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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

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.

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

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

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.

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

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.

Primary Healthcare Providers' Knowledge on the Early Detection of Biliary Atresia Prihaningtyas, Rendi Aji; Setyoboedi, Bagus; Nesa, Ni Nyoman Metriani; Masturina, Melinda; Utomo, Martono Tri; Arief, Sjamsul
Folia Medica Indonesiana Vol. 60, No. 1
Publisher : Folia Medica Indonesiana

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

Highlights: 1. An evaluation is necessary to verify that the knowledge of healthcare providers is sufficient for the early detection of biliary atresia. 2. It is crucial to promote education on prolonged jaundice to ensure early detection of biliary atresia in primary health facilities, as some primary healthcare providers have insufficient knowledge on the subject. Abstract Primary healthcare providers frequently fail to recognize biliary atresia because it closely mimics physiological jaundice. Early detection plays an important role in ensuring the optimal treatment of biliary atresia. Delays in referring to biliary atresia cases remain a problem around the world. The objective of this study was to analyze the knowledge of primary healthcare providers regarding prolonged jaundice as an early sign of biliary atresia. A cross-sectional study was conducted at primary healthcare centers using a self-administered questionnaire. The data were analyzed descriptively with the assistance of IBM SPSS Statistics for Windows, version 21.0 (IBM Corp., Armonk, N.Y., USA). A total of 271 respondents participated in this study, including midwives (63.8%), nurses (24.4%), and medical doctors (8.5%). This study found that 73.8% of the respondents agreed that infants with prolonged jaundice need to undergo a serum bilirubin level assessment. However, 40.2% of the respondents believed that pale stools and dark urine in yellow infants were symptoms of physiological jaundice. Concerning the definition of prolonged jaundice, 209 (77.1%) respondents provided a response indicating more than two weeks. A total of 137 (50.6%) respondents stated that biliary atresia is a disease that must be ruled out first in cases of prolonged jaundice. Nevertheless, 86 (31.7%) and 87 (32.1%) respondents also mentioned that breastfeeding jaundice and breast milk jaundice must be considered as potential causes to be investigated. These results indicated that primary healthcare providers still had limited knowledge regarding cholestasis. Education on prolonged jaundice in primary healthcare centers should be more improved to enhance the early detection of biliary atresia.

INTERVENSI EDUKASI SINGKAT TENTANG ATRESIA BILIER PADA TENAGA KESEHATAN: BUKTI PENINGKATAN PENGETAHUAN DAN KESIAPAN SKRINING DENGAN KARTU WARNA TINJA Setyoboedi, Bagus; Prihaningtyas, Rendi Aji; Savitri, Yovani; Tri Utomo, Martono; Arief, Sjamsul
Jurnal Abdi Insani Vol 12 No 12 (2025): Jurnal Abdi Insani
Publisher : Universitas Mataram

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.29303/abdiinsani.v12i12.3114

Biliary atresia is the most common cause of neonatal cholestasis, characterized by prolonged jaundice, pale stools, and dark-colored urine. Early detection is crucial to prevent further liver damage. However, delayed diagnosis is still frequently encountered, mainly due to the lack of healthcare providers’ knowledge in recognizing the early signs of biliary atresia. This study aimed to determine the significance of a targeted health education intervention in improving knowledge of early detection of biliary atresia among healthcare providers. A quasi-experimental study was conducted at Padangan District General Hospital, Bojonegoro, East Java, using a pre-test/post-test questionnaire design. The health intervention consisted of education on biliary atresia and the introduction of the stool color card as an early detection tool, delivered by a Pediatric gastroenterologist and hepatologist. The Wilcoxon signed-rank test was performed with a significance level of p<0.05.A total of 101 respondents participated, predominantly female (88.1%), with the largest age group being 30–39 years (37.6%). Most respondents had more than five years of work experience (81.2%). The mean knowledge score significantly increased from 86.3 in the pre-test to 93.2 in the post-test (p < 0.001). Prior to the intervention, only 12.9% of respondents scored <70, whereas after the intervention nearly all participants (98%) scored >70. Knowledge regarding early symptoms of biliary atresia, the risks of delayed diagnosis, and the use of the Stool Color Card showed meaningful improvement on early detection of biliary atresia. A health education intervention is effective in improving healthcare providers’ knowledge of early detection of biliary atresia.

SOSIALISASI DAN PENGENALAN KARTU WARNA TINJA: STRATEGI PENINGKATAN PENGETAHUAN TENAGA KESEHATAN DALAM SKRINING ATRESIA BILIER Utomo, Martono Tri; Prihaningtyas, Rendi Aji; Savitri, Yovani; Setyoboedi, Bagus; Arief, Sjamsul
Jurnal Abdi Insani Vol 12 No 12 (2025): Jurnal Abdi Insani
Publisher : Universitas Mataram

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.29303/abdiinsani.v12i12.3245

Jaundice in newborns is a common condition, but it is important to distinguish between physiological and pathological forms. One of the main causes of pathological jaundice is cholestasis, with biliary atresia being the most frequent etiology. Biliary atresia is progressive and potentially fatal if treatment is delayed. Early detection is a key to successful therapy; however, limited knowledge among healthcare workers and inadequate screening tools contribute to delayed referrals. This study aimed to improve healthcare workers’ understanding of early detection of biliary atresia through education and introduction of stool color cards at RSUD dr. Sayidiman, Magetan. This study employed a quasi-experimental pre-test–post-test design involving 83 healthcare workers. Educational sessions were delivered by a pediatric gastrohepatology specialist, and participants completed questionnaires before (pre-test) and after (post-test) the intervention. Data were analyzed using the Wilcoxon signed-rank test to assess the improvement in knowledge scores, with statistical significance set at p < 0.05. A total of 83 participants took part, predominantly female (94%) with the largest age group being 30–39 years (43.4%). Most participants had more than 5 years of work experience (79.5%) and held a bachelor’s degree (55.4%). The mean knowledge score increased significantly from 90 in the pre-test to 95.3 in the post-test (p < 0.001). Socialization and introduction of stool color cards effectively improve healthcare workers’ knowledge regarding early detection of biliary atresia.

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