Rajanayagam Jeremy, Coman David, Cartwright David, Lewindon Peter J
Department of Pediatric Gastroenterology, Royal Children's Hospital, Herston, Brisbane, QLD 4006, Australia.
Pediatr Transplant. 2013 Jun;17(4):362-8. doi: 10.1111/petr.12083. Epub 2013 Apr 16.
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.
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。
