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首页> 外文期刊>Pediatric critical care medicine: a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies >Fulminant Wilson's disease in children: appraisal of a critical diagnosis.
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Fulminant Wilson's disease in children: appraisal of a critical diagnosis.

机译：儿童威尔逊性暴发性疾病：一项重要诊断的评估。

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OBJECTIVES: To characterize fulminant Wilson's disease in children, outline clinical and biochemical differences to fulminant hepatic failure of other etiologies, and compare sensitivity and specificity in diagnosing fulminant Wilson's disease of alkaline phosphatase to total bilirubin ratio and aspartate aminotransferase to alanine aminotransferase ratio. DESIGN: Retrospective case study. SETTING: Twenty-two-bed multidisciplinary pediatric intensive care unit in a tertiary university hospital, national referral center for pediatric liver transplantation. PATIENTS: Between March 1986 and December 1999, seven children, aged between nine and 16 yrs, were admitted in our unit for fulminant Wilson's disease. During the same period of time, aged-matched analysis identified 12 other children with fulminant hepatic failure of other etiologies. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: On admission, pediatric risk of mortality score, coagulation variables, and hepatic encephalopathy grade were similar in both groups. Children with fulminant Wilson's disease (n = 7) were characterized by lowered hemoglobin and serum transaminases, elevated serum bilirubin, and the occurrence of Kayser-Fleischer rings. Although some admission variables were significantly different between both groups, the elevated variability did not allow us to define acceptable cutoff values. Receiver operating characteristics curve for alkaline phosphatase to total bilirubin ratio showed a good discriminative power (Az = 0.87) in differentiating fulminant Wilson's disease from fulminant hepatic failure of other causes, and a ratio <1 showed a 86% sensitivity and 50% specificity (odds ratio, 6; 95% confidence interval, 66-0.5; p =.173) for fulminant Wilson's disease diagnosis. CONCLUSIONS: Although requiring prospective study to confirm, Kayser-Fleischer rings and serum alkaline phosphatase to total bilirubin ratio may assist in the early diagnosis of fulminant Wilson's disease.
机译：目的：鉴定儿童暴发性威尔逊病的特征，概述其他病因导致暴发性肝衰竭的临床和生化差异，并比较诊断碱性磷酸酶与总胆红素比率和天冬氨酸转氨酶与丙氨酸转氨酶比率的暴发性威尔逊病的敏感性和特异性。设计：回顾性案例研究。地点：国家级小儿肝移植转诊中心三级大学医院的二十二张床多学科儿科重症监护室。患者：1986年3月至1999年12月之间，有7名年龄在9至16岁之间的儿童因暴发性威尔逊氏病入院。在同一时间段内，年龄匹配分析确定了其他12例患有其他病因的暴发性肝衰竭的儿童。干预措施：无。测量和主要结果：入院时，两组的儿童死亡率，得分，凝血变量和肝性脑病等级均相似。患威尔逊型暴发性疾病（n = 7）的儿童的特征是血红蛋白和血清转氨酶降低，血清胆红素升高以及出现Kayser-Fleischer环。尽管两组之间的某些准入变量存在显着差异，但较高的可变性不允许我们定义可接受的临界值。碱性磷酸酶与总胆红素之比的受体工作特征曲线显示出良好的判别力（Az = 0.87），可将暴发性威尔逊氏病与其他原因的暴发性肝衰竭区分开，比率＜1表明敏感性为86％，特异性为50％（奇数）比率为6; 95％置信区间为66-0.5; p = .173），以诊断暴发性威尔逊氏病。结论：尽管需要前瞻性研究来证实，但Kayser-Fleischer环和血清碱性磷酸酶与总胆红素之比可能有助于早期诊断暴发性威尔逊氏病。

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