儿童并不是缩小版的成人，其疾病特点、治疗转归都与成人有很大的区别。据悉，在明年印度APASL年会上将会设立全新的小儿肝病学论坛，印度新德里肝胆病研究所小儿肝病科主任Seema Alam教授将主持该论坛的筹备工作。在第26届APASL会议上，《国际肝病》采访了Alam教授，向她请教小儿肝衰竭与成人肝衰竭的相关知识。

 

　　小儿急性肝衰竭的主要病因

 

　　Alam教授：与成人相似，病毒感染是导致小儿急性肝衰竭（acute liver failure，ALF）的最常见原因，另一个导致三岁以下小儿ALF的重要原因是遗传性代谢障碍。这些遗传性代谢障碍影响肝脏功能甚至导致肝衰竭。成人ALF中，由遗传性代谢障碍导致者十分少见，但在处理小儿ALF患者时临床医生需要警惕该病因。

 

　　The common causes of acute liver failure as far as children are concerned after the viruses that are common with the adults, because these causes are there in the adults also, the more important causes after that especially so in the younger children less than 3 years of age is the causes which are inborn errors of metabolism. And these inborn errors of metabolism also involve the liver and cause liver failure. This is not usually seen in adults and this needs to be kept in mind when you are managing a patient with acute liver failure in childhood.

 

　　小儿急性肝衰竭的特有表现是什么

 

　　Alam教授：尽管肝性脑病（HE）在成人ALF患者中十分常见，但对于内科或儿科医生而言，要准确诊断小儿、尤其是婴儿或年幼小儿的HE是十分困难的。因此，基于出现HE来判断小儿ALF的方法不可行。观察小儿是否有黄疸、厌食、气色差、不活泼，更有助于小儿ALF的诊断和管理，发现以上征象时及时进行相应的检查及观察能够明确ALF诊断。

 

　　It is important to remember that it is very difficult even for a physician or paediatrician to be able to diagnose hepatic encephalopathy which you usually see so commonly in adults, that is not so easily seen in younger children especially infants and younger children. And sometimes becomes very difficult to diagnose. So to depend on hepatic encephalopathy for diagnose of acute liver failure is not the way to go. For acute liver failure management in children it is better to look for jaundice, it is better to look for the fact that the child is not eating well, not looking well, not playful. And then of course go forward and do the examination and the investigations which can lead to the diagnosis.

 

　　小儿急性肝衰竭的治疗特点

 

　　Alam教授：在治疗方面，小儿ALF与成人ALF的区别更大。许多药物已被证实用于小儿疗效欠佳。其中需要注意的一个药物是N-乙酰半胱氨酸（NAC），有研究明确证实NAC用于非药物性ALF并无益处。因为小儿应用NAC时的相关病死率较高，所以小儿不应使用NAC，尤其是2岁以下小儿。

 

　　另一方面，在成人ALF患者中，部分病因仅通过饮食调整或限制就可以很容易地找到，而这在小儿ALF患者却不适用。因此对于医生来讲，这两类ALF人群的临床处理是有所差别的。

 

　　The management is more less the same, but in a few drugs which have not be found to be so beneficial in children, there is one drug called NAC that is a N-acetylcysteine there are studies very clearly mentioning that there is no benefit of NAC in non-paracetamol acute liver failure which is common on our country and then the NAC should not be used in children, especially not in under 2 years where there has been found the mortality is higher with NAC. The other thing that it is different from the rest of the management in adults is that there is definitely causes which can be easily looked after by just a change of diet and a diet restriction. Hence for doctors the difference that you find in the management of the two groups.

 

　　小儿急性肝衰竭并发症的监测和治疗进展

 

　　Alam教授：首先开发用于监测脑水肿的全新方法十分重要，有助于更有效地发现脑水肿患者，尤其是小儿患者--这是因为脑水肿是ALF最重要的致死性并发症之一，可以使用超声波检测视神经鞘直径来发现小儿脑水肿。

 

　　其次，ALF的另一个重要并发症是小儿患者入住ICU两周后容易出现感染，需要特别加以关注，而且与成人不同的是小儿ALF患者少见真菌感染。

 

　　第三，未来我们还可以开发一些特殊的饮食疗法。

 

　　第四，目前出现了一些基因治疗方法，不过这些基因治疗仅对特定病因所致的部分ALF有效，而并非所有ALF。

 

　　最后是关于疾病的特异性治疗。目前有多种疾病特异性评分系统已用于临床，例如用于Wilsons病的评分能够帮助医生决定肝移植的时机，以及判断患者是否真正需要进行肝移植。几乎所有的肝病都需要这样的简易评分系统。

 

　　I think very important now is that new modalities which have come up to monitor the cerebral edema in children which is there even in adults but in children also it can be done. And it is more effective in looking after the patients with cerebral edema. Definitely it is one of the largest killers in acute liver failure and that is ultrasound based optic nerve sheath diameter which can be seen in children. The other thing is that the infections that are commonly seen in children after two weeks of them staying in the ICU are different from that of the adults and have to be look after, fungal infections are not very common in children. The third thing like I just told you is the special diets which can be used in specific management of some of the diseases.

 

　　Then of course the gene therapies are now available. These gene therapies are available for some of the causes, not for all the causes, so for that it can be look after.

 

　　Finally, like you said, disease specific management. There are these disease specific scoring systems available, like one available for Wilsons disease also which can help you in deciding the timing of liver transplant for these patients and would the patients actually need a liver transplant or what is the time for the liver transplant. Such small score actually needed for almost all diseases.