Care-Pathway Timeliness in Biliary Atresia and Its Association With Clinical and Liver Histopathologic Outcomes: A Retrospective Cohort Study From Indonesia

Abstract Background: Biliary atresia (BA) requires timely diagnosis and Kasai portoenterostomy (KPE). We mapped care-pathway timeliness and examined unadjusted associations with in-hospital outcome and liver histopathology. Methods: This retrospective cohort included children aged <6 years with BA undergoing KPE and liver biopsy at a tertiary referral hospital in Bandung, West Java, Indonesia, during 2021–2025. Age >60 days at each care stage defined an age-based indicator, without attributing provider-specific waiting time. Outcomes were documented death during the index KPE hospitalization or discharge alive, and histopathology categorized as normal, fibrosis, or cirrhosis. Analyses were unadjusted. Results: Of 86 identified patients, 26 did not undergo KPE and were excluded; 60 were analyzed. Median ages at diagnosis and KPE were 129.5 and 175.0 days. The proportions exceeding 60 days were 23.3% at primary-care presentation, 65.0% at pediatrician assessment, 78.3% at subspecialist assessment, 81.7% at surgical assessment, and 96.7% at KPE. Documented death occurred in 33/60 (55.0%) and cirrhosis in 35/60 (58.3%). Median KPE age was 194.0 days among children who died versus 154.0 days among those discharged alive (Hodges–Lehmann estimate 47.0 days, 95% CI 3.0–91.0). At subspecialist referral, cirrhosis occurred in 32/47 (68.1%) children aged >60 days versus 3/13 (23.1%) aged ≤60 days. Conclusions: The proportion exceeding 60 days increased across care stages. Older KPE age was associated with documented death; later-stage age indicators were associated with more severe histopathology. These exploratory associations warrant further referral-process evaluation.

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Publication Details

Journal
Research Square
Published
2026-11-01
DOI
https://doi.org/10.21203/rs.3.rs-11212816/v1
Primary Topic
Pediatric Hepatobiliary Diseases and Treatments
Type
preprint

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preprint

Care-Pathway Timeliness in Biliary Atresia and Its Association With Clinical and Liver Histopathologic Outcomes: A Retrospective Cohort Study From Indonesia

Sri Endah Rahayuningsih, Dikki Drajat Kusmayadi, Yudith Setiati Ermaya, Nur Suryawan et al.
Research Square
Pediatric Hepatobiliary Diseases and Treatments
preprint

Care-Pathway Timeliness in Biliary Atresia and Its Association With Clinical and Liver Histopathologic Outcomes: A Retrospective Cohort Study From Indonesia

Sri Endah Rahayuningsih, Dikki Drajat Kusmayadi, Yudith Setiati Ermaya, Nur Suryawan, Gartika Sapartini, Dwi Prasetyo, Muhammad Ibrahim Sugiyono
preprint en

Abstract

Abstract Background: Biliary atresia (BA) requires timely diagnosis and Kasai portoenterostomy (KPE). We mapped care-pathway timeliness and examined unadjusted associations with in-hospital outcome and liver histopathology. Methods: This retrospective cohort included children aged <6 years with BA undergoing KPE and liver biopsy at a tertiary referral hospital in Bandung, West Java, Indonesia, during 2021–2025. Age >60 days at each care stage defined an age-based indicator, without attributing provider-specific waiting time. Outcomes were documented death during the index KPE hospitalization or discharge alive, and histopathology categorized as normal, fibrosis, or cirrhosis. Analyses were unadjusted. Results: Of 86 identified patients, 26 did not undergo KPE and were excluded; 60 were analyzed. Median ages at diagnosis and KPE were 129.5 and 175.0 days. The proportions exceeding 60 days were 23.3% at primary-care presentation, 65.0% at pediatrician assessment, 78.3% at subspecialist assessment, 81.7% at surgical assessment, and 96.7% at KPE. Documented death occurred in 33/60 (55.0%) and cirrhosis in 35/60 (58.3%). Median KPE age was 194.0 days among children who died versus 154.0 days among those discharged alive (Hodges–Lehmann estimate 47.0 days, 95% CI 3.0–91.0). At subspecialist referral, cirrhosis occurred in 32/47 (68.1%) children aged >60 days versus 3/13 (23.1%) aged ≤60 days. Conclusions: The proportion exceeding 60 days increased across care stages. Older KPE age was associated with documented death; later-stage age indicators were associated with more severe histopathology. These exploratory associations warrant further referral-process evaluation.

Research Square
Padjadjaran University (ID)
Lembaga Pengelola Dana Pendidikan, Universitas Padjadjaran
Good health and well-being
Pediatric Hepatobiliary Diseases and Treatments
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