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临床特征鉴别先天性胆道闭锁与其他新生儿胆汁淤积症的病因。

Clinical features differentiating biliary atresia from other causes of neonatal cholestasis.

机构信息

Departments of Paediatrics, University of Malaya Medical Centre, Malaysia.

出版信息

Ann Acad Med Singap. 2010 Aug;39(8):648-54.

Abstract

INTRODUCTION

This study determined any clinical features which may help to differentiate biliary atresia (BA) from other causes of neonatal cholestasis (NC).

MATERIALS AND METHODS

A prospective and observational study was conducted on consecutive infants with NC referred to the University of Malaya Medical Centre, Malaysia, between November 1996 and May 2004.

RESULTS

The 3 most common causes of cholestasis among the 146 infants with NC studied were idiopathic neonatal hepatitis (n = 63, 43%), BA (n = 35, 24%) and congenital cytomegalovirus hepatitis (n = 13, 9%). Common clinical features at presentation were jaundice (100%), hepatomegaly (95%), splenomegaly (52%) and pale stools (47%). Three clinical features noted to be sensitive for BA were the presence of acholic or variably acholic stools on admission, a liver which was firm/hard in consistency and a palpable liver of ≥4 cm (sensitivity of 77%, 80% and 94%, respectively), but the corresponding specificity was poor (51%, 65% and 39%, respectively). The stools of 2 children with BA were pigmented initially but became acholic subsequently.

CONCLUSIONS

We did not find any single clinical feature with sufficient sensitivity and specificity to differentiate BA from other causes of NC. Repeated inspection of stools colour is necessary as occasionally, patients with BA may have initial pigmented stools. Biochemical assessment and imaging studies are important in the assessment of any infant with NC.

摘要

介绍

本研究旨在确定有助于鉴别胆道闭锁(BA)与其他新生儿胆汁淤积(NC)病因的临床特征。

材料与方法

对 1996 年 11 月至 2004 年 5 月期间在马来西亚马来亚大学医学中心就诊的连续 NC 婴儿进行了一项前瞻性和观察性研究。

结果

在研究的 146 例 NC 婴儿中,胆汁淤积的 3 个最常见病因分别为特发性新生儿肝炎(n = 63,43%)、BA(n = 35,24%)和先天性巨细胞病毒肝炎(n = 13,9%)。入院时常见的临床特征为黄疸(100%)、肝肿大(95%)、脾肿大(52%)和粪便色浅(47%)。3 种临床上有助于诊断 BA 的特征为入院时粪便呈白陶土色或变浅陶土色、肝脏质地坚硬/硬实和可触及肝脏≥4 cm(敏感性分别为 77%、80%和 94%),但相应的特异性较差(51%、65%和 39%)。2 例 BA 患儿的粪便起初呈色素性,但随后变为白陶土色。

结论

我们未发现任何具有足够敏感性和特异性的单一临床特征可用于鉴别 BA 与其他 NC 病因。需要反复检查粪便颜色,因为 BA 患儿偶尔会出现初始色素性粪便。对于任何 NC 婴儿,生化评估和影像学检查均很重要。

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