{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T02:36:30Z","timestamp":1789698990509,"version":"4.0.1"},"reference-count":41,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2005,7,5]],"date-time":"2005-07-05T00:00:00Z","timestamp":1120521600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2005,8]]},"abstract":"<jats:p><jats:bold>Abstract: Background and Aims: <\/jats:bold> Congenital hepatic fibrosis (CHF) is characterized by dense portal\/septal fibrosis and bile duct proliferation and tortuosity. In this study, the roles and significance of fibrosis\u2010related cells and molecules in the process of progressive and unresolving fibrosis of CHF were examined in comparison with other fibrotic liver diseases.<\/jats:p><jats:p><jats:bold>Methods: <\/jats:bold> Seven CHF livers were examined, and a total of 74 control livers (chronic viral hepatitis (CVH), alcoholic fibrosis\/cirrhosis (F\/C), extrahepatic biliary obstruction and livers showing non\u2010specific reactive changes) were used as controls. All of these livers were wedge biopsied or surgically resected ones, and were formalin fixed and paraffin embedded. In addition to histologic observations, expression of heparan sulfate proteoglycan (HSPG), connective tissue growth factor (CTGF), mast cell\u2010specific tryptase, \u03b1\u2010smooth muscle actin for activated hepatic stellate cells (HSC) or myofibroblasts (MF) were immunohistochemically surveyed. HSPG and CTGF at mRNA were also examined by <jats:italic>in situ<\/jats:italic> hybridization.<\/jats:p><jats:p><jats:bold>Results: <\/jats:bold> Portal\/septal fibrosis of CHF were mature collagenous and elastic fiber poor, when compared with controls. HSPG and CTGF were diffusely abundant in fibrous portal tracts\/septa in CHF, while they were more or less accentuated at periportal areas in alcoholic F\/C and CVH. In CHF, the number of interface and portal\/septal MF was increased from mild\u2010to\u2010moderate degree, while their increase was moderate to marked in alcoholic F\/C and CVH, particularly F3\/F4. While activated HSC were frequent in alcoholic F\/C and CVH and they were continuous with interface MF, activated HSC in CHF were scanty. Instead, mast cells were increased in portal\/septal fibrosis of CHF. Portal mononuclear cells and endothelial cells were positive for HSPG mRNA, and mononuclear cells for CTGF mRNA, and such cells were accentuated around proliferated bile ducts and ductules in CHF.<\/jats:p><jats:p><jats:bold>Conclusions: <\/jats:bold> Abundant CTGF retained diffusely in HSPG in the fibrous portal tracts\/septa may be responsible for non\u2010resolving hepatic fibrosis in CHF, and many mast cells and portal MF not related to HSC may causally relate to such characteristic finding in CHF.<\/jats:p>","DOI":"10.1111\/j.1478-3231.2005.01067.x","type":"journal-article","created":{"date-parts":[[2005,7,5]],"date-time":"2005-07-05T18:53:21Z","timestamp":1120589601000},"page":"817-828","source":"Crossref","is-referenced-by-count":31,"title":["Diffuse expression of heparan sulfate proteoglycan and connective tissue growth factor in fibrous septa with many mast cells relate to unresolving hepatic fibrosis of congenital hepatic 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