Background Biliary atresia (BA) is a chronic fibroinflammatory condition that destroys inflames intra- and extra-hepatic bile ducts, causing fibrosis and cirrhosis. Early identification of significant fibrosis is crucial for risk stratification and outcome prediction. In this study, we aimed to clarify the clinical role of shear wave elastography (SWE) relative to serum fibrosis indices, particularly APRI and FIB-4, in fibrosis assessment and early outcome stratification in children with BA after Kasai portoenterostomy (KPE). Because previous studies have demonstrated the diagnostic value of SWE in BA, the present study focused on whether SWE provides more clinically useful information than serum-based fibrosis scores and whether these markers are associated with early KPE outcomes. Patients and methods This descriptive analytical study included 53 children with BA. Clinical and laboratory data, including liver fibrosis scores such as the aspartate aminotransferase-to-platelet ratio index (APRI) and fibrosis-4 (FIB-4), and shear wave elastography (SWE), were obtained at the time of study enrollment. Liver biopsy findings at presentation were reviewed. Results Liver stiffness measurements by SWE were markedly higher in our patients than in controls (p < 0.001), with mean values of 25.23 ± 17.43 kPa (3.155 ± 1.34 m/s). SWE correlated with APRI (kPa: r = 0.369, p = 0.007; m/s: r = 0.406, p = 0.003) but not with FIB-, suggesting that APRI and SWE may provide complementary information, whereas FIB-4 adds limited value in this pediatric BA cohort. Both SWE and APRI were higher in patients with marked fibrosis in liver biopsy. Regarding the KPE outcome, successful KPE was observed in 41 patients (77.4%), while failure occurred in 12 patients (22.6%). Patients who underwent KPE at age ≥ 90 days reported a higher frequency of KPE failure (p = 0.001) and showed more distorted architecture and a marked degree of fibrosis on liver biopsy (p = 0.009 and 0.021, respectively). Patients with failed KPE had higher APRI and SWE readings (kPa and m/s, respectively) than the successful KPE group (p = 0.034 and < 0.001, respectively). Conclusions In this cohort, SWE provided the clearest non-invasive signal of fibrosis burden and early KPE outcome, while APRI served as a practical adjunctive serum marker, and FIB-4 showed limited clinical value. These findings support a simplified marker hierarchy in post-KPE BA follow-up: prioritize SWE when available, retain APRI as a low-cost complementary marker, and de-emphasize FIB-4 in infants and young children. Early age at KPE predicts a favorable outcome.

Assessment of hepatic fibrosis in children with biliary atresia

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2026年6月16日

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Alaa Magdy Gohar,
Engy Adel Mogahed,
Wessam Elzayat,
Al-Hussien Ahmed Sayed,
Sawsan Okasha

摘要

Background

Biliary atresia (BA) is a chronic fibroinflammatory condition that destroys inflames intra- and extra-hepatic bile ducts, causing fibrosis and cirrhosis. Early identification of significant fibrosis is crucial for risk stratification and outcome prediction. In this study, we aimed to clarify the clinical role of shear wave elastography (SWE) relative to serum fibrosis indices, particularly APRI and FIB-4, in fibrosis assessment and early outcome stratification in children with BA after Kasai portoenterostomy (KPE). Because previous studies have demonstrated the diagnostic value of SWE in BA, the present study focused on whether SWE provides more clinically useful information than serum-based fibrosis scores and whether these markers are associated with early KPE outcomes.

Patients and methods

This descriptive analytical study included 53 children with BA. Clinical and laboratory data, including liver fibrosis scores such as the aspartate aminotransferase-to-platelet ratio index (APRI) and fibrosis-4 (FIB-4), and shear wave elastography (SWE), were obtained at the time of study enrollment. Liver biopsy findings at presentation were reviewed.

Results

Liver stiffness measurements by SWE were markedly higher in our patients than in controls (p < 0.001), with mean values of 25.23 ± 17.43 kPa (3.155 ± 1.34 m/s). SWE correlated with APRI (kPa: r = 0.369, p = 0.007; m/s: r = 0.406, p = 0.003) but not with FIB-, suggesting that APRI and SWE may provide complementary information, whereas FIB-4 adds limited value in this pediatric BA cohort. Both SWE and APRI were higher in patients with marked fibrosis in liver biopsy. Regarding the KPE outcome, successful KPE was observed in 41 patients (77.4%), while failure occurred in 12 patients (22.6%). Patients who underwent KPE at age ≥ 90 days reported a higher frequency of KPE failure (p = 0.001) and showed more distorted architecture and a marked degree of fibrosis on liver biopsy (p = 0.009 and 0.021, respectively). Patients with failed KPE had higher APRI and SWE readings (kPa and m/s, respectively) than the successful KPE group (p = 0.034 and < 0.001, respectively).

Conclusions

In this cohort, SWE provided the clearest non-invasive signal of fibrosis burden and early KPE outcome, while APRI served as a practical adjunctive serum marker, and FIB-4 showed limited clinical value. These findings support a simplified marker hierarchy in post-KPE BA follow-up: prioritize SWE when available, retain APRI as a low-cost complementary marker, and de-emphasize FIB-4 in infants and young children. Early age at KPE predicts a favorable outcome.