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Published on: October 13, 2018
Biliary Atresia - Clinical Series
Bárbara Neto1, Mariana Borges-Dias1, Eunice Trindade2
1Department of Pediatric Surgery, Centro Hospitalar São João, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.
Insights
Biliary atresia treatment in children shows improved surgical success and survival rates. Early Kasai portoenterostomy before 72 days and avoiding recurrent cholangitis are key for better prognosis in pediatric liver disease.
Area of Science:
- Pediatric Hepatology
- Surgical Gastroenterology
- Pediatric Liver Transplantation
Background:
- Biliary atresia is a leading cause of pediatric liver failure and the primary indication for liver transplantation.
- This study focuses on patients diagnosed and treated for biliary atresia between 2000 and 2015.
Purpose of the Study:
- To analyze the epidemiological, clinical, biochemical, and imaging data of pediatric patients with biliary atresia.
- To evaluate the outcomes, complications, and survival rates in this patient cohort.
- To identify prognostic factors influencing the disease's progression and treatment success.
Main Methods:
- A descriptive, observational, and retrospective study design was employed.
- Data collected included patient demographics, clinical presentation, biochemical markers, imaging results, and treatment outcomes.
- Analysis focused on patients diagnosed and treated between January 1, 2000, and December 31, 2015.
Main Results:
- Eighteen patients were evaluated, with a median age of 63 days at Kasai portoenterostomy.
- Surgical success was achieved in two-thirds of cases, with better prognosis for those operated on before 72 days.
- Survival rates with native liver were 77.8% at 1 year, 72.2% at 5 years, and 64.2% at 10 years; recurrent cholangitis was associated with survival.
Conclusions:
- The study observed higher surgical success and long-term survival rates compared to many published series.
- Age at surgery and recurrent cholangitis were identified as significant prognostic factors.
- Despite a small patient cohort, outcomes align with those reported by major reference centers.
Introduction:
Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João.
Material And Methods:
Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status.
Results:
Eighteen patients were evaluated. The median age at time of Kasai portoenterostomy was 63 days of life, with better prognosis for those patients who had surgery before 72 days. The procedure was successful in 2/3 of cases. There was a significant association between recurrent cholangitis and survival. Five cases of transplant and 2 deaths, one of them after transplant, were registered. Survival with native liver was 77.8%, 72.2%, and 64.2% at 1, 5, and 10 years of follow-up, respectively.
Discussion:
The presentation and evolution of patients was similar to other studies. However, there was a higher surgical success and survival rates at 5 and 10 years of follow-up than most series. Age at surgery and recurrence of cholangitis were the only factors significantly related to prognosis.
Conclusion:
In spite of the low number of patients (1,125/year), our results were similar to those of other reference centers.
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