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The American Journal of Digestive Diseases

, Volume 19, Issue 4, pp 325–333 | Cite as

Congenital hepatic fibrosis

Two cases simulating hepatic cirrhosis
  • Hernan A. Campana
  • Yung S. Park
  • George D. Gourgoutis
Original Articles

Abstract

Congenital hepatic fibrosis (CHF) was detected in a 10-year-old boy with hepatosplenomegaly and leukopenia and in a 45-year-old man with hepatomegaly. Several years after these original findings, both patients developed bleeding that was attributed to esophageal varices and were treated by elective portasystemic shunts. In both, the preshunt liver function tests were essentially normal and the liver scans revealed homogeneous uptake. The gross aspect of the livers at laparotomy was indistinguishable from that of cirrhosis, and the liver wedge biopsies showed generalized portal and interlobular fibrosis and bile-duct hyperplasia but no evidence of previous liver-cell necrosis. Hepatic vein catheterization and portal venography in the adult patient indicated intrahepatic presinusoidal block. Neither patient had jaundice, ascites, or polycystic kidneys. Both cases appear sporadic.

Keywords

Liver Function Liver Function Test Hepatic Vein Leukopenia Esophageal Varix 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References

  1. 1.
    MacMahon HE: Congenital anomalies of the liver. Am. J Pathol 5:499–508, 1929Google Scholar
  2. 2.
    McCarthy LJ, Baggenstoss AH, Logan GB: Congenital hepatic fibrosis. Gastroenterology 49:27–36, 1965Google Scholar
  3. 3.
    Sommerschild HC, Langmark F, Maureeth K: Congenital hepatic fibrosis: report of two new cases and review of the literature. Surgery 73:53–58, 1973Google Scholar
  4. 4.
    Parker RGF: Fibrosis of the liver as a congenital anomaly. J Pathol Bacteriol 71:359–368, 1956Google Scholar
  5. 5.
    Kerr DN, Harrison CV, Sherlock S, Walker RM: Congenital hepatic fibrosis. Q J Med 30:91–117, 1961Google Scholar
  6. 6.
    Farmer RG, Hermann RE, Sullivan BH Jr: Congenital hepatic fibrosis of Riedel's lobe causing portal hypertension: report of a case. Cleve Clin Q 36:143–148, 1969Google Scholar
  7. 7.
    Maddrey WC, Iber FL: Familial cirrhosis: a clinical and pathological study. Ann Intern Med 61:667–679, 1964Google Scholar
  8. 8.
    Murray-Lyon IM, Shilkin KB, Laws JW, Illing RC, Williams R: Non-obstructive dilatation of the intrahepatic biliary tree with cholangitis. Q J Med 41:477–489, 1972Google Scholar
  9. 9.
    Schaefer JW, Schiff L, Gall EA, Oikawa Y: Progression of acute hepatitis to post-necrotic cirrhosis. Am J Med 42:348–358, 1967Google Scholar
  10. 10.
    Leevy CM, Cherrick GR, Davidson CS: Portal hypertension. N Engl J Med 262:397–403, 1960Google Scholar
  11. 11.
    McAfee JG, Ause RG, Wagner HN Jr: Diagnostic value of scintillation scanning of the liver. Arch Intern Med 116:95–110, 1965Google Scholar
  12. 12.
    Zeegen R, Stansfeld AG, Dawson AM, Hunt AH: Prolonged survival after portal decompression of patients with noncirrhotic intrahepatic portal hypertension. Gut 11:610–617, 1970Google Scholar
  13. 13.
    Ruggieri BA, Baggenstoss AH, Logan GB: Juvenile cirrhosis: a clinicopathologic study of twenty-seven cases. Am J Dis Child 94:64–76, 1957Google Scholar
  14. 14.
    Sherlock S: Hepatolenticular degeneration, Diseases of the Liver and Biliary System, 4th ed, Philadelphia, FA Davis Co, 1968, pp 456–466Google Scholar
  15. 15.
    Cohen KL, Rubin PE, Echevarria RA, Sharp HL, Teague PO, Alpha 1-antitrypsin deficiency, emphysema, and cirrhosis in an adult. Ann Intern Med 78:227–232, 1973Google Scholar
  16. 16.
    Talamo RC, Feingold M: Infantile cirrhosis with hereditary α-antitrypsim deficiency. Am J Dis Child 125:845–847, 1973Google Scholar
  17. 17.
    Smetana HF, Olen E: Hereditary galactose disease. Am J Clin Pathol 38:3–25, 1962Google Scholar
  18. 18.
    Murray-Lyon IM, Ockenden BG, Williams R: Congenital hepatic fibrosis—is it a single clinical entity? Gastroenterology 64:653–656, 1973Google Scholar
  19. 19.
    Popper H, Schaffner F: Review of the clinical problems and classification of cirrhosis, Liver: Structure and Function, New York, McGraw-Hill Co, 1957, pp 520–531Google Scholar

Copyright information

© Digestive Disease Systems, Inc. 1974

Authors and Affiliations

  • Hernan A. Campana
    • 1
    • 2
  • Yung S. Park
    • 1
    • 2
  • George D. Gourgoutis
    • 1
    • 2
  1. 1.Laboratory and Medical ServicesVeterans Administration CenterDayton
  2. 2.the Ohio State University College of MedicineColumbus

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