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All Journal SAINS MEDIKA : JURNAL KEDOKTERAN DAN KESEHATAN Jurnal Profesi Medika: Jurnal Kedokteran dan Kesehatan Bioscientia Medicina : Journal of Biomedicine and Translational Research JOURNAL OF COMMUNITY MEDICINE AND PUBLIC HEALTH RESEARCH CURRENT INTERNAL MEDICINE RESEARCH AND PRACTICE SURABAYA JOURNAL INDONESIAN MIDWIFERY HEALTH AND SCIENCES JOURNAL Bioscientia Medicina : Journal of Biomedicine and Translational Research Caring : Jurnal Pengabdian Masyarakat MEDICINUS
Prihaningtyas, Rendi Aji
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Biochemistry, Genetics & Molecular Biology Education Health Professions Immunology & microbiology Medicine & Pharmacology Neuroscience Nursing Public Health

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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

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.
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.
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.
Co-Authors Akbas, Ahmad Maulana Ifan Ardhanariswari, Zubaity Bagus Setyoboedi Irawan, Muhammad Jasin, Yayu Dwinita Martono Tri Utomo Muhammad Faizi, Muhammad Octariyandra, Syania Mega Pratiwi, Fauziah Rendi Aji Prihaningtyas Rini, Muji Retnaning Situmorang, Lasmauli Sjamsul Arief Sjamsul Arief, Sjamsul Syahbani, Primadita Teddy Ontoseno Winahyu, Anindya Kusuma