Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity Study
Magnetic resonance cholangiopancreatography (MRCP) has not been assessed as a surrogate biomarker in pediatrics. We aimed to determine the inter‐rater reliability, prognostic utility, and construct validity of the modified Majoie endoscopic retrograde cholangiopancreatography classification applied...
| Main Authors: | , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Health/LWW
2020-02-01
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| Series: | Hepatology Communications |
| Online Access: | https://doi.org/10.1002/hep4.1454 |
| _version_ | 1797934552461606912 |
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| author | Kedar Patil Amanda Ricciuto Alaa Alsharief Jehan Al‐Rayahi Afsaneh Amirabadi Peter C. Church Binita M. Kamath Mary‐Louise C. Greer |
| author_facet | Kedar Patil Amanda Ricciuto Alaa Alsharief Jehan Al‐Rayahi Afsaneh Amirabadi Peter C. Church Binita M. Kamath Mary‐Louise C. Greer |
| author_sort | Kedar Patil |
| collection | DOAJ |
| description | Magnetic resonance cholangiopancreatography (MRCP) has not been assessed as a surrogate biomarker in pediatrics. We aimed to determine the inter‐rater reliability, prognostic utility, and construct validity of the modified Majoie endoscopic retrograde cholangiopancreatography classification applied to MRCP in a pediatric primary sclerosing cholangitis (PSC) cohort. This single‐center, retrospective, cohort study included children with PSC undergoing diagnostic MRCP between 2008 and 2016. Six variations of the Majoie classification were examined: 1) intrahepatic duct (IHD) score, 2) extrahepatic duct (EHD) score (representing the worst intrahepatic and extrahepatic regions, respectively), 3) sum IHD‐EHD score, 4) average IHD score, 5) average EHD score, and 6) sum average IHD‐EHD score. Inter‐rater reliability was assessed using weighted kappas and intraclass correlation coefficients (ICCs). Ability to predict time to PSC‐related complications (ascites, esophageal varices, variceal bleed, liver transplant [LT], or cholangiocarcinoma) (primary outcome) and LT (secondary outcome) was assessed with Harrell’s concordance statistic (c‐statistic) and univariate/multivariable survival analysis. Construct validity was further assessed with Spearman correlations. Forty‐five children were included (67% boys; median, 13.6 years). The inter‐rater reliability of MRCP scores was substantial to excellent (kappas/ICCs, 0.78‐0.82). The sum IHD‐EHD score had the best predictive ability for time to PSC complication and LT (c‐statistic, 0.80 and SE, 0.06; and c‐statistic, 0.97 and SE, 0.01, respectively). Higher MRCP scores were independently associated with a higher rate of PSC‐related complications, even after adjusting for the PSC Mayo risk score (hazard ratio, 1.74; 95% confidence interval, 1.14‐2.). MRCP sum scores correlated significantly with METAVIR fibrosis stage, total bilirubin, and platelets (r = 0.42, r = 0.33, r = −0.31, respectively; P < 0.05). Conclusion: An MRCP score incorporating the worst affected intrahepatic and extrahepatic regions is reliable and predicts meaningful outcomes in pediatric PSC. Next steps include prospective validation and responsiveness assessment. |
| first_indexed | 2024-04-10T18:00:47Z |
| format | Article |
| id | doaj.art-a58dfe159cd243279106c2937a928948 |
| institution | Directory Open Access Journal |
| issn | 2471-254X |
| language | English |
| last_indexed | 2024-04-10T18:00:47Z |
| publishDate | 2020-02-01 |
| publisher | Wolters Kluwer Health/LWW |
| record_format | Article |
| series | Hepatology Communications |
