Verma Snigdha, Alam Seema, Lal Bikrant Bihari, Biswas Tamoghna, Sood Vikrant, Khanna Rajeev, Bajpai Meenu
Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, Delhi, India.
Department of Transfusion Medicine, Institute of Liver and Biliary Sciences, New Delhi, Delhi, India.
Hepatol Int. 2025 May 2. doi: 10.1007/s12072-025-10821-7.
Decision about liver transplant is difficult in Wilson disease (WD) with liver failure, especially with conflicting reports about new Wilson index (NWI). Therapeutic plasma exchange (TPE) can provide survival with native liver (SNL) in WD. This study was done to see the effect of TPE on outcome and identify factors for SNL.
All cases of WD with liver failure (INR. ≥ 2.5) from prospectively maintained data were included for propensity score matching (PSM) to select TPE (n = 48) and no-TPE (n = 48) groups. Three sessions of TPE on three consecutive days were given to TPE group.
One hundred fifty-nine cases were included in the PSM with NWI & hepatic encephalopathy (HE) grading as predictors. SNL was comparable (26 vs. 17 cases (OR 1.45, p = 0.05) when the analysis was done in the whole cohort of 96 patients. SNL significantly improved when performed in those with no to early HE: TPE group (24/37) versus no-TPE group (14/34) (OR = 1.70, p = 0.03). Kaplan-Meier survival curves were significantly (log rank 0.019) improved in the TPE group when analyzing in no to early HE. Lower INR (adjusted OR 0.47, 95%CI 0.28-0.79, p = 0.005) and TPE administration (adjusted OR 3.12, 95%CI 1.10-9.4, p = 0.032) at enrollment were independently associated with SNL. Lower NWI (adjusted OR 0.686, 95%CI 0.53-0.89, p = 0.005) at 96 h was independently associated with SNL.
TPE is independently associated with improvement in SNL by threefold in patients with NWI ≥ 11 and no to early HE. Patients with advanced HE should be offered immediate liver transplant. After 3 sessions of TPE, NWI < 11 increases SNL by 32%. Hence, NWI should be maintained below 11 with more sessions of TPE.
对于患有肝衰竭的威尔逊病（WD）患者，肝移植决策十分困难，尤其是关于新威尔逊指数（NWI）的报道存在矛盾。治疗性血浆置换（TPE）可使WD患者通过保留自身肝脏存活（SNL）。本研究旨在观察TPE对预后的影响，并确定SNL的相关因素。
纳入前瞻性收集数据中所有肝衰竭（国际标准化比值≥2.5）的WD病例，进行倾向评分匹配（PSM）以选择TPE组（n = 48）和非TPE组（n = 48）。TPE组连续三天接受三次TPE治疗。
159例病例纳入PSM，以NWI和肝性脑病（HE）分级作为预测指标。在96例患者的整个队列中进行分析时，SNL相当（26例 vs. 17例（比值比1.45，p = 0.05））。在无至早期HE的患者中进行TPE时，SNL显著改善：TPE组（24/37）与非TPE组（14/34）（比值比 = 1.70，p = 0.03）。在分析无至早期HE的患者时，TPE组的Kaplan-Meier生存曲线显著改善（对数秩检验p = 0.019）。入组时较低的国际标准化比值（校正比值比0.47，95%置信区间0.28 - 0.79，p = 0.005）和TPE治疗（校正比值比3.12，95%置信区间1.10 - 9.4，p = 0.032）与SNL独立相关。96小时时较低的NWI（校正比值比0.686，95%置信区间0.53 - 0.89，p = 0.005）与SNL独立相关。
对于NWI≥11且无至早期HE的患者，TPE与SNL改善三倍独立相关。晚期HE患者应立即接受肝移植。三次TPE治疗后，NWI < 11可使SNL增加32%。因此，应通过更多次TPE治疗使NWI维持在11以下。
