The role of CK7, Ki-67, CD34 and vimentin in the differentiation between biliary atresia and idiopathic neonatal hepatitis in Egyptian cholestatic neonates

Apmis ◽  
2012 ◽  
Vol 120 (7) ◽  
pp. 529-538 ◽  
Author(s):  
HAYAM ABDEL SAMIE AIAD ◽  
MONA ABDEL HALIM KANDIL ◽  
REHAB MONIR SAMAKA ◽  
MERVAT MAHMOUD SULTAN ◽  
MOHAMED TAWFIK BADR ◽  
...  
PEDIATRICS ◽  
1992 ◽  
Vol 89 (2) ◽  
pp. 329-330
Author(s):  
PIETRO VAJRO ◽  
ANGIOLA FONTANELLA ◽  
EMILIA VUTTARIELLO ◽  
ANNA DE VINCENZO ◽  
GIULIANA FORTUNATO

For the practicing pediatrician, the most commonly encountered causes of neonatal cholestasis are idiopathic neonatal hepatitis (INH) and extrahepatic biliary atresia (EHBA). However, despite extensive research, little improvement in our understanding of the etiology of INH and EHBA has occurred.1 It has been proposed that both of them may be part of a continuum of hepatobiliary disease caused by a common insult with a different predominant injury site.2 In this respect, it is interesting that, up to the present, several cases of discordance for EHBA in twins of various zygosity have been reported,3-8 whereas no data are available about either concordance or discordance for INH both in monozygotic and dizygotic twins.


2009 ◽  
Vol 33 (6) ◽  
pp. 439-446 ◽  
Author(s):  
Ji-Gang Yang ◽  
Da-Qing Ma ◽  
Yun Peng ◽  
Lei Song ◽  
Chun-Lin Li

Author(s):  
Kim Oanh Bui

Aim: The aim of this study is to find out the cholestatic etiologies in infants and differences of clinical features, laboratory investigations between biliary atresia and other causes of cholestasis at Vietnam Children Hospital. Background: Cholestasis is defined as reduced bile formation or biliary flow. It results of varied causes. Early detection of biliary atresia is to intervene in time and have the best outcome. Patient and methods: In this retrospective study, 305 infants under 12 months of age with cholestasis were studied in Vietnam Children Hospital during 1/2017-7/2018. Demographic data, duration of jaundice, signs and symptoms as well as laboratory, imaging, liver biopsy and the causes of cholestasis were recorded, divided into 2 group BA and Non-BA. Results: 305 infants (194 boys, 111 girls) with cholestasis and mean age of 83,22±72,10 days were included in the study. The most common causes of cholestasis were idiopathic neonatal hepatitis (33,8%), biliary atresia (25,9%), cytomegalovirus infection (21,6%). In BA group, pale stool (100%), Hepatomegaly (98,7%);  increasing less AST, ALT, more GGT level than Non-BA. Find out GGT cutoff > 212,05 UI/l in diagnosing BA. Conclusion: Biliary atresia and idiopathic neonatal hepatitis are the most common causes of infantile cholestasis. Pale stool, hepatomegaly and GGT elevation > 212,05 UI/l are the most reliable tests for diagnosing BA.  


2015 ◽  
Vol 4 (1) ◽  
pp. 30-36
Author(s):  
Kaniz Sultana ◽  
Ariful Haque ◽  
Nadira Musabbir ◽  
Syeda Afria Anwar ◽  
Faika Hussain ◽  
...  

Background: The two most common and important causes of neonatal cholestasis (NC) are biliary atresia (BA) and Idiopathic neonatal hepatitis (INH). There is no single test that can definitely differentiate these two entities. Objective: To evaluate the diagnostic accuracy of clinical and laboratory parameters for diagnosis of biliary atresia. Methods: This cross-sectional study was conducted at the department of Pediatric Gastroenterology and Nutrition of Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh, from August 2013 through July 2015 among purposively sampled infants with neonatal cholestasis. Results: Total 86 neonatal cholestatic cases were studied. Term baby and good birth weight are significantly higher in BA cases. The presence of persistent pale colored stool is significantly more in patients with BA (p 0.000). GGT is the only liver enzyme that was found to be useful differentiating BA from INH at a cut-off value ≥ 524U/L or 9.5 times higher than upper limit normal with sensitivity and specificity of 81.6% and 72.9% respectively. In the present study the diagnostic accuracy of persistent pale colored stool found to be highest (79.1 %). Conclusion: The present study showed that Persistent pale colored stool and serum level of GGT with a cut-off value ≥524 U/L or 9.5 times higher than upper limit normal can be considered as predictive markers for differentiation of Biliary atresia from Idiopathic neonatal hepatitis. CBMJ 2015 January: Vol. 04 No. 01 P: 30-36


