjamsul Arief Sjamsul Arief Sjamsul Arief Sjamsul Arief Sjamsul Arief, Sjamsul Sri Umijati Sulistiawati Sulistiawati Sulistiawati Sulistiawati Syahbani, Primadita Teddy Ontoseno Tiyas Kusumaningrum Ulfa Kholili Utamayasa, I Ketut Alit Viky Nafi&#039;ah Rahma Maulidia Viky Nafi'ah Rahma Maulidia Widati Fatmaningrum Winahyu, Anindya Kusuma Woro Setia Ningtyas Yovani Savitri Yovani Savitri Zizilia, Anggi Sepfana