2001, Number 4
<< Back Next >>
Rev Med Hosp Gen Mex 2001; 64 (4)
Importance of the ductal plate malformation in the development of congenital hepatic fibrosis. An immunohistochemical study
Escobar AJ, Aguirre GJ, Durán MA
Language: Spanish
References: 10
Page: 201-204
PDF size: 141.37 Kb.
ABSTRACT
Congenital hepatic fibrosis (CHF) is an autosomal recessive disease. Its morphological hallmark is the presence of fibrous enlargement of the portal tracts with middle sized bile ducts. This is an immunohistochemical study of the ductal plate and the role in the genesis of CHF. We studies four cases with CHF and histologic slides of liver from embryos of 8, 9 and 10 weeks of gestational age, using immunohistochemical stains (cytokeratin 7, 8, 18, 19 and laminin). The primitive hepatocytes around the portal veins shown ad reactivity for cytokeratin 7, 8, 18, 19 and laminin. The lack of remodelation of the ductal plate results in a persistence of the embryonal bile ducts and the anomalies of the ductal plate may be responsible of congenital diseases of intrahepatic bile ducts including CHF. The results demonstrate that the cells that develop the ductal plate show reactivity for cytokeratin 8, 18, 19 and laminin at the 8th week of gestational age, and this reactivity persists in the bile ducts in the CHF. These findings support the hypothesis that the development of CHF is due to the lack of remodelation of the ductal plate.
REFERENCES
Desmet V. What is congenital hepatic fibrosis? Hepatology 1992; 20: 465-477.
Desmet V. Congenital diseases of intrahepatic bile ducts: variations on the theme “ductal plate malformation”. Hepatology 1992; 4: 1069-1083.
Lieberman E, Salinas L, Gwinn J, Brennan L, Fine R, Landing B. Infantile polycystic disease of the kidneys and liver: clinical, pathological and radiological correlations and comparison with congenital hepatic fibrosis. Medicine 1971; 4: 277-318.
Nishimura G, Nakayama M, Fuke Y, Suehara N. A letal osteochondrodysplasia with mesomelic brachymelia, round pelvis, and congenital hepatic fibrosis: two siblings born to consanguineous parents. Pediatr Radiol 1998; 28: 43-47.
Bayraktar Y, Tuncer Z, Kabukcu A, Uzunalimoglu B, Ayhan A. Pregnancy complicated by congenital hepatic fibrosis with cavernous transformation of the portal vein: A case report. Am J Obstet Gynecol 1997; 2: 459-461.
Fujiwara Y, Ohizumi T, Kakizaki G, Fujiwara T. Congenital dilatation of intrahepatic and common bile ducts with congenital hepatic fibrosis. J Pediatr Surg 1976; 2: 273-274.
Tazelaar H, Payne J, Patel N. Congenital hepatic fibrosis and asymptomatic familial adult-type polycystic kidney disease in a 19-year-old woman. Gastroenterology 1984; 86: 757-760.
Eyken P, Sciot R, Callea F, Van Der Steen K, Moerman P, Desmet V. The development of the intrahepatic bile ducts in man: A keratin-immunohistochemical study. Hepatology 1988; 6: 1586-1595.
Shah K, Gerber M. Development of intrahepatic bile ducts in humans. Arch Pathol Lab Med 1990; 114: 597-600.
Stosiek P, Kasper M, Karsten U. Expression of cytokeratin 19 during human liver organogenesis. Liver 1990; 10: 59-63.