| spelling | doaj.art-a58dfe159cd243279106c2937a9289482023-02-02T15:42:16ZengWolters Kluwer Health/LWWHepatology Communications2471-254X2020-02-014220821810.1002/hep4.1454Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity StudyKedar Patil0Amanda Ricciuto1Alaa Alsharief2Jehan Al‐Rayahi3Afsaneh Amirabadi4Peter C. Church5Binita M. Kamath6Mary‐Louise C. Greer7Department of Diagnostic Imaging The Hospital for Sick Children Toronto CanadaDivision of Gastroenterology, Hepatology, and Nutrition The Hospital for Sick Children Toronto CanadaDepartment of Diagnostic Imaging The Hospital for Sick Children Toronto CanadaDepartment of Diagnostic Imaging The Hospital for Sick Children Toronto CanadaDepartment of Diagnostic Imaging The Hospital for Sick Children Toronto CanadaDivision of Gastroenterology, Hepatology, and Nutrition The Hospital for Sick Children Toronto CanadaDivision of Gastroenterology, Hepatology, and Nutrition The Hospital for Sick Children Toronto CanadaDepartment of Diagnostic Imaging The Hospital for Sick Children Toronto CanadaMagnetic resonance cholangiopancreatography (MRCP) has not been assessed as a surrogate biomarker in pediatrics. We aimed to determine the inter‐rater reliability, prognostic utility, and construct validity of the modified Majoie endoscopic retrograde cholangiopancreatography classification applied to MRCP in a pediatric primary sclerosing cholangitis (PSC) cohort. This single‐center, retrospective, cohort study included children with PSC undergoing diagnostic MRCP between 2008 and 2016. Six variations of the Majoie classification were examined: 1) intrahepatic duct (IHD) score, 2) extrahepatic duct (EHD) score (representing the worst intrahepatic and extrahepatic regions, respectively), 3) sum IHD‐EHD score, 4) average IHD score, 5) average EHD score, and 6) sum average IHD‐EHD score. Inter‐rater reliability was assessed using weighted kappas and intraclass correlation coefficients (ICCs). Ability to predict time to PSC‐related complications (ascites, esophageal varices, variceal bleed, liver transplant [LT], or cholangiocarcinoma) (primary outcome) and LT (secondary outcome) was assessed with Harrell’s concordance statistic (c‐statistic) and univariate/multivariable survival analysis. Construct validity was further assessed with Spearman correlations. Forty‐five children were included (67% boys; median, 13.6 years). The inter‐rater reliability of MRCP scores was substantial to excellent (kappas/ICCs, 0.78‐0.82). The sum IHD‐EHD score had the best predictive ability for time to PSC complication and LT (c‐statistic, 0.80 and SE, 0.06; and c‐statistic, 0.97 and SE, 0.01, respectively). Higher MRCP scores were independently associated with a higher rate of PSC‐related complications, even after adjusting for the PSC Mayo risk score (hazard ratio, 1.74; 95% confidence interval, 1.14‐2.). MRCP sum scores correlated significantly with METAVIR fibrosis stage, total bilirubin, and platelets (r = 0.42, r = 0.33, r = −0.31, respectively; P < 0.05). Conclusion: An MRCP score incorporating the worst affected intrahepatic and extrahepatic regions is reliable and predicts meaningful outcomes in pediatric PSC. Next steps include prospective validation and responsiveness assessment.https://doi.org/10.1002/hep4.1454 |
| spellingShingle | Kedar Patil Amanda Ricciuto Alaa Alsharief Jehan Al‐Rayahi Afsaneh Amirabadi Peter C. Church Binita M. Kamath Mary‐Louise C. Greer Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity Study Hepatology Communications |
| title | Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity Study |
| title_full | Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity Study |
| title_fullStr | Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity Study |
| title_full_unstemmed | Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity Study |
| title_short | Magnetic Resonance Cholangiopancreatography Severity Predicts Disease Outcomes in Pediatric Primary Sclerosing Cholangitis: A Reliability and Validity Study |
| title_sort | magnetic resonance cholangiopancreatography severity predicts disease outcomes in pediatric primary sclerosing cholangitis a reliability and validity study |
| url | https://doi.org/10.1002/hep4.1454 |
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