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小儿急性肝衰竭:病因、预后及小儿终末期肝病评分系列的作用。

Pediatric acute liver failure: etiology, outcomes, and the role of serial pediatric end-stage liver disease scores.

作者信息

Rajanayagam Jeremy, Coman David, Cartwright David, Lewindon Peter J

机构信息

Department of Pediatric Gastroenterology, Royal Children's Hospital, Herston, Brisbane, QLD 4006, Australia.

出版信息

Pediatr Transplant. 2013 Jun;17(4):362-8. doi: 10.1111/petr.12083. Epub 2013 Apr 16.

Abstract

To describe etiology, short-term outcomes and prognostic accuracy of serial PELD scores in PALF. Retrospective analysis of children aged ≤16 yr, admitted with PALF under the QLTS, Brisbane, Australia, between 1991 and 2011. PELD-MELD scores were ascertained at three time points (i) admission (ii), meeting PALF criteria, and (iii) peak value. Fifty-four children met criteria for PALF, median age 17 months (1 day-15.6 yr) and median weight 10.2 kg (1.9-57 kg). Etiology was known in 69%: 26% metabolic, 15% infective, 13% drug-induced, 6% autoimmune, and 9% hemophagocytic lymphohistiocytosis. Age <3 months and weight <4.7 kg predicted poor survival in non-transplanted children. Significant independent predictors of poor outcome (death or LT) were peak bilirubin > 220 μm/L and peak INR > 4. Serial PELD-MELD scores were higher in the 17 (32%) transplant recipients (mean: [i] 26.8, [ii] 31.8, [iii] 42.6); highest in the 12 (22%) non-transplanted non-survivors (mean: [i] 31.6, [ii] 37.2, [iii] 45.7) compared with the 25 (46%) transplant-free survivors (mean: [i] 25.3, [ii] 26.0, [iii] 30.3). PELD-MELD thresholds of ≥27 and ≥42 at (ii) meeting PALF criteria and (iii) peak predicted poor outcome (p < 0.001). High peak bilirubin and peak INR predict poor outcome and serial PELD-MELD is superior to single admission PELD-MELD score for predicting poor outcome.

摘要

描述儿童急性肝衰竭(PALF)中连续儿科终末期肝病评分(PELD)的病因、短期预后及预后准确性。对1991年至2011年间澳大利亚布里斯班QLTS下收治的年龄≤16岁的PALF患儿进行回顾性分析。在三个时间点确定PELD-终末期肝病模型(MELD)评分:(i)入院时,(ii)符合PALF标准时,(iii)峰值时。54名儿童符合PALF标准,中位年龄17个月(1天至15.6岁),中位体重10.2千克(1.9至57千克)。69%的病因明确:26%为代谢性,15%为感染性,13%为药物性,6%为自身免疫性,9%为噬血细胞性淋巴组织细胞增生症。年龄<3个月和体重<4.7千克预示未接受移植儿童的生存情况较差。不良结局(死亡或肝移植)的显著独立预测因素为峰值胆红素>220μmol/L和峰值国际标准化比值(INR)>4。17名(32%)移植受者的连续PELD-MELD评分较高(平均值:[i]26.8,[ii]31.8,[iii]42.6);与25名(46%)未接受移植的存活者(平均值:[i]25.3,[ii]26.0,[iii]30.3)相比,12名(22%)未接受移植的非存活者的评分最高(平均值:[i]31.6,[ii]37.2,[iii]45.7)。在(ii)符合PALF标准时和(iii)峰值时,PELD-MELD阈值≥27和≥42预示不良结局(p<0.001)。高峰值胆红素和峰值INR预示不良结局,且连续PELD-MELD在预测不良结局方面优于单次入院时的PELD-MELD评分。

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