Journal of Pathology and Translational Medicine

Original Articles

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

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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

Histopathologic Appearance of Cytomegaloviral Liver Diseases in Neonates and Infants.: Sun Hee Sung, Chan Il Park, Ho Guen Kim, Woo Hee Jung, Ki Sep Chung; Korean J Pathol. 1992;26(2):137-145.

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Abstract PDF: To provide ideas for the recognition of neonatal and infantile liver diseases caused by cytomegalovirus(CMV) infection, histopathological examinations were made on hepatic tissues obtained by biopsy or autopsy from 23 patients. All patients were sero-positive for IgM anti CMV and had no other known or suggested etiologic factors for their liver disease. There were five different types of liver diseases: 8 cases of giant cell hepatitis(34.8%), 4 cases of biliary atresia(17.4%), 5 cases of biliary atresia with changes of neonatal hepatitis(21.7%), 4 cases of diffuse hepatic fibrosis(17.4%) and 2 cases of hepatic necrosis with CMV inclusion(8.7%). The diffuse hepatic fibrosis involved both the hepatic lobules and portal areas without evidences of regeneration. This type of liver disease appeared to be a chronic progressive illness that began during the first week of life, and in 3 of 4 cases, the liver biopsy was dong at 5 to 9 months after birth. The two patients showing CMV inclusion in their liver were premature of debilitated, and died within I month after birth. Diffuse hepatic necrosis as well as the cytomegalic change of bile duct epithelium was characteristic. The findings suggest that the pattern of CMV liver disease depends on the major site of hepatic injury, the status of status of patient's defense mechanism and the chronicity of illness.

Neonatal Hepatitis and Extrahepatic Biliary Atresia : A Comparison by Scoring the Histological Parameters.: Sun Hee Sung, Woo Hee Jung, Ho guen Kim, Ki Sup Jeong, Chanil Park; Korean J Pathol. 1991;25(5):446-456.

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Abstract: Neonatal hepatitis(NH) and congenital extrahepatic biliary atresia(BA) are two major causes of neonatal cholestasis. The method of therapeutic trials for each disease is essentially different. Nonetheless it is very difficult to differentiate these diseases histologically, since most of the hepatic changes are mutual in both of them. This study is to aimed to find out major differences between the two by scoring various histological parameters. A total of 63 consecutive liver biopsies taken from 54 patients with suggested NH and BA were examined by applying morphometric scoring system. The detailed clinical histories, laboratory data including serology for HBsAg and TORCH infection and radiologic operative findings were reviewed. Among 54 patients, 27 were diagnosed as NH and 20 as BA. In two cases, features of both diseases were coexistent. The pathological diagnosis was not compatible with the final diagnosis in 5 cases(10.7%). In all of these 5 cases, biopsy had been performed at the age of one to two months. The seropositivity for TORCH was 59.3%(16.27) in NH, but 25.0%(5/20) in BA. Serum AST, ALT and alpha-fetoprotein values were higher in NH, and total bilirubin in BA. Of various histological parameters, scores of portal fibrosis, bile duct and ductular proliferation and bile thrombi were much higher in BA, and at the age of less than 2 months, extramedullary hemopoiesis(EMH) was found much more frequently in NH. Giant cell transformation of hepatocytes(GCT) was more commonly observed in NH. The numbers of GCT and EMH were particulary plentiful when the patients' sera were positive for HBsAg or TORCH. These results indicate that portal fibrosis, biliary proliferation and bile thrombi are the three major histologic features of BA, and therefore erroneous histological diagnosis may ensue when scores of those features are low as in some early BA.

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