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Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome

  1. Carla Colombo M.D.1,*,
  2. Pier Maria Battezzati2,
  3. Andrea Crosignani2,
  4. Alberto Morabito3,
  5. Diana Costantini1,
  6. Rita Padoan1,
  7. Annamaria Giunta1

Article first published online: 7 MAR 2007

DOI: 10.1002/hep.1840360613

Issue

Hepatology

Hepatology

Volume 36, Issue 6, pages 1374–1382, December 2002

Additional Information

How to Cite

Colombo, C., Battezzati, P. M., Crosignani, A., Morabito, A., Costantini, D., Padoan, R. and Giunta, A. (2002), Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome. Hepatology, 36: 1374–1382. doi: 10.1002/hep.1840360613

Author Information

  1. 1

    CF Center, Department of Pediatrics, University of Milan

  2. 2

    Cattedra di Medicina Interna II, University of Milan, Milan, Italy

  3. 3

    Istituto di Biometria e Statistica Medica, School of Medicine Ospedale San Paolo, University of Milan, Milan, Italy

*Department of Pediatrics, University of Milan, Via Commenda 9, 20122 Milan, Italy. fax: (39) 02 57992814

Publication History

  1. Issue published online: 7 MAR 2007
  2. Article first published online: 7 MAR 2007
  3. Manuscript Accepted: 18 SEP 2002
  4. Manuscript Received: 20 FEB 2002

