Garuda - Garba Rujukan Digital

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

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.

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.

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.

Faktor-Faktor Yang Memengaruhi Pola Konsumsi Pangan Rumah Tangga : (Studi Kasus Desa Sumberejo Kecamatan Wonosalam Kabupaten Jombang) Arief, Sjamsul; Kusmaningtyas, Amiartuti
Civic-Culture : Jurnal Ilmu Pendidikan PKN dan Sosial Budaya Vol. 5 No. 1 (2021): March
Publisher : Penerbit STKIP PGRI Bangkalan

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.31597/ccj.v5i1.585

This study aims to examine, analyze and prove Engel's Law which says that "the smaller a person's income, the greater the portion of that income will be used to meet food consumption. Conversely, the greater a person's income, the smaller the portion of that income will be used to meet food consumption. Furthermore, this study will also analyze the factors that influence household food consumption patterns in accordance with prevailing theories and laws of demand, such as the level of household income, the price of goods, the number of household members and possibly the level of education. This research is a survey research using a questionnaire distributed to respondents (heads of families). The object of this research is the pattern of household food consumption in Sumberejo Village, Wonosalam District, Jombang Regency. The data used are primary data collected through direct interviews using a questionnaire addressed to each household (respondent) selected as the sample in this study. The data analysis used descriptive analysis to explain the real condition of the respondents and correlation regression to measure the influence of the factors on the consumption pattern of the family household. Statistical testing is done to test the validity and reliability of the data so that conclusions can be drawn from the results of the regression analysis. From the analysis and discussion of the factors that influence household food consumption patterns in Sumberejo Village, Wonosalam District, Jombang Regency, it can be concluded as follows: 1) The share of household expenditure on food consumption is still relatively high, and this shows that the level of household welfare the ladder in the village of Sumberejo, Wonosalam district, Jombang district is still low. There is a difference in the share of non-farmer household expenditures which shows a smaller value compared to farmer households, which means that non-farmer households are more prosperous than farmer households, 2) Partially food prices, household income levels and number of household members tang has a significant (significant) effect on household food consumption patterns in the village of Sumberejo, Wonosalam district, Jombang district. On the other hand, the education level factor of the head of the household has an insignificant (insignificant) effect on the pattern of household food consumption in the village of Sumberejo, Wonosalam sub-district, Jombang district, and 3) Together (simultaneously), food prices, household income levels, The number of household members and the education level of the head of the household have a significant (significant) effect on the pattern of household food consumption in the village of Sumberejo, Wonosalam sub-district, Jombang Regency.

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.

Title

Found 17 Documents
Search

Abstract

Abstract

Abstract

Abstract

Abstract

Abstract

Abstract

Title Search

Found 17 Documents Search

Abstract

Abstract

Abstract

Abstract

Abstract

Abstract

Abstract

Title

Found 17 Documents
Search