{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T06:24:25Z","timestamp":1786947865374,"version":"build-2736575974"},"reference-count":13,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T00:00:00Z","timestamp":1786924800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Crigler\u2013Najjar syndrome type II (CNS-II) is a rare inherited disorder of bilirubin conjugation that typically responds to phenobarbital and is less frequently complicated by bilirubin neurotoxicity than CNS-I. During physiologic stress, unconjugated bilirubin may acutely rise, potentially increasing unbound bilirubin and neurologic risk.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A 15-year-old male with a known history of CNS-II presented with two weeks of progressive abnormal movements and worsening jaundice, followed by decreased consciousness and incoherent speech. Initial testing demonstrated severe isolated hyperbilirubinemia with minimal direct bilirubin fraction and no laboratory evidence of hepatocellular injury or synthetic dysfunction. He was transferred to the medical Intensive Care Unit with suspected bilirubin-induced neurologic dysfunction.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Intervention<\/jats:title>\n                    <jats:p>Given ongoing neurologic deterioration and severe hyperbilirubinemia despite supportive management, therapeutic plasma exchange (TPE) was initiated on the day of ICU admission and performed daily for seven sessions via central venous access with fresh frozen plasma used as the replacement fluid.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Outcome<\/jats:title>\n                    <jats:p>Total bilirubin declined from 35.0\u2005mg\/dL on admission (16-Feb-2025) to 28.8\u2005mg\/dL by session 2 (17-Feb-2025),to 27.4\u2005mg\/dL by session 3 (18-Feb-2025), to 24\u2005mg\/dL by sessions 4-5 (19-20-Feb-2025),to 19.5\u2005mg\/dL by sessions 6-7(21-22-Feb-2025) and approximately 18.8\u2005mg\/dL after seven sessions (23-Feb-2025), accompanied by clinical improvement in alertness and abnormal movements. Brain MRI obtained after TPE sessions demonstrate no Acute Bilirubin Encephalopathy-pattern or basal ganglia involvement.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case supports consideration of TPE as a rescue strategy for severe unconjugated hyperbilirubinemia with neurologic dysfunction in older children with CNS-II when conventional measures are insufficient or impractical, while underscoring the need for standardized reporting of TPE protocols and longer-term neurodevelopmental outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fped.2026.1845433","type":"journal-article","created":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T05:26:30Z","timestamp":1786944390000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Therapeutic plasma exchange for severe unconjugated hyperbilirubinemia with bilirubin-induced neurologic dysfunction in an adolescent with Crigler\u2013Najjar syndrome type II"],"prefix":"10.3389","volume":"14","author":[{"given":"Mohanad","family":"Jaber","sequence":"first","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University","place":["Hebron, Palestine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ammir","family":"Abuzahra","sequence":"additional","affiliation":[{"name":"College of Medicine, Hebron University","place":["Hebron, Palestine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ayah Kamel","family":"Karajat","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University","place":["Hebron, Palestine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bara","family":"Abu Shama'a","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University","place":["Hebron, Palestine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lama K.","family":"Hunyhen","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University","place":["Hebron, Palestine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lara","family":"Baradia","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University","place":["Hebron, Palestine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Loai","family":"Muhtasib","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University","place":["Hebron, Palestine"]},{"name":"Medical Intensive Care Unit, Al-Ahli Hospital","place":["Hebron, Palestine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashraf","family":"Alzughayyar","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University","place":["Hebron, Palestine"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hasan I. 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