Indian childhood cirrhosis (ICC) & ICC-like diseases: The changing scenario of facts versus notions
Indian childhood cirrhosis (ICC) & ICC-like diseases: The changing scenario of facts versus notions
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发表时间：
2013-06
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通讯作者：
N. Nayak;A. Chitale
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作者：
N. Nayak;A. Chitale
印度儿童肝硬化（ICC），一种被认为是印度特有的地方病，现在已经在其他国家的非印度血统的儿童中记录在案。最近，印度医学研究理事会赞助的一项大型多中心研究得出了一些结果，消除了一些普遍接受的观念，并就该疾病的不同方面确立了一些新的事实。对迄今为止关于ICC和ICC样疾病的所有相关报告进行了审查，以获得我们对这种非威尔逊铜超载肝病的理解现状的正确观点。早期在印度的研究基础上对外源性铜在引起疾病中的主要作用进行了辩论，但国外研究人员对一些散发病例和一系列地方性ICC样疾病的研究支持在遗传易感的ICC样疾病和ICC中的婴儿和儿童中摄入铜引起肝毒性损伤。然而，基于225例ICC和426例对照的良好对照的ICMR研究中的流行病学和形态学结果令人信服地驳斥了这一概念。此外，这项研究表明，与以前所认为的不同，3岁以上的年龄较大的儿童可以患上这种疾病，并且在其自然病程中，肝脏组织学可以在被认为是诊断性的特征和其他一些模式之间转换，其中任何一种都可以是患者首次出现的形态学表现。年龄较大的儿童和病例在介绍时有较轻的形态学变化有较长的生存期。从所有可用数据的批判性分析得出的总体推断是，ICC和ICC样疾病在临床上表现为任何年龄的儿童，尽管在年幼的儿童中很常见，并且必须在任何患有所谓的“隐源性膀胱炎”的儿童中进行临床诊断。暴露于食物、牛奶和水中的外源性铜不应是这一考虑的先决条件。在可行的情况下，肝活检应是强制性的确认，理解肝脏的形态学变化可以呈现除了目前被认为是诊断性的特征之外的一些其他模式。目前ICC发病率下降的原因可能部分是由于漏诊，部分是由于与时间相关的经济和社会文化变化导致的发病率真正下降。
Indian childhood cirrhosis (ICC), a disease considered to have been endemic in and unique to India has now been documented in children of non-Indian origin from other countries. More recently available findings from a large multicentre study sponsored by the Indian Council of Medical Research (ICMR) have dispelled some of the generally accepted notions and have established several new facts on different aspects of the disease. All relevant reports on ICC and ICC-like diseases, till date, were reviewed to obtain a proper perspective on the current state of our understanding on this non-Wilsonian copper overload liver disease. A primary role of exogenous copper in causing the disease was earlier debated on the basis of studies in India but investigators abroad studying some sporadic cases and a series of endemic ICC-like diseases supported a hepatotoxic injury by ingested copper in genetically susceptible infants and children in ICC- like disease and in ICC. Epidemiologic and morphologic findings in the well controlled ICMR study based on 225 cases of ICC and 426 controls, all confirmed on liver biopsy, have however, convincingly refuted this concept. Additionally, this study revealed that unlike what has been believed earlier, older children more than 3 yr age can get the disease and that in its natural course the hepatic histology can transform between the characteristic one considered diagnostic and some other patterns, any one of which can be the morphologic manifestation at first presentation of the patient. Older children and cases with milder morphologic changes at presentation had longer survival. The overall inference from critical analysis of all available data is that ICC and ICC-like diseases clinically manifest in a child of any age though common in younger ones, and a clinical diagnosis must be made in any child with so-called ‘cryptogenic cirrhosis’. Exposure to exogenous copper in food, milk and water should not be a prerequisite for this consideration. A liver biopsy whenever feasible should be mandatory for confirmation with the understanding that the morphologic changes in liver can present a few other patterns besides the characteristic one currently taken to be diagnostic. The ascribed current decline in encountering ICC is likely to be due partly to missing a diagnosis and partly to a true reduction in incidence consequent on time related economic and socio-cultural changes.