COPPER-ASSOCIATED LIVER-DISEASE IN CHILDHOOD
COPPER-ASSOCIATED LIVER-DISEASE IN CHILDHOOD
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DOI：
10.1016/0168-8278(95)80059-x
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发表时间：
1995-11-01
影响因子：
25.7
通讯作者：
MOWAT, AP
中科院分区：
文献类型：
--
作者：
BAKER, A;GORMALLY, S;MOWAT, AP
背景/目的：印度儿童肝硬化与高肝铜浓度和高死亡率的进行性肝病相关，发现早期使用青霉胺治疗可降低死亡率并逆转肝损害，我们旨在确定印度次大陆外铜相关肝病的临床特征，并鼓励早期考虑隐源性肝病综合征。方法:3名欧洲儿童，年龄在10 - 29个月，出现腹胀、发热和肝脾肿大，在1-5周内，由于肝功能衰竭，病情迅速恶化，2名在发病2个月内死亡，1名接受了成功的肝移植，在2例中，诊断仅发生在原位肝移植或死亡后的肝脏检查中，使用光学显微镜，用血红素和伊红、网状蛋白和奥cein染色。用火焰原子吸收光谱法测定组织、血浆和水中的铜含量。结果：所有患者均为小结节性肝硬化和严重肝细胞坏死，伴有马洛里体和丰富的orcein阳性物质，肝铜浓度在1100 ~ 1310 μ g/g干重之间，其中2例患者使用高铜含量的生活用水制备饲料，第三例患者未发现环境来源的过量铜。结论：我们建议，上述在印度次大陆被称为印度儿童肝硬化，在其他地方被称为铜储存病的情况，最好被命名为“儿童铜相关肝病”，强调有必要在不明原因的肝脏疾病中考虑这种疾病，并寻找铜摄入过量的可能来源。(C)国际肝病杂志。
Background/Aims: Indian childhood cirrhosis is associated with high liver copper concentrations and progressive liver disease with a high mortality, Early treatment with penicillamine was found to reduce mortality and reverse liver damage, We aimed to define the clinical features of copper-associated liver disease outwith the Indian subcontinent and encourage the earlier consideration of the syndrome in cryptogenic liver disease.Methods: Three European children presented between 10 and 29 months of age with abdominal distension, pyrexia and hepatosplenomegaly, Over 1-5 weeks their condition deteriorated rapidly due to liver failure, Two died within 2 months of onset and one received a successful liver transplant, In two cases consideration of the diagnosis occurred only on examination of the liver after orthotopic liver transplant or death, Light microscopy was used, with haematoxylin and eosin, reticulin and orcein stains, Tissue, plasma and water copper levels were measured by flame atomic absorption spectrometry.Results: All had micronodular cirrhosis and severe hepatocellular necrosis with Mallory bodies and copious-orcein positive material, Liver copper concentrations ranged from 1100-1310 mu g/g dry weight, For two patients domestic water with high copper content had been used for the preparation of feeds, No environmental source of excess copper could be identified in the third case.Conclusions: We suggest that the above condition, which is called Indian childhood cirrhosis in the Indian subcontinent and Copper Storage Disease elsewhere, would be better named 'Copper-Associated Liver Disease in Childhood', emphasising the need to consider this disorder in unexplained liver disease and to seek possible sources of excessive copper intake. (C) Journal of Hepatology.