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Reference

  • 1
    Fitzsimmons SC. The changing epidemiology of cystic fibrosis. J Pediatr 1993; 122: 1–9.
  • 2
    Narkewicz MR. Markers of cystic fibrosis—associated liver disease. J Pediatr Gastroenterol Nutr 2001; 32: 421–422.
  • 3
    Cystic Fibrosis Foundation. Patient Registry 2000 Annual Report. Bethesda, MD: Cystic Fibrosis Foundation, 2001.
  • 4
    Sokol RJ, Durie PR. Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. J Pediatr Gastroenterol Nutr 1999; 28 (Suppl 1): 1–13.
  • 5
    Feranchak AP, Sokol RJ. Cholangiocyte biology and cystic fibrosis liver disease. Semin Liver Dis 2001; 21: 471–488.
  • 6
    Kinnman N, Lindblad A, Housset C, Buentke E, Scheynius A, Strandvik B, Hultcrantz R, et al. Expression of cystic fibrosis transmembrane conductance regulator in liver tissue from patients with cystic fibrosis. Hepatology 2000; 32: 334–340.
    Direct Link:
  • 7
    Gaskin KJ, Waters DLM, Howman-Giles R, De Silva M, Earl JW, Martin HCO, Kan AE, et al. Liver disease and common bile-duct stenosis in cystic fibrosis. N Engl J Med 1988; 318: 340–346.
  • 8
    Colombo C, Battezzati PM, Podda M. Hepatobiliary disease in cystic fibrosis. Semin Liver Dis 1994; 14: 259–269.
  • 9
    Feigelson J, Anagnostopoulos C, Poquet M, Pecau Y, Munck A, Navarro J. Liver cirrhosis in cystic fibrosis. Therapeutic implications and long-term follow-up. Arch Dis Child 1993; 68: 653–657.
  • 10
    Debray D, Lykavieris P, Gauthier F, Dousset B, Sardet A, Munck A, Laselve H, et al. Outcome of liver cirrhosis in cystic fibrosis. Management of portal hypertension. J Hepatol 1999; 31: 77–83.
  • 11
    Laenerts C, Rault G. Natural history of liver disease and cirrhosis in cystic fibrosis [Abstract]. Pediatr Pulmonol 2000; 29: 318.
  • 12
    Lindblad A, Glaumann H, Strandvik B. Natural history of liver disease in cystic fibrosis. Hepatology 1999; 30: 1151–1158.
    Direct Link:
  • 13
    Ling SC, Wilkinson JD, Hollman AS, McColl J, Evans TJ, Paton JY. The evolution of liver disease in cystic fibrosis. Arch Dis Child 1999; 81: 129–132.
  • 14
    Hayllar KM, Williams SGJ, Wise AE, Pouria S, Lombard M, Hodson ME, Westaby D. A prognostic model for prediction of survival in cystic fibrosis. Thorax 1997; 52: 313–317.
  • 15
    Kerem E, Reisman J, Corey M, Canny GJ, Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med 1992; 326: 1187–1191.
  • 16
    Liou TG, Adler FR, Fitzsimmons SC, Cahill BC, Hibbs JR, Marshall BC. Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol 2001; 153: 345–352.
  • 17
    Knudson RJ, Lebowitz MD, Holberg CJ, Burrows B. Changes in the normal maximal expiratory flow-volume curve with growth and aging. Am Rev Respir Dis 1983; 127: 725–734.
  • 18
    Andersen JB, Qwist J, Kann T. Recruiting collapsed lung through collateral channels with positive end-expiratory pressure. Scand J Respir Dis 1979; 60: 260–266.
  • 19
    Colombo C, Crosignani A, Assaisso ML, Battezzati PM, Podda M, Giunta A, Zimmer-Nechemias L, et al. Ursodeoxycholic acid therapy in cystic fibrosis associated liver disease: a dose-response study. Hepatology 1992; 16: 924–930.
    Direct Link:
  • 20
    Kristidis P, Bozon D, Corey M, Markiewicz D, Rommens J, Tsui LC, Durie P. Genetic determination of exocrine pancreatic function in cystic fibrosis. Am J Hum Genet 1992; 50: 1178–1184.
  • 21
    Tanner JM, Whitehouse RH. Clinical longitudinal standards for height, weight, height velocity, weight velocity, and stages of puberty. Arch Dis Child 1976; 51: 170–179.
  • 22
    Colombo C, Crosignani A, Battezzati PM. Liver involvement in cystic fibrosis. J Hepatol 1999; 31: 946–954.
  • 23
    Scott-Jupp R, Lama M, Tanner MS. Prevalence of liver disease in cystic fibrosis. Arch Dis Child 1991; 66: 698–701.
  • 24
    Sokol RJ, Narkewicz MR, Carroll NM, Wagener JS, Accurso FJ. Prospective evaluation of liver blood tests during the first 8 years of life in children with cystic fibrosis identified by newborn screening [Abstract]. Hepatology 1994; 4: 402A.
  • 25
    Williams SGJ, Evanson JE, Barrett N, Hodson ME, Boultbee JE, Westaby D. An ultrasound scoring system for the diagnosis of liver disease in cystic fibrosis. J Hepatol 1995; 22: 513–521.
  • 26
    Patriquin H, Lenaerts C, Smith L, Perreault G, Grignon A, Filiatrault D, Boisvert J, et al. Liver disease in children with cystic fibrosis: US-biochemical comparison in 195 patients. Radiology 1999; 211: 229–232.
  • 27
    Joseph AE, Saverymuttu SH, al-Sam S, Cook MG, Maxwell JD. Comparison of liver histology with ultrasonography in assessing diffuse parenchymal liver disease. Clin Radiol 1991; 43: 26–31.
  • 28
    Willi UV, Reddish JM, Teele RL. Cystic fibrosis: its characteristic appearance on abdominal sonography. AJR Am J Roentgenol 1980; 143: 1005–1010.
  • 29
    Colombo C, Apostolo MG, Ferrari M, Seia M, Genoni S, Giunta A, Piceni Sereni L. Analysis of risk factors for the development of liver disease associated with cystic fibrosis. J Pediatr 1994; 124: 393–399.
  • 30
    Wilschanski M, Rivlin J, Cohen S, Augarten A, Blau H, Aviram M, Bentur L, et al. Clinical and genetic risk factors for CF-related liver disease. Pediatrics 1999; 103: 52–57.
  • 31
    Shapira R, Hadzic R, Francavilla R, Koukulis G, Price GF, Mieli-Vergani G. Retrospective review of cystic fibrosis presenting as infantile liver disease. Arch Dis Child 1999; 81: 125–128.
  • 32
    Lindblad A, Hjelte L, Strandvik B. Incidence of liver disease in patients with cystic fibrosis and meconium ileus. J Pediatr 1995; 125: 155.
  • 33
    Fuchs JR, Langer CL. Long-term outcome after neonatal meconium obstruction. Pediatrics 1998; 101: 1–6.
  • 34
    Kappler MJ, Lang T, Harms HK, Bertele-Harms RM. Regular screening for serum liver enzymes and appropriate early therapy with ursodeoxycholic acid delays severe liver disease in CF [Abstract]. Pediatr Pulmonol 2001; 22 (Suppl): 340.
  • 35
    Zielenski J. Genotype and phenotype in cystic fibrosis. Respiration 2000; 67: 117–133.
  • 36
    Friedman K, Ling SC, Macek M, Handler AJ, Zhou Z, Pace RG, Mack DR, et al. Complex multigenic inheritance influences the development of severe CF liver disease [Abstract]. Pediatr Pulmonol 2001; 22 (Suppl): 340.
  • 37
    Zielenski J, Corey M, Rozmahel R, Markiewicz D, Aznarez I, Casals T, Larriba S, et al. Detection of a cystic fibrosis modifier locus for meconium ileus on human chromosome 19q13 [Letter]. Nat Genet 1999; 22: 128–129.
  • 38
    Francavilla A, Polimeno L, Di Leo A, Barone M, Ove P, Coetzee M, Eagon PK, et al. The effect of estrogen and tamoxifen on hepatocyte proliferation in vivo and in vitro. Hepatology 1989; 9: 614–620.
    Direct Link:
  • 39
    Alvaro D, Alpini G, Onori P, Perego L, Svegliati Baroni G, Franchitto A, Baiocchi L, et al. Estrogens stimulate proliferation of intrahepatic biliary epithelium in rats. Gastroenterology 2000; 119: 1681–1691.
  • 40
    Duthie A, Doherty DG, Williams C, Scott-Jupp R, Waener JO, Tanner MS, Williamson R, et al. Genotype analysis for DF 508, G551D and R553X mutations in children and young adults with cystic fibrosis with and without liver disease. Hepatology 1992; 15: 660–664.
    Direct Link:
  • 41
    Kovesi T, Corey M, Tsui L-C, Levison H, Durie P. The association between liver disease and mutations of the cystic fibrosis gene [Abstract]. Pediatr Pulmonol 1992; 8 (Suppl): 244.
  • 42
    Sherlock S, Dooley J. Hepatic cirrhosis. In: Diseases of the liver and biliary system. 10th ed. Oxford: Blackwell Science, 1997; 371–384.
  • 43
    Cheng K, Ashby D, Smyth R. Ursodeoxycholic acid for cystic fibrosis related liver disease (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software, 2000.
  • 44
    Colombo C, Battezzati PM, Podda M, Bettinardi, Giunta A, and the Italian Group for the Study of Ursodeoxycholic acid in Cystic Fibrosis. Ursodeoxycholic acid for liver disease associated with cystic fibrosis: a double-blind multicenter trial. Hepatology 1996; 23: 1484–1490.
    Direct Link:
  • 45
    Morrison AL. The clinical course of screen-detected disease. In: Screening in chronic disease. 2nd ed. New York: Oxford University Press, 1992; 129–147.
  • 46
    Feinstein AR, