重视儿童急性肝衰竭的诊治

儿童急性肝衰竭(PALF)临床上虽少见，但起病急，进展快，严重威胁着儿童的生命和健康。其病因多样，目前较多患儿最终仍未能明确病因。PALF的临床表现与成人有所不同，婴幼儿早期肝性脑病的判断有一定的难度。维持内环境的稳定及病因治疗对于PALF极为重要，应避免乱用药、滥用血制品，有指征的患儿可行血液净化治疗为自体肝功能恢复和肝移植赢得更多的时间。PALF的精准诊治需要受到更多的重视。     

人工肝技术治疗儿童暴发性肝衰竭的临床分析及预后判断

目的观察人工肝治疗儿童暴发性肝衰竭(PALF)的疗效并对预后因素进行分析。方法回顾性分析2012年1月-2015年2月于吉林大学第一医院儿科重症监护病房住院的23例PALF并行人工肝治疗患儿的临床资料,依据随访3个月内临床转归情况分为存活组(n=15)和死亡组(n=8)。分别观察两组患儿治疗前后肝功能、凝血功能的变化并比较两组患儿治疗前的相关指标和MELD评分,分析人工肝治疗PALF的疗效以及影响预后判断的因素。计量资料组间比较采用t检验。结果予人工肝治疗后,存活组ALT、TBil、血氨以及乳酸(Lac)水平均较治疗前明显降低,差异具有统计学意义(t值分别为8.812、6.243、8.431、6.721,P值均0.01);死亡组中仅ALT水平较治疗前降低,差异有统计学意义(t=2.532,P0.05)。与治疗前相比,存活组治疗后的凝血酶原时间(PT)、凝血酶原活动度(PTA)、国际标准化比值(INR)均明显改善,差异均具有统计学意义(t值分别为6.256、-2.738、6.711,P值均0.05)。与存活组治疗前比较,死亡组治疗前ALT水平明显降低(t=6.283,P0.01),TBil明显增高(t=-3.938,P=0.001),PT延长(t=-2.394,P=0.026),MELD评分增高(t=-6.239,P0.01)。结论人工肝技术是治疗儿童PALF的一种有效方法。当患儿ALT水平较高、PT较短、MELD评分相对较好时,一旦确诊,尽快实施人工肝技术可提高抢救存活率,而酶胆分离现象、PT以及MELD评分有助于判断PALF的预后。     

儿童急性肝衰竭的病因分析

儿童急性肝衰竭(PALF)是一种复杂且快速进展的综合征,病因具有年龄依赖性。分析了临床上PALF的常见病因,认为能引起PALF的病因包括:感染因素、遗传代谢性因素、中毒和药物、异常灌注和自身免疫性疾病等,其中以感染因素最常见。随着诊疗技术的提高,遗传代谢性疾病和自身免疫性疾病导致的PALF诊出率越来越高。由于PALF患儿较少,在病因诊断上尚缺乏经验,总结分析已报道的文献,以期对PALF的病因筛查提供一定的参考。     

肝移植与肠道菌群的相互作用关系及临床治疗进展

肝移植的术后并发症一直是制约肝移植预后的主要因素。肠道菌群作为“肠-肝轴”的重要组成部分,在肝移植术后呈现特定的菌群组成的改变,与肝移植术后的预后及并发症有密切关系。本文综述了肝移植术前后患者肠道菌群的差异,肠道菌群的变化与肝移植术后预后及各类并发症的关系,以及肠道菌群为靶点改善肝移植术预后的临床治疗进展和发展趋势,为减少肝移植并发症,提高肝移植患者长期存活和生活质量提供参考。     

新生儿胆道闭锁治疗过程中的常见临床问题

胆道闭锁是外科新生儿黄疸的重要原因,也是儿童肝移植手术的首要病因。肝门空肠吻合术(Kasai手术)是治疗胆道闭锁的有效方法,简要介绍了Kasai术后并发症及长期预后情况;强调胆道闭锁首选Kasai手术,分析了可能影响手术效果的相关因素;简述胆道闭锁术后自体肝长期生存可能存在的问题;提出需更多关注Kasai术后至肝移植前的综合治疗。     

肝移植术后早期肝功能不全影响因素的研究进展

肝移植作为终末期肝病最有效的治疗手段,术后往往会出现早期移植物功能不全。肝移植术后早期肝功能不全已成为肝移植术后的并发症之一,严重影响移植物和患者生存。因此,减少其发生可作为改善肝移植术后受体预后的重要手段。现就肝移植术后受体早期肝功能不全的定义、影响因素、预后、预测模型等研究进展作一论述,以期为更好地防治移植术后早期肝功能不全,进一步提高肝移植手术成功率,改善患者预后提供有效参考。     

甲型肝炎患儿的肝衰竭

关于甲型肝炎患儿肝衰竭的报导极少,有关暴发性甲型肝炎患者进行肝移植的筛选标准通常不可用于对儿童的评价。本文对儿童甲型肝炎的严重性和预后进行了回顾性研究。法国儿科肝移植中心的24名甲肝并出现了肝衰的患儿被根据分析目地分了组。24人中     

胆道闭锁患儿肝移植术后并发症分析

目的分析肝移植治疗胆道闭锁患儿术后并发症及预后。方法回顾性分析2006年6月-2014年4月于重庆医科大学附属儿童医院完成初次肝移植的41例胆道闭锁患儿临床资料,其中活体肝移植28例,心脏死亡器官捐献(DCD)供肝肝移植13例。活体与DCD肝移植受者术后随访时间分别为67~90个月和15~56个月,总结围手术期及随访期并发症发生情况及预后。计数资料组间比较采用Fisher's精确概率法。结果 41例肝移植受者围手术期并发症包括:血管并发症、腹腔出血、肠穿孔、排斥反应、感染并发症等。DCD肝移植组肝动脉栓塞(HAT)的发生率明显高于活体肝移植组(P=0.02)。围手术期死亡10例,其中活体肝移植组4例,包括HAT 1例;因HAT再次行DCD肝移植1例,后因原发肝无功能死亡;呕吐窒息1例;多器官功能衰竭1例。DCD肝移植死亡6例,包括HAT 1例;肺部肺炎克雷伯杆菌感染1例;肠漏后感染性休克1例;循环衰竭1例;严重毛细血管渗漏综合征1例;原发肝无功能1例。随访期活体肝移植死亡4例,包括肝静脉狭窄2例;胆道感染1例;胆道狭窄1例。DCD肝移植随访期间未发生死亡及其他并发症。结论胆道闭锁肝移植术后并发症多样,是影响肝移植手术成功率及长期生存率的重要因素,早期预防、早期发现、及时治疗,对改善肝移植患儿预后至关重要。     

血管内支架植入治疗部分肝移植术后流出道狭窄

目的 探讨血管内支架治疗肝移植术后流出道狭窄的适应症、手术时机、操作要领、后续治疗等。方法 总结我院2例左半肝移植术后流出道狭窄患者的诊断和治疗经过,通过股静脉进行肝静脉造影并测压,证实2例患者存在肝静脉-下腔静脉吻合口狭窄,经球囊血管扩张后置入血管内支架。结果 2例患者经血管支架治疗后,肝静脉内压力迅速回降,腹水等症状逐渐消失,肝功能恢复正常。观察6个月,无相关并发症发生,最终解决了肝静脉流出道狭窄的问题。结论 肝移植术后流出道狭窄严重影响患者近期和远期预后,早期诊断、早期干预是治疗肝移植术后流出道狭窄的关键。介入下血管内支架植入是肝移植术后流出道狭窄的有效治疗措施之一,可以一定程度改善患者预后。     

亲体肝移植治疗儿童门静脉海绵样变性3例临床分析

目的 评估亲体肝移植手术治疗儿童门静脉海绵样变性的疗效.方法 3例因反复上消化道大出血、顽固性腹水和脾功能亢进患儿,经门静脉显影、螺旋CT扫描及门静脉三维影像重建技术确诊为门静脉海绵样变性,伴有严重的门静脉高压症患儿,分别于2006年7月、11月和2007年1月施行亲体肝移植术治疗.术后从临床表现、血液学指标和影像学资料等方面对手术疗效进行评价.结果 术后3例患儿中有2例消化道出血停止、腹水消失、门静脉高压症状消失,血红蛋白、红细胞压积、血小板水平和血浆白蛋白等均恢复正常,随访至今无复发;另1例术后3 d因术后门静脉血栓形成死亡. 结论 亲体肝移植可能是治疗儿童门静脉海绵样变性伴严重门静脉高压的有效方法,但门静脉重建是此类疾病进行亲体肝移植手术的难点.     

