{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:16:53Z","timestamp":1788221813970,"version":"build-2803163510"},"reference-count":22,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Dubin-Johnson syndrome (DJS) presents during the neonatal period with a phenotype that overlaps with a broad list of causes of neonatal cholestasis (NC), which makes the identification of DJS challenging for clinicians. We conducted a case-controlled study to investigate the utility of urinary coproporphyrins (UCP) I% as a potential diagnostic biomarker.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>We reviewed our database of 533 cases of NC and identified 28 neonates with disease-causing variants in ATP-binding cassette-subfamily C member 2 (ABCC2) gene \u201cCases\u201d (Study period 2008\u20132019). Another 20 neonates with cholestasis due to non-DJS diagnoses were included as \u201ccontrols.\u201d Both groups underwent UCP analysis to measure CP isomer I percentage (%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Serum alanine aminotransferase (ALT) levels were within the normal range in 26 patients (92%) and mildly elevated in 2 patients. ALT levels were significantly lower in neonates with DJS than in NC from other causes (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt; 0.001). The use of normal serum ALT levels to predict DJS among neonates with cholestasis had a sensitivity of 93%, specificity 90%, positive predictive value (PPV) 34%, and negative predictive value (NPV) 99.5%. The median UCPI% was significantly higher in DJS patients [88%, interquartile range (IQR) 1\u2013IQR3, 84.2%\u201392.7%] than in NC from other causes [67%, (IQR1\u2013IQR3, 61%\u201371.5%; Confidence interval 0.18\u20130.28;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt; 0.001)]. The use of UCPI% &gt;80% to predict DJS had a sensitivity, specificity, PPV, and NPV of 100%.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>\n                      Based on the results from our study, we propose sequencing of the\n                      <jats:italic toggle=\"yes\">ABCC2<\/jats:italic>\n                      gene in neonates with normal ALT, presence of cholestasis and UCP1% &gt;80%.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/sjg.sjg_480_22","type":"journal-article","created":{"date-parts":[[2023,2,2]],"date-time":"2023-02-02T09:42:42Z","timestamp":1675330962000},"page":"183-190","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":5,"title":["Urinary coproporphyrins as a diagnostic biomarker of Dubin-Johnson syndrome in neonates: A 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