In a significant retrospective evaluation, researchers at a leading pediatric tertiary referral center have compiled an extensive review of long-term prognostic indicators for patients with biliary atresia (BA). Over 20 years, they have carefully examined the clinical outcomes of 81 children diagnosed with BA, presenting their findings in May 2019 in The Turkish Journal of Gastroenterology. This comprehensive review, bearing the title “Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center”, offers critical insights into disease management and factors associated with treatment success and patient survival.
Biliary atresia, a rare pediatric liver disease marked by the progressive obliteration of the bile ducts, poses a significant management challenge due to its often insidious and unpredictable progression. Without timely intervention, BA can lead to cirrhosis, liver failure, and a desperate need for liver transplantation.
The study (DOI: 10.5152/tjg.2019.18590) led by Damla Hanalioğlu and a team of experts from Hacettepe University School of Medicine in Ankara, Turkey, examined an extensive patient cohort from 1994 to 2014. Their objective was to assess the clinical pathways and results of cases treated for BA, focusing on determining the prognostic factors crucial for clinical outcomes.
Prognostic Indicators and Longevity: The Essential Findings
Of the 81 patients studied, including 49 males and 32 females, the mean age at diagnosis was approximately 73 days. Remarkably, 96% of the diagnosed individuals underwent a portoenterostomy procedure. The procedure’s timing was critical, with a median of 72 days at operation. The analysis revealed a surgical success rate of 64.8%, and the critical determining factor was the age of diagnosis and ensuing surgical intervention – the younger the age, the greater the chance of a successful outcome.
These observations align with international evidence emphasizing the importance of early detection and intervention in BA management. For instance, an international incidence and outcomes study by Jimenez-Rivera et al. in 2013 suggested similar conclusions (DOI: 10.1097/MPG.0b013e318282a913).
Overall and Native Liver Survival: A Closer Glimpse
Integrating all patients, both with and without liver transplants, the study outlined the 2-, 5-, and 10-year overall survival (OS) rates as 75%, 73%, and 71% respectively. The survival with native liver (SNL) figures were less optimistic, standing at 69%, 61%, and 57% for the same intervals.
The prognosis beyond successful surgery tied closely with several factors. These included the presence and extent of fibrosis or cirrhosis at diagnosis and the prothrombin time, as measured by the international normalized ratio (INR), suggesting the vital role of early liver pathology in guiding therapeutic approaches. This is corroborated by prior research, including the study by Chardot and Serinet who reflected upon the prospects of enhancing BA prognosis (DOI: 10.1016/j.jpeds.2006.01.049).
The Importance of Early Diagnosis
The Turkish study’s identification of a brief gap between diagnosis and surgery highlights the centrality of prompt disease recognition in achieving better outcomes. Such an assertion is backed by the collective knowledge base, including the work by Wildhaber (2012), which reviewed 50 years of advancements post the first Kasai portoenterostomy, recognizing the need for timely surgery (DOI: 10.5402/2012/132089).
Insights into Surgical Success and Implications for Patient Management
The examination of factors such as surgical success, liver pathology, and preoperative metrics such as INR underscores multidimensional considerations for BA treatment. Experts like Lopez et al., in their 2017 review of early and peri-operative prognostic indicators, also emphasized similar aspects in the context of hepatic portoenterostomy (DOI: 10.1007/s11894-017-0555-z).
Implications for Future Patient Care and Research
This study is not only of value for its direct clinical implications but also as a guide for future research. It points out the pressing need for standardized protocols, reinforced by the insights of Serinet et al. from France (DOI: 10.1002/hep.21219). Additionally, outcomes from this research may indeed advocate for refined surgical training, given the technical complexity of the Kasai procedure, as highlighted by various surgeons, such as Subramaniam et al. (DOI: 10.1053/jpsu.2000.0350593).
Conclusion: The Path Ahead
The extensive review conducted by Hanalioğlu and colleagues provides a vibrant, data-driven picture of BA over two decades, emphasizing the determinants of long-term patient prognosis. Their work vitalizes the quest for improved BA management and the formulation of therapeutic strategies anchored in empirical realities. Such diligent scholarly efforts set an enduring precedent for pediatric hepatology and the management of biliary atresia worldwide.
Keywords
1. Biliary Atresia Prognosis
2. Portoenterostomy Success Rate
3. Pediatric Liver Disease Management
4. Early Diagnosis of Biliary Atresia
5. Survival Rates in Biliary Atresia
References
1. Hanalioğlu, D., Özen, H., Karhan, A., Gümüş, E., Demir, H., Saltık-Temizel, İ. N., … & Yüce, A. (2019). Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center. The Turkish Journal of Gastroenterology, 30(5), 467-474. DOI: 10.5152/tjg.2019.18590
2. Jimenez-Rivera, C., Jolin-Dahel, K. S., Fortinsky, K. J., Gozdyra, P., & Benchimol, E. I. (2013). International incidence and outcomes of biliary atresia. Journal of Pediatric Gastroenterology and Nutrition, 56(4), 344-354. DOI: 10.1097/MPG.0b013e318282a913
3. Chardot, C., & Serinet, M. O. (2006). Prognosis of biliary atresia: What can be further improved?. The Journal of Pediatrics, 148(4), 432-435. DOI: 10.1016/j.jpeds.2006.01.049
4. Wildhaber, B. E. (2012). Biliary atresia: 50 years after the first Kasai. ISRN Surgery, 2012. DOI: 10.5402/2012/132089
5. Lopez, R. N., Ooi, C. Y., & Krishnan, U. (2017). Early and peri-operative prognostic indicators in infants undergoing hepatic portoenterostomy for biliary atresia: a review. Current Gastroenterology Reports, 19(3), 16. DOI: 10.1007/s11894-017-0555-z