{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,7]],"date-time":"2026-06-07T13:59:16Z","timestamp":1780840756250,"version":"3.54.1"},"reference-count":24,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:00:00Z","timestamp":1780272000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:00:00Z","timestamp":1780272000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Clin Med Insights Case Rep"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Biliary atresia is a rare, progressive cholangiopathy of infancy and a leading cause of pediatric liver failure. Syndromic forms associated with laterality defects and congenital heart disease are uncommon and pose diagnostic challenges.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>A 3-month-old female infant, born at term via home delivery to a consanguineous couple, presented with persistent jaundice since birth. Examination revealed icterus, anemia, hepatomegaly, and a pansystolic murmur. Laboratory tests showed conjugated hyperbilirubinemia, elevated liver enzymes, and coagulopathy, while infectious, metabolic, and endocrine causes were excluded. Imaging revealed a small gallbladder with a triangular cord sign, biliary obstruction on HIDA scan, and situs inversus. Echocardiography identified a small perimembranous ventricular septal defect. Percutaneous liver biopsy confirmed bile duct proliferation, fibrosis, and cholestasis, consistent with extrahepatic biliary atresia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Diagnostic Assessment<\/jats:title>\n                    <jats:p>Syndromic biliary atresia was diagnosed based on clinical, radiological, and histopathological findings, with associated situs inversus and a congenital heart defect.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Therapeutic Intervention and Outcome<\/jats:title>\n                    <jats:p>The patient received supportive care including vitamin K and nutritional supplementation. Pediatric surgical consultation was obtained; however, in view of advanced fibrosis, the syndromic constellation, and the family\u2019s decision to decline operative intervention after counseling, Kasai portoenterostomy was not performed. Liver transplantation was discussed but declined by the family. The infant\u2019s condition deteriorated and she died approximately one month after diagnosis from complications of advanced liver disease.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Early recognition of biliary atresia and its syndromic associations is crucial. Delayed presentation and limited access to specialized care can adversely affect outcomes, underscoring the need for timely diagnosis, multidisciplinary evaluation, and family counseling.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/11795476261459498","type":"journal-article","created":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T18:59:57Z","timestamp":1780685997000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Extrahepatic Biliary Atresia With Situs Inversus and Ventricular Septal Defect: A Case Report"],"prefix":"10.1177","volume":"19","author":[{"given":"Marium","family":"Khan","sequence":"first","affiliation":[{"name":"Jinnah Sindh Medical University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-6211-7522","authenticated-orcid":false,"given":"Kanza","family":"Farhan","sequence":"additional","affiliation":[{"name":"Jinnah Sindh Medical University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maliha","family":"Rahim","sequence":"additional","affiliation":[{"name":"Jinnah Sindh Medical University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aisha","family":"Farooq","sequence":"additional","affiliation":[{"name":"ER Department, Abbasi Shaheed Hospital, Karachi, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-2930-8188","authenticated-orcid":false,"given":"Ahmed Asad","family":"Raza","sequence":"additional","affiliation":[{"name":"Jinnah Sindh Medical University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-8614-3924","authenticated-orcid":false,"given":"Abedin","family":"Samadi","sequence":"additional","affiliation":[{"name":"Kabul University of Medical Science"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fehmina","family":"Arif","sequence":"additional","affiliation":[{"name":"Dow University of Health Science"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,6,5]]},"reference":[{"key":"e_1_3_8_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.510230652"},{"key":"e_1_3_8_3_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1478-3231.2011.02578.x"},{"key":"e_1_3_8_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(09)60946-6"},{"key":"e_1_3_8_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(03)00178-7"},{"key":"e_1_3_8_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(74)80508-1"},{"key":"e_1_3_8_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/hep.24023"},{"key":"e_1_3_8_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-017-4160-x"},{"key":"e_1_3_8_9_2","doi-asserted-by":"publisher","DOI":"10.4103\/0971-9261.158095"},{"key":"e_1_3_8_10_2","first-page":"36","article-title":"Biliary atresia with rare associations: a case report","volume":"19","author":"Bowal NS","year":"2023","unstructured":"Bowal NS, Patel J, Khan F, et al. Biliary atresia with rare associations: a case report. Ann Pediatr Surg. 2023;19:36.","journal-title":"Ann Pediatr Surg"},{"key":"e_1_3_8_11_2","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics10090686"},{"key":"e_1_3_8_12_2","doi-asserted-by":"publisher","DOI":"10.3390\/ijms23094841"},{"key":"e_1_3_8_13_2","author":"National Institute of Diabetes and Digestive and Kidney Diseases","unstructured":"National Institute of Diabetes and Digestive and Kidney Diseases. Biliary Atresia. https:\/\/www.niddk.nih.gov\/health-information\/liver-disease\/biliary-atresia.","journal-title":"Biliary Atresia"},{"key":"e_1_3_8_14_2","volume-title":"StatPearls. Treasure Island (FL)","author":"Bezerra JA","year":"2024","unstructured":"Bezerra JA, Wells RG, Mack CL, et al. Biliary Atresia. StatPearls. Treasure Island (FL): StatPearls Publishing; 2024. https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK537262\/"},{"key":"e_1_3_8_15_2","doi-asserted-by":"publisher","DOI":"10.13029\/aps.2020.26.2.67"},{"issue":"12","key":"e_1_3_8_16_2","first-page":"1814","article-title":"The incidence of associated anomalies in children with congenital duodenal obstruction\u2014a retrospective cohort study of 112 patients","volume":"9","author":"Pijpers AGH","year":"2022","unstructured":"Pijpers AGH, Eeftinck Schattenkerk LD, Straver B, et al. The incidence of associated anomalies in children with congenital duodenal obstruction\u2014a retrospective cohort study of 112 patients. 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