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Original Article
- The Clinicopathological Parameters for Making the Differential Diagnosis of Neonatal Cholestasis.
- Heejin Lee, Jun Kang, Kyung Mo Kim, Joo Young Jang, Se Jin Jang, Eunsil Yu
- Korean J Pathol. 2009;43(1):43-47.
- DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.1.43
- 4,067 View
- 40 Download
- 5 Crossref
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Abstract
PDF - BACKGROUND
The diseases that cause neonatal cholestasis display several overlapping clinical feature. Making the differential diagnosis using liver biopsy specimens from infants with neonatal cholestasis is important for delivering the proper treatment.
METHODS
We assessed the clinical manifestations, laboratory data, and histopathologic features of the pretreatment liver biopsy specimens from patients suffering with biliary atresia (n=66), intrahepatic bile duct paucity (n=15), and neonatal hepatitis (n=21).
RESULTS
The gender distribution was nearly equal for the patients with biliary atresia and intrahepatic bile duct paucity, whereas males predominated for the cases of neonatal hepatitis. Only the gamma-glutamyl transferase level differed significantly amongst the groups. The diagnostic features for making the differential diagnosis of bile duct lesions included marked bile ductular proliferation, severe fibrosis, and bile duct loss. The difference of the average percentage of portal tracts with bile duct loss was statistically significant between the patients with intrahepatic bile duct paucity (73.9%) and those patients with neonatal hepatitis (39.1%) (p<0.001).
CONCLUSIONS
Bile ductular proliferation, bile duct loss, and advanced fibrosis are useful for the differential diagnosis of neonatal cholestasis. Moreover, stricter diagnostic criteria for bile duct loss (more than 2/3 of bile ducts) should be applied for the definitive diagnosis of intrahepatic bile duct paucity, because bile duct loss also frequently occurs in infants suffering with neonatal hepatitis. -
Citations
Citations to this article as recorded by
- False-negative Hepatobiliary Scintigraphy for Biliary Atresia
Hyunji Kim, Sujin Park, Sejin Ha, Jae Seung Kim, Dae Yeon Kim, Minyoung Oh
Nuclear Medicine and Molecular Imaging.2019; 53(5): 356. CrossRef - Morphometric assessment of liver fibrosis may enhance early diagnosis of biliary atresia
Ahmed F. Abdalla, Abeer Fathy, Khaled R. Zalata, Ahmed Megahed, Ahmed Abo-Alyazeed, Mohammed Ezz El regal
World Journal of Pediatrics.2013; 9(4): 330. CrossRef - Differential hepatic expression of CD56 can discriminate biliary atresia from other neonatal cholestatic disorders
Mostafa Mohamed Sira, Mohamed Abdel-Salam El-Guindi, Magdy Anwar Saber, Nermin Ahmad Ehsan, Marwa Sabry Rizk
European Journal of Gastroenterology & Hepatology.2012; 24(10): 1227. CrossRef - Biliary Atresia: A Multidisciplinary Approach to Diagnosis and Management
Roger Klein Moreira, Rodrigo Cabral, Robert A. Cowles, Steven J. Lobritto
Archives of Pathology & Laboratory Medicine.2012; 136(7): 746. CrossRef - Tentative Proposal of Optimal Timing of Kasai Operation for Biliary Atresia Based on Fibroscan Results
Hwa Young Lee, Young A Park, Seok Joo Han, Hong Koh
Korean Journal of Pediatric Gastroenterology and Nutrition.2011; 14(1): 74. CrossRef
- False-negative Hepatobiliary Scintigraphy for Biliary Atresia
Case Report
- Alagille Syndrome: A Case Report.
- Hyosup Shim, Chanil Park, Soon Il Kim, Young Nyun Park
- Korean J Pathol. 2004;38(1):56-59.
- 2,651 View
- 88 Download
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Abstract
PDF
- Alagille syndrome is a rare autosomal dominant disorder showing complicated systemic manifestations, although the hepatic manifestations are predominant in many patients. We report a case of Alagille syndrome in a male baby who presented with a heart murmur at 2 days old and his echocardiography showed aortic stenosis. He presented with acholic stool and jaundice at 3 months old and a liver biopsy revealed paucity of the interlobular bile duct in the portal tract. This progressed to cirrhosis, for which a liver transplantation was performed at 10 months old. The explanted liver showed biliary-type cirrhosis with severe cholestasis. There was an absence of the interlobular bile ducts on microscopic examination. Bile duct paucity, associated with cholestasis, a peculiar face (prominent forehead, deep-set eyes, pointed mandible and bulbous nasal tip), and cardiac anomaly were observed, which were consistent with Alagille syndrome. He died of heart failure.
Original Article
- Development of Intrahepatic Bile Duct in Human Embryos and Fetuses; Histologic and Immunohfstochemical Observations.
- Yeon Lim Suh, Je G Chi
- Korean J Pathol. 1994;28(1):8-21.
- 1,461 View
- 16 Download
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Abstract
PDF
- Sequential development pattern of the intrahepatic bile ducts is analyzed histologically and immunohistochemically using 50 embryonal and fetal liver specimens. Serial sections are made to reconstruct the intrahepatic biliary system, and monoclonal antibodies were used for differential antigenic expression of the biliary system. By 9 weeks of gestation a layer of small round cells, namely plate cells, became first noticed around large portal vein branches close to the hepatic hilum. These plate cells extended subsequently to more distal branches. The ductal plates became duplicated to contain cleft-like spaces and tubular structures. These tubules gradually became incorporated into surrounding connective tissue around the protal vein, and gave the appearance of the individualized bile duct. At term(40 weeks of gestation) the smallest branches of the portal vein were still surrounded by a discontinuous ductal plate. The ductal plate cells showed strong positive reaction for CAM 5.2 and AE1/AE3. They also expressed CK 19 and AE1 from 9 weeks of gestation on. The immunoreactivity of bile duct cells for cytokeratins persisted throughout the whole gestational period. Carcinoembryonic antigen was expressed along the luminal border of the bile duct, duplicated ductal plate and intrahepatic bile canaliculi. Laminin was demonstrated along the basement membrane of the bile duct cells from 9 weeks of gestation. Few duplicated ductal plates were composed of two different types of cells, duct-like cells and hepatocyte-like cells. The duct-like cells and hepatocyte-like cells showed same imrhunoreactivity with the hepatocytes and the bile duct cells, respectively, suggesting that the intrahepatic bile duct cells are actually coming from the hepatocytes around the branches of the portal vein.
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