Summary
The familial type of cholangiodysplastic pseudocirrhosis is presented. This chronic liver disease is caused by malformation of the intrahepatic bile ducts. The female infant was 5 months old when the diagnosis was established. The liver biopsy was studied by light and electron microscopy. Electron microscopic examination revealed active proliferation of ductular cells and progression of fibrogenesis, findings consistent with the rapid and fatal course of the disease. In the case presented an acute cholangitis occurred, but after healing the progression of the original process led to hepatic insufficiency. It is suggested that cholangiodysplastic pseudocirrhosis is a chronic, progressive liver disease the course of which might be hastened by the complication of cholangitis; the process itself causes liver cirrhosis without inflammation.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Albukerk, J., Duffy, J. L.: Fibrogenesis in congenital hepatic fibrosis. An electron and light microscopic study. Arch. Path. 92, 126–135 (1971)
Althoff, H.: Über familiäres Vorkommen intrahepatischer Gallengangswucherungen (cholangiodysplastische Pseudoleberzirrhose). Zb. allg. Path. path. Anat. 105, 324–330 (1964)
Balázs, M.: Elektronenmikroskopische Untersuchungen der Leber bei experimenteller Cholestase. Exp. Path. 5, 275–285 (1971)
Balázs, M.: Liver injury induced by alpha-naphthyl-isothiocyanate. Acta morph. Acad. Sci. hung. 19, 213–231 (1971)
Balázs, M., Lukács, V. F., Dénes, J.: Morphologische und klinische Angaben zur kongenitalen Gallenwegsatresie der Neugeborenen. Acta paediat. Acad. Sci. hung. 11, 323–335 (1970)
Balázs, M., Várkonyi, S.: Elektronenmikroskopische Untersuchungen in Fällen von chronischaggressiver Hepatitis. Acta Hepato-Gastroent. 20, 339–409 (1973)
Cain, H., Kraus, B.: Hepatozelluläre Carcinome im Säuglings- und Kindesalter bei angeborenen intrahepatischen Gallengangsfehlbildungen. Verh. dtsch. path. Ges. 55, 298–304 (1971)
Erlinger, S., Dhumeaux, D.: Mechanism and control of secretion of bile water and electrolytes. Gastroenterology 66, 281–304 (1974)
Essbach, H.: Paidopathologie. p. 222. Leipzig: VEB G. Thieme 1961
Fisher, J., Mekhjian, H., Pritchett, E. L. C., Charme, L. S.: Polycystic liver disease: Studies on the mechanism of cyst fluid formation. A case report. Gastroenterology 58, 423–429 (1974)
Foulk, W. T.: Congenital malformations of the intrahepatic biliary tree in the adult. Gastroenterology 58, 253–261 (1970)
Goldschmidt, B., Haas, M., Bajtai, A.: Portalis hypertensioval szövödött cholangiodysplasiás congenitalis pseudocirrhosis. Gyermekgyógyászat 20, 266–269 (1969)
Heymsfield, S.: Cystic dilatation of the intrahepatic biliary tree (Caroli's disease)-a suggested treatment. Gastroenterology 64, 663–671 (1973)
Huchzermeyer, H., Gerhard, L.: Zur Diagnose und Differentialdiagnose von Gallengangsadenomen (Mikrohamartomen) der Leber. Leber Magen Darm 2, 227–230 (1972)
Jorgensen, M.: Three-dimensional reconstruction of intrahepatic bile ducts in a case of polycystic disease of the liver in an infant. Acta path. microbiol. scand. Sect. A 80, 201–206 (1972)
Kerr, D. N. S., Harrison, C. V., Sherlock, S., Walker, R. M.: Congenital hepatic fibrosis. Quart. J. Med. 30, 91–96 (1961)
Korányi, Gy., Mészáros, J.: Congenitalis májfibrosis. Orv. Hetil. 112, 1588–1591 (1971)
Kovács, I., Donáth, O., Szamosi, J., Tóth, J., Madarász, J.: Cacchiés Ricci syndroma elöfordulása testvérpáron Gyermekgyógyászat 20, 261–266 (1969)
Krausz (Krasznai), G.: A maj cholangiodysplasiás congenitalis pseudocirrhosisa. Morph. Ig. Orv. Szemle 4, 281–285 (1964)
Longmire, W. P., Mandiola, S. A., Gordon, H. E.: Congenital cystic disease of the liver and biliary system. Ann. Surg. 174, 711–726 (1971)
Lukács, V. F., Balázs, M., Dénes, J.: Angaben zum Krankheitsbild des “eingedichten Gallen-Syndrom” (inspissated bile syndrome). Acta paediat. Acad. Sci. hung. 13, 139–150 (1972)
Murray-Lyon, I. M., Ockenden, B. G., Williams, R.: Congenital hepatic fibrosis—is it a single clinical entity? Gastroenterology 64, 653–656 (1973)
Sasaki, H., Schaffner, F., Popper, H.: Bile ductules in cholestasis: Morphologic evidence for secretion and absorption in man. Lab. Invest. 16, 84–95 (1967)
Sherlock, Sh.: Diseases of the Liver and Biliary System, p. 534. Oxford: Blackwell 1968
Sommerschild, H. C., Langmark, F., Maurseth, K.: Congenital hepatis fibrosis: Report of two new cases and review of the literature. Surgery 78, 53–58 (1973)
Sternlieb, I.: Functional implications of human portal and bile ductular ultrastructure. Gastroenterology 63, 321–327 (1972)
Strauch, G., Heber, J., Willnow, U.: Zur Genese der intrahepatischen Gallengangsdysplasien. Frankfurt. Z. Path. 74, 205–216 (1964)
Thaler, M. M., Ogata, E. S., Goodman, J. R., Pel, C. F., Korobkin, M. T.: Congenital fibrosis and polycystic disease of liver and kidneys. Amer. J. Dis. Child. 126, 374–380 (1973)
Turnberg, L. A., Jones, E. A., Sherlock, S.: Biliary secretion in a patient with cystic dilatation of the intrahepatic biliary tree. Gastroenterology 54, 1155–1163 (1968)
Wohlgemuth, B., Mühl, H.: Simultane Lebercirrhosen und Cystennieren im Kindesalter. Virchows Arch. path. Anat. 336, 470–476 (1963)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Balázs, M., Lukács, V.F., Dénes, J. et al. Cholangiodysplastic pseudocirrhosis. Virchows Arch. A Path. Anat. and Histol. 368, 61–71 (1975). https://doi.org/10.1007/BF00432167
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00432167