Management of chronic hepatitis B in severe liver disease

In the past few decades,chronic hepatitis B(CHB)has evolved from a disease that was untreatable and progressive,to one that can be easily controlled with antiviral therapy.However,patients with severe liver disease still remain difficult to treat despite the availability of highly potent nucleos(t)ide analogs.These include those with underlying cirrhosis,severe flares of CHB,hepatocellular carcinoma(HCC),and for those undergoing liver transplantation.For those with established cirrhosis,antiviral therapy should be considered for all,as unpredictable flares can still occur,which can be fatal for those with advanced chronic liver disease.However,even with effective viral suppression,the development of HCC can still occur.For patients with severe flares of CHB,although the use of antiviral can improve long term outcomes,a significant proportion may still die without liver transplantation.The short term prognosis of these patients is dependent on both the severity of flare and underlying pre-existing liver disease.In patients with decompensated cirrhosis,liver failure secondary to severe flares,or those with HCC,liver transplantation may be curative.After liver transplantation,long term antiviral therapy is required to prevent graft loss from recurrent hepatitis B infection.The use of hepatitis B immune globulin(HBIG)in combination with an oral antiviral agent has been the mainstay of post-transplant antiviral regimen for over adecade.With newer and more potent antiviral agents such as tenofovir and entecavir,use of these agents along with HBIG have demonstrated to be effective in preventing significant recurrence in the long term.     

肝癌肝移植转化治疗策略进展

肝移植能够在彻底去除肿瘤的同时完整移除病肝,对于合并肝硬化的肝癌患者是最佳选择。近年来,随着肝癌综合治疗研究的进展,转化治疗理念被引入肝癌外科治疗领域以及肝移植领域,成功的转化治疗有望将超出标准肝癌患者转变为符合标准而接受肝移植手术,从而极大改善其预后。就肝癌肝移植转化治疗的进展进行简要介绍。     

免疫治疗时代下的肝癌肝移植

我国大部分肝癌患者在就诊时不能获得根治性手术治疗,近年来肝癌免疫治疗的突破性进展为这些中晚期肝癌患者带来了曙光。在肝癌肝移植领域,免疫治疗更是因为在肿瘤免疫和移植免疫中扮演的双重角色而备受关注。在术前降期治疗和移植术后肿瘤复发的治疗中均有创新性的应用。在免疫治疗的时代下如何应用移植肿瘤学思维使肝癌肝移植患者获益是一个崭新的课题,这需要在临床实践中进行多学科团队协作,探讨最佳的肝癌肝移植患者治疗策略,最终改善中晚期肝癌患者的预后。     

Liver transplantation in acute liver failure:A challenging scenario

Acute liver failure is a critical medical condition defined as rapid development of hepatic dysfunction associated with encephalopathy. The prognosis in these patients is highly variable and depends on the etiology, intervalbetween jaundice and encephalopathy, age, and the degree of coagulopathy. Determining the prognosis for this population is vital. Unfortunately, prognostic models with both high sensitivity and specificity for prediction of death have not been developed. Liver transplantation has dramatically improved survival in patients with acute liver failure. Still, 25% to 45% of patients will survive with medical treatment. The identification of patients who will eventually require liver transplantation should be carefully addressed through the combination of current prognostic models and continuous medical assessment. The concerns of inaccurate selection for transplantation are significant, exposing the recipient to a complex surgery and lifelong immunosuppression. In this challenging scenario, where organ shortage remains one of the main problems, alternatives to conventional orthotopic liver transplantation, such as living-donor liver transplantation, auxiliary liver transplant, and ABO-incompatible grafts, should be explored. Although overall outcomes after liver transplantation for acute liver failure are improving, they are not yet comparable to elective transplantation.     

