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Published on: October 13, 2018
Biliary Atresia: A Case Report
Maliha Muzaffer1, Anees Masarath1, Fareedullah Mohammed1
1Department of Pharmacy, Mesco College of Pharmacy, Hyderabad, IND.
Insights
Biliary atresia (BA) is a severe infant liver disease. Liver transplantation offers a definitive solution for infants with BA who experience Kasai procedure failure.
Area of Science:
- Pediatric Hepatology
- Surgical Gastroenterology
- Infant Liver Disease
Background:
- Biliary atresia (BA) is a life-threatening infant hepatobiliary disorder.
- Early diagnosis and intervention are crucial for improving prognosis in BA.
- Untreated BA leads to liver damage and mortality.
Observation:
- A 9-month-old infant presented with jaundice, acholic feces, and hepatomegaly.
- Diagnostic tests including elevated liver enzymes and HIDA scan confirmed BA.
- Histopathology showed fibrosis, cholestasis, and an atretic gallbladder.
Findings:
- A modified Kasai portoenterostomy (KPE) was performed, but the infant showed persistent jaundice and liver dysfunction.
- The Kasai procedure ultimately failed, indicating end-stage liver disease.
- Liver transplantation (LT) was deemed the necessary definitive treatment.
Implications:
- Liver transplantation is effective for treating BA cases with failed Kasai procedures.
- This case underscores the potential for improved outcomes with LT in infants with end-stage BA.
- Timely LT can significantly enhance the prognosis for infants with severe biliary atresia.
Abstract:
Biliary atresia (BA) is a serious hepatobiliary disorder that occurs due to progressive inflammation and scarring obstruction in the bile ducts, posing a threat to life. This condition usually appears in infants, and timely identification is fundamental for a better prognosis. If left untreated, individuals will inevitably experience liver damage and mortality. This case report describes a nine-month-old female infant presenting with jaundice, icteric sclera, yellowish skin, acholic feces, and hepatomegaly. Elevated liver enzymes and a hepatobiliary iminodiacetic acid (HIDA) scan confirmed BA. Histopathological examination revealed fibrosis, cholestatic disease, and an atretic gallbladder. A modified Kasai portoenterostomy (KPE) with Roux-en-Y jejunojejunostomy was performed, and the infant was discharged with supportive care. However, seven months post-Kasai portoenterostomy, the infant presented with persistent jaundice and progressive deterioration of liver function, indicative of a failed Kasai procedure. Consequently, she was scheduled to undergo liver transplantation (LT) as a definitive treatment. BA is a rare disorder that is observed across nearly all ethnic groups, though the incidence rates vary significantly. This case highlights the efficacy of liver transplantation in treating failed Kasai procedures and demonstrates the potential for enhanced outcomes in infants with end-stage liver disease.

