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Liver international : official journal of the International Association for the Study of the Liver · 2018

Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd-Chiari syndrome in children.

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Singh Sumit K, Sen Sarma Moinak, Yadav Rajanikant, Kumar Sheo, Prasad Raghunandan, Yachha Surender K, Srivastava Anshu, Poddar Ujjal

Аннотация

BACKGROUND AND AIM: Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children.

METHODS: Chronic BCS children were analysed in four subgroups: (i) SI: successful intervention (primary or secondary stent patency) (ii) PO: poor outcome (refractory stent block or requirement of liver transplantation), (iii) NU: naïve unintervened (awaiting RI) and (iv) DBI: died before intervention. PSS analysed included Paediatric end-stage liver disease (PELD), Rotterdam, BCS-Transjuglar intrahepatic Portosystemic shunt (BCS-TIPS) index, Zeitoun, Child-Pugh and Model for end-stage liver disease.

RESULTS: Of 113 BCS children, 48 children underwent 53 successful primary RI. Actuarial probability of vascular patency was 87% at 1 year and 82% at 5 years follow-up. Four groups (SI: n = 40, PO: n = 7, NU: n = 13, DBI: n = 6) were analysed. Univariate analysis showed pre-intervention PELD score [PO: 11 (-1-23) vs SI: 2 (-8-25), P = .009] with a cut-off of 4 (AUC: 0.809, 86% sensitivity, 75% specificity) determined PO following intervention. In unintervened group (NU vs DBI), multivariate analysis demonstrated that Zeitoun score predicted death independently (OR 15.4, 95% CI: 1.17-203.56, P = .04) with a cut-off of 4.3 (AUC: 0.923, 83% sensitivity and 77% specificity).

CONCLUSIONS: Children with BCS have a favourable long-term outcome. Among those undergoing RI, pre-intervention PELD score determines the outcome. Survival is determined by Zeitoun score in those unintervened.

Предметные рубрики MeSH

ИП Урсолов Владлен Сергеевич

ИНН 500118482352 · ОГРНИП 314500117700049

Реквизиты и контакты