The Sclerosing Cholangitis Outcomes in Pediatrics (SCOPE) Index: A Prognostic Tool for Children.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Deneau Mark R, Mack Cara, Perito Emily R, Ricciuto Amanda, Valentino Pamela L, Amin Mansi, Amir Achiya Z, Aumar Madeleine, Auth Marcus, Broderick Annemarie, DiGuglielmo Matthew, Draijer Laura G, Tavares Fagundes Eleonora Druve, El-Matary Wael, Ferrari Federica, Furuya Katryn N, Gupta Nitika, Hochberg Jessica T, Homan Matjaz, Horslen Simon, Iorio Raffaele, Jensen M Kyle, Jonas Maureen M, Kamath Binita M, Kerkar Nanda, Kim Kyung Mo, Kolho Kaija-Leena, Koot Bart G P, Laborda Trevor J, Lee Christine K, Loomes Kathleen M, Martinez Mercedes, Miethke Alexander, Miloh Tamir, Mogul Douglas, Mohammad Saeed, Mohan Parvathi, Moroz Stacy, Ovchinsky Nadia, Palle Sirish, Papadopoulou Alexandra, Rao Girish, Rodrigues Ferreira Alexandre, Sathya Pushpa, Schwarz Kathleen B, Shah Uzma, Shteyer Eyal, Singh Ruchi, Smolka Vratislav, Soufi Nisreen, Tanaka Atsushi, Varier Raghu, Vitola Bernadette, Woynarowski Marek, Zerofsky Melissa, Zizzo Andréanne, Guthery Stephen L
Аннотация
APPROACH AND RESULTS: We used retrospective data from the Pediatric PSC Consortium. The training cohort contained 1,012 patients from 40 centers. We generated a multivariate risk index (Sclerosing Cholangitis Outcomes in Pediatrics [SCOPE] index) that contained total bilirubin, albumin, platelet count, gamma glutamyltransferase, and cholangiography to predict a primary outcome of liver transplantation or death (TD) and a broader secondary outcome that included portal hypertensive, biliary, and cancer complications termed hepatobiliary complications (HBCs). The model stratified patients as low, medium, or high risk based on progression to TD at rates of <1%, 3%, and 9% annually and to HBCs at rates of 2%, 6%, and 13% annually, respectively (P < 0.001). C-statistics to discriminate outcomes at 1 and 5 years were 0.95 and 0.82 for TD and 0.80 and 0.76 for HBCs, respectively. Baseline hepatic fibrosis stage was worse with increasing risk score, with extensive fibrosis in 8% of the lowest versus 100% with the highest risk index (P < 0.001). The model was validated in 240 children from 11 additional centers and performed well.
CONCLUSIONS: The SCOPE index is a pediatric-specific prognostic tool for PSC. It uses routinely obtained, objective data to predict a complicated clinical course. It correlates strongly with biopsy-proven liver fibrosis. SCOPE can be used with families for shared decision making on clinical care based on a patient's individual risk, and to account for variable disease progression when designing future clinical trials.