Gautam Vipul, Lal Bikrant B, Kumar Vikram, Agarwal Shaleen, Sood Vikrant, Khanna Rajeev, Alam Seema, Gupta Subhash
Department of Pediatric Hepatology, Centre for Liver and Biliary Sciences, Max Superspeciality Hospital, Saket, New Delhi, India.
Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.
J Clin Exp Hepatol. 2024 Sep-Oct;14(5):101427. doi: 10.1016/j.jceh.2024.101427. Epub 2024 Apr 16.
Hepatitis A virus (HAV) infection is the commonest cause of pediatric acute liver failure (PALF) in developing countries. Literature has shown good outcomes of HAV-induced PALF as compared to other etiologies. The advanced critical care and use of extracorporeal liver assist devices (ELAD) have improved the survival with native liver in PALF and overall outcomes. Various liver transplant listing criteria have been proposed in PALF, however none of them is specific enough to predict the outcome. The timing of liver transplant in living donor setting has never been straightforward. Dynamic clinical and biochemical monitoring of the ALF child is the key to decide for LT.
Here we report three children with HAV-induced PALF presented with advanced hepatic encephalopathy (HE) and high international normalized ratio (INR > 10). These children survived with native liver despite fulfilling the liver transplant criteria. The first child is a 14-year-old male who had peak INR of more than 10.2 and grade 3-4 HE with cerebral edema and acute kidney injury. He responded to medical management and CRRT as liver assist device. The second one is a 7-year-old male child who also recovered well with native liver despite advanced HE and INR of more than 10. Third child is a 16-year-old male who had peak INR of 12.6 and grade 2 HE. He received ELAD (Therapeutic plasma exchange and CRRT) and survived with native liver.
Children with HAV-induced PALF can recover with their native liver despite extremely poor prognostic markers like very high INR, ammonia and advanced HE.
甲型肝炎病毒(HAV)感染是发展中国家儿童急性肝衰竭(PALF)最常见的病因。文献表明,与其他病因相比,HAV所致PALF的预后良好。先进的重症监护和体外肝脏辅助装置(ELAD)的使用提高了PALF患者保留自身肝脏的生存率及总体预后。PALF已提出了各种肝移植列入标准,但均不够特异,无法预测预后。活体供肝情况下肝移植的时机一直不明确。对急性肝衰竭患儿进行动态临床和生化监测是决定是否进行肝移植的关键。
我们在此报告3例HAV所致PALF患儿,均出现严重肝性脑病(HE)且国际标准化比值很高(INR>10)。尽管符合肝移植标准,但这些患儿均保留自身肝脏存活下来。首例患儿为14岁男性,最高INR超过10.2,3-4级HE伴脑水肿和急性肾损伤。他对药物治疗及作为肝脏辅助装置的连续性肾脏替代治疗(CRRT)有反应。第二例是一名7岁男童,尽管有严重HE且INR超过10,但也保留自身肝脏恢复良好。第三例患儿为16岁男性,最高INR为12.6,2级HE。他接受了ELAD(治疗性血浆置换和CRRT),并保留自身肝脏存活。
HAV所致PALF患儿尽管有非常高的INR、血氨水平及严重HE等极差的预后指标,但仍可通过自身肝脏恢复。