Congenital hepatic fibrosis with saccular dilatation of intrahepatic bile ducts and infantile polycystic kidneys

1986 ◽  
Vol 16 (4) ◽  
pp. 302-305 ◽  
Author(s):  
C. H. Davies ◽  
D. A. Stringer ◽  
H. Whyte ◽  
A. Daneman ◽  
K. Mancer
Keyword(s):  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Congenital Hepatic Fibrosis ◽  
Polycystic Kidneys ◽  
Intrahepatic Bile Ducts

scholarly journals Congenital Hepatic Fibrosis With Dilation of Intrahepatic Bile Ducts

1976 ◽  
Vol 71 (5) ◽  
pp. 839-843 ◽  
Author(s):  
M. Dusol ◽  
J.U. Levi ◽  
K. Glasser ◽  
E.R. Schiff
Keyword(s):  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Congenital Hepatic Fibrosis ◽  
Intrahepatic Bile Ducts

Matrix proteins of basement membrane of intrahepatic bile ducts are degraded in congenital hepatic fibrosis and Caroli's disease

2009 ◽  
Vol 217 (3) ◽  
pp. 442-451 ◽  
Author(s):  
Mitsue Yasoshima ◽  
Yasunori Sato ◽  
Shinichi Furubo ◽  
Kazuo Kizawa ◽  
Takahiro Sanzen ◽  
...  
Keyword(s):  
Basement Membrane ◽  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Matrix Proteins ◽  
Congenital Hepatic Fibrosis ◽  
Caroli's Disease ◽  
Caroli’S Disease ◽  
Intrahepatic Bile Ducts

scholarly journals Caroli Syndrome in a Child: A Case Report

2019 ◽  
Author(s):  
Pantea Tajik ◽  
Amir Hossein Goudarzian
Keyword(s):  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Definitive Treatment ◽  
Congenital Hepatic Fibrosis ◽  
Portal Vein Pressure ◽  
Abdominal Sonography ◽  
Case Presentation ◽  
Intrahepatic Bile Ducts ◽  
Caroli Syndrome ◽  
Multiple Cysts

Introduction: Caroli syndrome is a congenital disorder characterized by multiple segmental or saccular dilatations of the intrahepatic bile ducts associated with congenital hepatic fibrosis. Case Presentation: A 3-year-old boy with abdominal distention was referred to gastroentrology ward of Amiralmomenin hospital (Semnan, Iran) in summer 2018. In his abdominal sonography, multiple cysts were detected in the liver with hepatomegaly, and the portal vein pressure was 10 mm. Also, in liver biopsy, dilated portal bile ducts (trichrome stain) with inspissated bile and congenital hepatic fibrosis were reported. He was discharged after conservative therapy and followed up. Conclusion: Definitive treatment, i.e surgery, should be offered to prevent future complications.

scholarly journals Congenital hepatic fibrosis associated with ascites in a nellore calf

2020 ◽  
Vol 50 (5) ◽  
Author(s):  
Daniela Dantas de Gois ◽  
Isabelle Vieira de Sousa ◽  
Francisca Maria Sousa Barbosa ◽  
José Ferreira da Silva Neto ◽  
Rubia Avlade Guedes Sampaio ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Bile Duct ◽  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Autosomal Recessive ◽  
Variable Degree ◽  
Congenital Hepatic Fibrosis ◽  
Pathological Findings ◽  
Bile Duct Proliferation ◽  
Subcutaneous Edema

ABSTRACT: Congenital hepatic fibrosis (CHF) is an autosomal recessive malformation characterized by a variable degree of fibrosis and bile duct proliferation, mainly described in people and rarely reported in bovine European breeds. In addition to liver fibrosis, this syndrome has been associated with ascites and subcutaneous edema in calves. This paper described the pathological findings of the first report of CHF in a Nelore bovine fetus. A stillborn calf was removed by cesarean section because of dystocia. At necropsy, characteristic changes of CHF were observed, such as a large increase in abdominal volume associated with hepatic fibrosis and marked subcutaneous edema. Histological examination of liver revealed periportal and port-portal islands of fibrosis separating the parenchyma into nodules of variable sizes and containing numerous abnormally shaped bile ducts. The CHF should be considered in the differential diagnosis in young calves that present with ascites.

scholarly journals Congenital hepatic fibrosis and its mimics: a clinicopathologic study of 19 cases at a single institution

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Irene Y. Chen ◽  
Christa L. Whitney-Miller ◽  
Xiaoyan Liao
Keyword(s):  
Kidney Disease ◽  
Hepatic Fibrosis ◽  
Kidney Diseases ◽  
Wide Spectrum ◽  
Nodular Regenerative Hyperplasia ◽  
Congenital Hepatic Fibrosis ◽  
Clinicopathologic Features ◽  
Polycystic Kidney ◽  
Intrahepatic Bile Ducts ◽  
Clinicopathologic Study

Abstract Background Congenital hepatic fibrosis (CHF) is a rare inherited form of ductal plate malformation associated with polycystic kidney disease. The diagnosis requires histopathologic confirmation, but can be challenging to distinguish from other undefined fibrocystic liver diseases. We aimed to describe the clinicopathologic features of congenital hepatic fibrosis (CHF), with comparisons to other entities that may clinically and/or histologically mimic CHF. Methods Nineteen cases that carried a clinical and/or histologic impression of CHF were identified at our institution, of which the histology was reassessed and reappraised into two categories: CHF (n=13) and mimics (n=6). The clinicopathologic features between the two groups were analyzed and compared. Results The CHF group was further sub-classified into those with clinical suspicion (CHF-c, n=8) and those as incidental histology findings (CHF-i, n=5). Patients of CHF-i were much older than CHF-c or mimics (P<0.05). Male and female were equally affected. Six of 8 CHF-c (66.7%) had concurrent kidney diseases, including 5 polycystic kidney diseases. Five of 6 mimics (83.3%) had various kidney diseases, including nephronophthisis, Alport syndrome, renal agenesis, and nephrolithiasis. None of the CHF-i patients had kidney disease, but 3 were associated with hepatic carcinomas. Histology analysis demonstrated characteristic triads (bile duct abnormalities, portal vein hypoplasia, and fibrosis) in all CHF cases. One mimic had paucity of intrahepatic bile ducts, while the other 5 mimics showed abnormal portal veins and nodular regenerative hyperplasia consistent with hepatoportal sclerosis (HPS). Conclusions Our study demonstrates classic histology triad of CHF despite a wide spectrum of clinical presentations. HPS is unexpectedly a clinical mimicker of CHF, which can be distinguished histologically.

Congenital dilatation of intrahepatic and common bile ducts with congenital hepatic fibrosis

1976 ◽  
Vol 11 (2) ◽  
pp. 273-274 ◽  
Author(s):  
Yoshiyuki Fujiwara ◽  
Tetsunosuke Ohizumi ◽  
Goro Kakizaki ◽  
Tetsuro Fujiwara
Keyword(s):  
Hepatic Fibrosis ◽  
Bile Ducts ◽  
Congenital Hepatic Fibrosis
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