Balamourougane P, Dattagupta S, Bhatnagar V
Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi-110029, India.
Trop Gastroenterol. 2009 Jul-Sep;30(3):167-70.
Biliary atresia (BA) and idiopathic neonatal hepatitis (NH) account for 50-70% of all cases with neonatal cholestasis. The treatment of the former is early surgical intervention, while the latter requires non-surgical supportive care. Failure to differentiate the two conditions may result in avoidable surgery in NH, which may significantly increase morbidity. The lack of differentiating clinical features, biochemical markers and other specific investigations to distinguish the two is still a major problem.
This study was thus initiated to evaluate electron microscopic changes in the liver in patients with NH and BA, to correlate these with changes on light microscopy and look for specific differentiating features between the two.
Ten patients with neonatal cholestasis whose liver specimens were available for electron microscopic analysis were included in the study. There were 6 patients with BA and 4 patients with NH.
Among the biochemical parameters, serum alkaline phosphatase and gamma glutamyl transpeptidase were significantly higher in BA than in patients with NH. On light microscopy, giant cell transformation was seen in 75% patients with NH and 33.3% of patients with BA. Even in BA, intracellular cholestasis was more prominent than ductular cholestasis (100% vs. 50%). Ductular proliferation was seen in 50% of NH patients and all patients of BA. Electron microscopy revealed prominent endoplasmic changes in all patients with NH and to a milder degree in BA. Changes in mitochondria and glycogen content were similar in both groups.
Ultrastructural changes in neonatal cholestasis seen through electron microscopy are largely non-specific and do not differentiate BA from NH.
胆道闭锁（BA）和特发性新生儿肝炎（NH）占所有新生儿胆汁淤积病例的50%-70%。前者的治疗是早期手术干预，而后者需要非手术支持治疗。未能区分这两种情况可能导致NH患者接受不必要的手术，这可能会显著增加发病率。缺乏区分这两种疾病的临床特征、生化标志物和其他特异性检查仍然是一个主要问题。
因此开展本研究，以评估NH和BA患者肝脏的电子显微镜变化，将这些变化与光学显微镜下的变化相关联，并寻找两者之间的特异性鉴别特征。
本研究纳入了10例有肝脏标本可用于电子显微镜分析的新生儿胆汁淤积患者。其中6例为BA患者，4例为NH患者。
在生化参数中，BA患者的血清碱性磷酸酶和γ-谷氨酰转肽酶显著高于NH患者。在光学显微镜下，75%的NH患者和33.3%的BA患者可见巨细胞转化。即使在BA患者中，细胞内胆汁淤积也比胆小管胆汁淤积更突出（100%对50%）。50%的NH患者和所有BA患者可见胆小管增生。电子显微镜显示，所有NH患者均有明显的内质网变化，BA患者内质网变化程度较轻。两组线粒体和糖原含量的变化相似。
通过电子显微镜观察到的新生儿胆汁淤积的超微结构变化在很大程度上是非特异性的，无法区分BA和NH。
