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Collodion Baby And Cholestasis: A Rare Case Report Setyoboedi, Bagus; Prihaningtyas, Rendi Aji; Octariyandra, Syania Mega
Jurnal Profesi Medika : Jurnal Kedokteran dan Kesehatan Vol 18 No 1 (2024): Jurnal Profesi Medika : Jurnal Kedokteran dan Kesehatan
Publisher : Fakultas Kedokteran UPN Veteran Jakarta Kerja Sama KNPT

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.33533/jpm.v18i1.7392

Abstract

Collodion baby with cholestasis is an uncommon condition. The term "collodion baby" refers to newborns with thick skin covering their entire body. Neonatal cholestasis is a form of conjugated cholestasis hyperbilirubinemia caused by reduced bile flow. The condition causes persistent jaundice (over two weeks) and elevated conjugated bilirubin. Genetic abnormalities or gene mutations have been reported in neonatal ichthyosis syndrome with cholestasis. A case report of a collodion baby, a rare congenital condition characterized by a parchment-like membrane covering the entire body, was brought to Dr. Soetomo General Academic Hospital. Prolonged jaundice was one of the other findings. A skin biopsy found characteristics consistent with the diagnosis of collodion in a newborn. The paediatric team managed this case, which was treated in collaboration with a dermatologist. This case showed that newborn collodion syndrome required skin care, cholestasis screening, and jaundice management from birth.
Socialization of stool color card for early detection of biliary atresia among healthcare professionals in Primary Healthcare facilities in Probolinggo Setyoboedi, Bagus; Prihaningtyas, Rendi Aji; Utomo, Martono Tri; Akbas, Ahmad Maulana Ifan; Octariyandra, Syania Mega; Arief, Sjamsul
Caring: Jurnal Pengabdian Masyarakat Vol 4, No 3 (2024): CARING Jurnal Pengabdian Masyarakat (Desember 2024)
Publisher : Caring: Jurnal Pengabdian Masyarakat

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.21776/ub.caringjpm.2024.004.03.4

Abstract

AbstractBackground: Biliary Atresia (BA) is an idiopathic progressive fibro obliterative disease affecting the extrahepatic biliary duct with clinical manifestations of biliary obstruction in the neonatal period. Infants with BA are characterized with acholic stools which are frequently unidentified due to daily variations in stool color. Early detection is essential to reduce morbidity and mortality rates in infants with BA. Objective: This project aimed to improve the knowledge of health workers in primary health facilities in Probolinggo about BA through socialization of stool color cards as an early detection tool. Methods: Socialization was conducted to midwives in primary health facilities in Probolinggo with pre-test and post-test methods. Results: A total of 35 midwives participated in this activity. The evaluation showed a significant increase in health workers' understanding of biliary atresia. The average pre-test score was 83.82 and increased to 92.18 in the post-test with a p-value=0.00, indicating the effectiveness of counseling in improving health workers' knowledge. In addition, the implementation of the stool color card as an early detection tool received a positive response from the participants, who stated that the tool is easy to use and helps in identifying infants at risk of biliary atresia more accurately. Conclusion: The level of knowledge of health workers in primary health facilities regarding BA is still varied. Socialization of stool color cards may improve the understanding of health workers to accelerate early detection of cholestasis and improve the prognosis of infants with BA.
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.
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.
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.