2021 ◽  
pp. 109352662098373
Author(s):  
Sunayana Misra ◽  
Kaushik Majumdar ◽  
Puja Sakhuja ◽  
Priyanka Jain ◽  
Lavleen Singh ◽  
...  

Background and Aims Differentiating biliary atresia (BA) from idiopathic neonatal hepatitis (INH) is vital in routine pediatric practice. However, on liver biopsy, few cases offer a diagnostic challenge to discriminate these entities with certainty. Bile ductular reaction (DR), intermediate hepatobiliary cells (IHBC) and extra-portal ductules (EPD) indicate progenitor cell activation, as a response to various hepatic insults. The present study aims to quantify DR, IHBC and EPD by Keratin 7 (CK7) immunohistochemistry (IHC) in BA and INH and to devise a mathematical approach to better differentiate the two, especially in histologically equivocal cases. Methods A total of 98 cases were categorized on biopsy as BA, INH or equivocal histology, favoring BA or INH. CK7 DR mean, IHBC mean and EPD mean values were compared between BA and INH. A formula was derived to help distinguish these two entities, the cut-off value, sensitivity and specificity of which were determined by receiver operating characteristic (ROC) curve. This formula was applied and validated on histologically equivocal cases. Results Univariate logistic regression revealed significant difference between BA and INH with respect to CK7 DR and CK7 EPD mean (p < 0.001 in both); however, CK7 IHBC mean was not significant (p = 0.08). On multivariate logistic regression, only CK7 DR had significant impact on diagnosis (p < 0.001). A formula: (CK7 DR) 2  + (CK7 EPD)/(CK7 IHBC) was derived to help distinguish BA from INH. Cut off value of 10.5 and above, determined by ROC curve, favored a diagnosis of BA (sensitivity= 93.4%, specificity= 94.6%). Histologically equivocal and discrepant cases could be correctly categorized using this formula. Conclusions Formula using CK7 IHC parameters may aid pathologists better distinguish BA from INH, especially in histologically equivocal cases.


Author(s):  
Nsreen R. A. Mohamadien ◽  
Rania Makboul ◽  
Shereen M. Galal ◽  
Nadia M. Mostafa

Abstract Background Biliary atresia (BA) and neonatal hepatitis (NH) are the two major causes of neonatal cholestasis (NC). However, both conditions had entirely different therapeutic schedule and prognosis. Considering BA as a surgical emergency, it is pretty important to accurately differentiate the two entities. The aim of the study is to evaluate the diagnostic utility of hepatobiliary scintigraphy (HBS) using a semi-quantitative technique as well as 15-point histopathological scoring system in differentiating BA from NH. Results The sensitivity, specificity, and overall accuracy of HBS in the diagnosis of BA was 90.5%, 80%, and 83.6%, respectively. The median values of kidney-liver ratio (KLR), intestinal-liver ratio (ILR), and background-liver ratio (BLR) were significantly higher in patients with BA, while that of the liver-kidney ratio (LKR) was significantly lower in cases with BA. Moreover, KLR had the largest area under curve (AUC); advocates it to be the best of the semi-quantitative parameters that can predicts BA. Histopathological scoring using a cutoff point ≥ 7 was helpful in discriminating BA from NH with 85.7% sensitivity, 95% specificity and 91.8% accuracy. Conclusions HBS is a non-invasive diagnostic tool frequently used in diagnosis of BA, yet it has a relatively low specificity. To overcome this challenge, we kindly recommend the use of semi-quantitative parameters that could possibly improve the accuracy of HBS for diagnosing BA. Additionally, the use of 15-point scoring for liver biopsy was useful.


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