Abstract：
UNASSIGNED: Cholestatic disorders are a significant cause of morbidity and mortality in infants. Characterization of these disorders and differentiating biliary atresia (BA) from other causes of intrahepatic cholestasis is an age-old problem.
UNASSIGNED: To study the spectrum of different infantile cholestatic disorders in our population, to differentiate BA from other causes of neonatal cholestasis (NC) on a liver biopsy, and validation of the available scoring system for the characterization of these disorders.
UNASSIGNED: This is an observational cross-sectional study performed over a period of 3 years between 2018 and 2021, done on neonates and infants presenting with cholestatic jaundice. The changes on liver biopsy were evaluated by different histological parameters and available scoring systems to differentiate BA from non-BA causes. Correlation with clinical, biochemical, and imaging findings was done in all cases.
UNASSIGNED: This study included 87 cases of NC, of which BA comprised 28 cases (32%), whereas idiopathic neonatal hepatitis (INH) comprised only 12 cases (14%). Portal neutrophilic inflammation (P = 0.000053), ductal cholestasis (P < 0.001), neoductular bile plugs (P < 0.001) and bile ductular proliferation (P < 0.0001) were significantly more in BA, whereas lobular lymphocytic inflammation (P = 0.001) and giant cell transformation of hepatocytes (P = 0.0024) were more frequent in the non-BA group. Using the Lee and Looi scoring system, a histologic score ≥7 was helpful in identifying BA with 85.7% sensitivity, 92.6% specificity, and 90.6% accuracy.
UNASSIGNED: BA is the commonest cause of NC in neonates, whereas the frequency of INH is declining. Detailed histomorphologic analysis of liver biopsy, aided with IHC, is the cornerstone for the diagnosis of these disorders.
UNASSIGNED: To study the spectrum of different infantile cholestatic disorders in our population, to differentiate BA from other causes of neonatal cholestasis (NC) on a liver biopsy, and validation of the available scoring system for the characterization of these disorders.
UNASSIGNED: This is an observational cross-sectional study performed over a period of 3 years between 2018 and 2021, done on neonates and infants presenting with cholestatic jaundice. The changes on liver biopsy were evaluated by different histological parameters and available scoring systems to differentiate BA from non-BA causes. Correlation with clinical, biochemical, and imaging findings was done in all cases.
UNASSIGNED: This study included 87 cases of NC, of which BA comprised 28 cases (32%), whereas idiopathic neonatal hepatitis (INH) comprised only 12 cases (14%). Portal neutrophilic inflammation (P = 0.000053), ductal cholestasis (P < 0.001), neoductular bile plugs (P < 0.001) and bile ductular proliferation (P < 0.0001) were significantly more in BA, whereas lobular lymphocytic inflammation (P = 0.001) and giant cell transformation of hepatocytes (P = 0.0024) were more frequent in the non-BA group. Using the Lee and Looi scoring system, a histologic score ≥7 was helpful in identifying BA with 85.7% sensitivity, 92.6% specificity, and 90.6% accuracy.
UNASSIGNED: BA is the commonest cause of NC in neonates, whereas the frequency of INH is declining. Detailed histomorphologic analysis of liver biopsy, aided with IHC, is the cornerstone for the diagnosis of these disorders.
摘要：
胆汁淤积性障碍是婴儿发病和死亡的重要原因。这些疾病的特征以及将胆道闭锁（BA）与肝内胆汁淤积的其他原因区分开来是一个古老的问题。
■为了研究我们人群中不同婴儿胆汁淤积症的频谱，为了区分BA和其他原因的新生儿胆汁淤积（NC）的肝活检，并验证可用的评分系统来表征这些疾病。
■这是一项观察性横断面研究，在2018年至2021年之间进行了3年，针对出现胆汁淤积性黄疸的新生儿和婴儿。通过不同的组织学参数和可用的评分系统来评估肝活检的变化，以区分BA和非BA原因。与临床相关，生物化学，所有病例均有影像学检查结果.
■本研究包括87例NC，其中BA包括28例(32%)，而特发性新生儿肝炎（INH）仅包含12例（14％）。门静脉中性粒细胞炎症(P=0.000053),导管胆汁淤积（P<0.001），新胆管胆栓（P<0.001）和胆管增生（P<0.0001）在BA中明显增多，而小叶淋巴细胞炎症（P=0.001）和肝细胞巨细胞转化（P=0.0024）在非BA组中更为常见。使用Lee和Looi评分系统,组织学评分≥7有助于识别BA，灵敏度为85.7%，92.6%的特异性，和90.6%的精度。
■BA是新生儿NC的最常见原因，而异烟肼的频率在下降。肝活检的详细组织形态学分析，在IHC的帮助下，是诊断这些疾病的基石。
■为了研究我们人群中不同婴儿胆汁淤积症的频谱，为了区分BA和其他原因的新生儿胆汁淤积（NC）的肝活检，并验证可用的评分系统来表征这些疾病。
■这是一项观察性横断面研究，在2018年至2021年之间进行了3年，针对出现胆汁淤积性黄疸的新生儿和婴儿。通过不同的组织学参数和可用的评分系统来评估肝活检的变化，以区分BA和非BA原因。与临床相关，生物化学，所有病例均有影像学检查结果.
■本研究包括87例NC，其中BA包括28例(32%)，而特发性新生儿肝炎（INH）仅包含12例（14％）。门静脉中性粒细胞炎症(P=0.000053),导管胆汁淤积（P<0.001），新胆管胆栓（P<0.001）和胆管增生（P<0.0001）在BA中明显增多，而小叶淋巴细胞炎症（P=0.001）和肝细胞巨细胞转化（P=0.0024）在非BA组中更为常见。使用Lee和Looi评分系统,组织学评分≥7有助于识别BA，灵敏度为85.7%，92.6%的特异性，和90.6%的精度。
■BA是新生儿NC的最常见原因，而异烟肼的频率在下降。肝活检的详细组织形态学分析，在IHC的帮助下，是诊断这些疾病的基石。
