Design and validation of the biliary atresia research consortium histologic assessment system for cholestasis in infancy.
Design and validation of the biliary atresia research consortium histologic assessment system for cholestasis in infancy.
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DOI：
10.1016/j.cgh.2011.01.003
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发表时间：
2011-04
影响因子：
12.6
通讯作者：
Sokol, Ronald J.
中科院分区：
文献类型：
--
作者：
Russo, Pierre;Magee, John C.;Boitnott, John;Bove, Kevin E.;Raghunathan, Trivellore;Finegold, Milton;Haas, Joel;Jaffe, Ronald;Kim, Grace E.;Magid, Margret;Melin-Aldana, Hector;White, Frances;Whitington, Peter F.;Sokol, Ronald J.
参与美国国立卫生研究院（NIH）资助的胆道闭锁（BA）研究联盟（BARC）的病理学家开发并评估了一种用于对胆汁淤积婴儿的肝活检进行组织学报告的标准化系统。
一组97份匿名肝活检样本被送往BARC中心的10位病理学家处。开发了一种具有16种组织学特征的半定量评分系统，然后由不了解临床病史、影像学结果或实验室数据的病理学家使用。对观察者间的一致性进行了统计学评估。使用加权κ统计量评估了每个特征评分以及病理学家诊断（与最终临床诊断相比）的一致性。
在识别胆管内胆汁栓、巨细胞转化、髓外造血和胆管增生方面，观察者间存在中度到高度的一致性。在临床确诊的BA病例中，病理学家对梗阻的诊断率为79% - 98%，阳性预测值（PPV）为90.7%。基于逻辑回归，对BA预测性最佳的组织学特征包括胆管增生、门脉纤维化和无窦周纤维化（每项P < 0.0001）。
BARC组织学评估系统识别了胆汁淤积婴儿肝活检的特征，观察者间一致性良好，可用于诊断和预后判断。该系统对BA的诊断具有较高的敏感性，并能以合理的观察者间一致性识别胆道梗阻婴儿。然而，如果没有足够的临床信息，无法区分BA与诸如全胃肠外营养相关肝病和α - 1 - 抗胰蛋白酶缺乏症等疾病。
Pathologists participating in the NIH-sponsored Biliary Atresia (BA) Research Consortium (BARC) developed and then evaluated a standardized system for histological reporting of liver biopsies from infants with cholestasis. A set of 97 anonymous liver biopsy samples was sent to 10 pathologists at BARC centers. A semi-quantitative scoring system that had 16 histologic features was developed and then used by the pathologists, who had no knowledge of clinical history, imaging results, or laboratory data. Inter-observer agreement was evaluated statistically. Agreement on scoring of each feature and on the pathologists’ diagnosis, compared with the final clinical diagnosis, were evaluated using weighted kappa statistics. There was moderate to substantial inter-observer agreement in identification of bile plugs in ducts, giant-cell transformation, extramedullary hematopoiesis, and bile duct proliferation. The pathologists’ diagnosis of obstruction in clinically proven cases of BA ranged from 79% to 98%, with a positive predictive value (PPV) of 90.7%. Histological features that best predicted BA, based on logistic regression, included bile duct proliferation, portal fibrosis, and absence of sinusoidal fibrosis (each P<0.0001). The BARC histological assessment system identified features of liver biopsies from cholestatic infants, with good inter-observer agreement, that might be used in diagnosis and determination of prognosis. The system diagnosed BA with a high level of sensitivity and identified infants with biliary obstruction with reasonable inter-observer agreement. However, distinguishing between BA and disorders such as total parenteral nutrition-associated liver disease and alpha-1-antitrypsin deficiency is not possible without adequate clinical information.
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DOI：
10.1111/j.1478-3231.1999.tb00033.x
发表时间：
1999-06-01
期刊：
LIVER
影响因子：
--
作者：
Westin, J;Lagging, LM;Dhillon, AP
通讯作者：
Dhillon, AP
影响因子：
5.1
作者：
Davenport, Mark;Tizzard, Sarah A.;Hadzic, Nedim
通讯作者：
Hadzic, Nedim
影响因子：
3.3
作者：
BROUGH, AJ;BERNSTEIN, J
通讯作者：
BERNSTEIN, J
影响因子：
5.2
作者：
MANOLAKI, AG;LARCHER, VF;HOWARD, ER
通讯作者：
HOWARD, ER
DOI：
10.1001/archpedi.1972.02110100031009
发表时间：
1972-01-01
影响因子：
--
作者：
NEZELOF, C
通讯作者：
NEZELOF, C