Outcomes of Acute Liver Injury in Adults Due to Wilson's Disease: Is Survival Without Transplant Possible?
Outcomes of Acute Liver Injury in Adults Due to Wilson's Disease: Is Survival Without Transplant Possible?
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DOI：
10.1002/lt.25703
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发表时间：
2020-03
期刊：
影响因子：
--
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中科院分区：
文献类型：
--
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肝豆状核变性（WD）是一种罕见的急性肝衰竭（ALF）的病因，通常认为不进行肝移植（LT）就会导致死亡。以前的研究提出了WD-ALF的诊断和预后标准。目前尚不清楚这些是否适用于表现为严重急性肝损伤（ALI）而无脑病的WD患者。从2008年到2018年，美国急性肝衰竭研究组（ALFSG）登记的822例ALI患者入组并进行前瞻性随访。8例患者确诊为WD-ALI。测定这些患者血清生化诊断WD-ALF（碱性磷酸酶[ALP]：总胆红素（TB）和天冬氨酸转氨酶[AST]：丙氨酸转氨酶[ALT]）的比率，并评估WD-ALI的预后预测因素。在这8例ALI-WD患者中，5例接受了LT治疗。4例LT患者入院时ALP:TB胆红素的比值<4，AST:ALT的比值为bbb2.2。所有LT患者均为女性。肝移植患者入院时终末期肝病模型评分普遍较高。所有移植患者的初始修正WD评分为>11（>10预测WD- alf无LT的不良预后），而在非LT患者中，2例评分为9,1例评分为13。同时，3例LT患者开始螯合治疗，2例开始血浆置换治疗，1例开始分子吸附剂再循环系统治疗。所有非lt患者均接受螯合治疗。在第21天，所有患者都存活并出院。总之，一些由WD引起的ALI患者可以在没有lt的情况下存活。修订后的Wilson指数评分bbbb10预测大多数WD-ALI患者的预后较差，与WD- alf一样，并且在本队列中，它们与ALI模型呈正相关。与WD- ali相比，诊断WD的生化比值更适用于ALF。
Wilson’s disease (WD) is a rare cause of acute liver failure (ALF) that is thought to have a uniformly fatal outcome without liver transplantation (LT). Previous studies proposed diagnostic and prognostic criteria for WD-ALF. It is not known whether these apply to WD patients presenting as severe acute liver injury (ALI) without encephalopathy. From 2008 to 2018, 822 patients with ALI in the US Acute Liver Failure Study Group (ALFSG) registry were enrolled and prospectively followed. The diagnosis of WD-ALI was confirmed in 8 patients. Serum biochemical diagnostic ratios predicting WD-ALF (alkaline phosphatase [ALP]: total bilirubin (TB) and aspartate aminotransferase [AST]:alanine aminotransferase [ALT]) were determined in these patients, and predictors of prognosis for WD-ALI were evaluated. Of these 8 ALI-WD patients, 5 received an LT. Ratios of both ALP:TB bilirubin of <4 and AST:ALT of >2.2 on study admission were met in 4 LT patients. All LT patients were female. The Model for End-Stage Liver Disease scores on admission were generally higher in LT patients. All transplanted patients had an initial revised WD score of >11 (>10 predicting poor outcome without LT in WD-ALF), whereas in non-LT patients, 2 had scores of 9, and 1 a score of 13. Also, 3 LT patients were started on chelation therapy, 2 were started on plasmapheresis, and 1 was started on Molecular Adsorbent Recirculating System therapy. All non-LT patients were treated with chelation. At 21 days, all patients were alive and discharged from the hospital. In conclusion, some patients with ALI due to WD may survive without LT. Revised Wilson index scores >10 predict poor outcome in most patients with WD-ALI, as they do for WD-ALF, and they correlate positively with the ALI model in this cohort. Biochemical ratios for WD diagnosis appear more applicable to ALF compared with WD-ALI.
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影响因子：
6.7
作者：
Ferenci, P;Caca, K;Berr, F
通讯作者：
Berr, F
影响因子：
5.1
作者：
SOKOL, RJ;FRANCIS, PD;AMBRUSO, DR
通讯作者：
AMBRUSO, DR
影响因子：
39.2
作者：
Reuben A;Tillman H;Fontana RJ;Davern T;McGuire B;Stravitz RT;Durkalski V;Larson AM;Liou I;Fix O;Schilsky M;McCashland T;Hay JE;Murray N;Shaikh OS;Ganger D;Zaman A;Han SB;Chung RT;Smith A;Brown R;Crippin J;Harrison ME;Koch D;Munoz S;Reddy KR;Rossaro L;Satyanarayana R;Hassanein T;Hanje AJ;Olson J;Subramanian R;Karvellas C;Hameed B;Sherker AH;Robuck P;Lee WM
通讯作者：
Lee WM
影响因子：
13.5
作者：
Kamath, PS;Wiesner, RH;Kim, WR
通讯作者：
Kim, WR
DOI：
10.1002/hep.22446
发表时间：
2008-10
期刊：
Hepatology (Baltimore, Md.)
影响因子：
--
作者：
Korman JD;Volenberg I;Balko J;Webster J;Schiodt FV;Squires RH Jr;Fontana RJ;Lee WM;Schilsky ML;Pediatric and Adult Acute Liver Failure Study Groups
通讯作者：
Pediatric and Adult Acute Liver Failure Study Groups