儿童肝脏恶性肿瘤的肝移植适应证

儿童肝脏恶性瘤与成人肝脏恶性肿瘤的起源和发展存在明显差别,即使是同一种肝脏恶性肿瘤,在儿童体内的发生、发展、治疗方法和治疗效果方面,也与成人存在较大的差别。肝脏恶性肿瘤的组织学表现和解剖定位可以反映出这些肿瘤侵袭转移的特性、外科手术根治的难度和药物治疗的敏感性。因此在治疗方式选择上,需要依据这些特点进行判断。儿童的肝脏恶性肿瘤对化疗等辅助治疗的敏感性更高。肝移植术前或术后,配合使用化疗等辅助治疗可以获得更为满意的疗效,肝移植的适应证范围也因此得到了一定的扩展。儿童肝脏恶性肿瘤中,肝母细胞瘤、肝细胞癌和未分化胚胎肉瘤较为常见,均可以通过肝移植进行治疗,且多数患者预后满意。儿童肝脏恶性肿瘤如果肝切除治疗预期效果不佳,而肿瘤局限于肝内,均可考虑肝移植。     

Etiology,outcome and prognostic factors of childhood acute liver failure in a German Single Center

Pediatric acute liver failure (PALF) is a progressive, potentially fatal clinical syndrome occurring in previously healthy children. Our study aimed to determine the current leading causes of PALF in a single center in Germany, identifying possible prognostic markers. Thirty-seven pediatric patients with PALF were included. Medical records were reviewed for demographic, laboratory and clinical data. Laboratory results on admission and at peak value, PELD and MELD score on admission, and intensive care support were assessed. Fifteen patients recovered spontaneously, 14 died without transplantation, and 8 received a liver transplant. Patients who survived were significantly older than patients who died. Specific causes of PALF could be identified as infectious diseases (16%), metabolic diseases (14%), toxic liver injury (11%), immunologic diseases (8%), or vascular diseases (8%). Causes of PALF remained indeterminate in 43%. High ammonia, low albumin, and low ALT levels on admission were associated with worse outcome. Absence of need of ventilation, hemodialysis, and circulatory support predicted spontaneous recovery. In conclusion, infections are the most common known cause of PALF. However, in a large proportion of patients the cause for PALF remains cryptic. Ammonia and albumin levels may be of prognostic value to predict outcomes.     

Pancreatic neuroendocrine tumor accompanied with multiple liver metastases

Pancreatic neuroendocrine tumor(P-NET) is rare and slow-growing. Current classifications predict its progno-sis and postoperative recurrence. Curative resection is ideal, although often difficult, because over 80% of pa-tients have unresectable multiple liver metastases and extrahepatic metastasis. Aggressive surgery for liver metastases is important to improve survival. Aggressive or cytoreductive surgery for liver metastases is indi-cated to reduce hormone levels and improve symptoms and prognosis. Liver transplantation was originally con-ceived as an ideal therapy for unresectable liver metas-tases. Unfortunately, there is no clear consensus on the role and timing of surgery for primary tumor and liver metastases. Surgeons still face questions in deciding the best surgical scenario in patients with P-NET with unresectable liver metastases.     

肝移植术后早期肝功能的动态变化及其对预后的价值

目的动态观察肝移植术后1周内肝功能的变化,明确变化规律及术式对其的影响,以筛选对判断预后有价值的指标。方法回顾性分析149例肝移植受者术前、术后1、3、7d时ALT、AST、总胆红素(TB)、直接胆红素(DB)及凝血酶原时间(PT)、部分凝血活酶时间(APTT)、纤维蛋白原的变化情况,采用logistic回归筛选与预后有关的指标。结果肝移植术后1个月内生存的患者在术后1周内肝功能呈单峰变化。术后1d,纤维蛋白原恢复;术后3d,PT、APTT恢复;术后7d,ALT、AST下降至正常值附近。术后1个月内死亡的患者胆红素出现双峰,PT、APTT高峰前移,各项指标恢复时间延迟。背驮式肝移植受者前期恢复慢于经典式受者,但在术后1周内两组均可恢复,且术式与预后无关。术后7d的AST水平,术前及术后1、3、7d的TB水平,术后1、3d的DB水平及术后1d的PT值与预后相关。结论肝移植术后1周内肝功能出现单峰改变,术后7d基本可恢复正常;肝移植术后1周内AST、TB、PT与术后早期预后相关。术后高峰形状及位置改变提示并发症的出现,对术后管理和判断预后有积极意义。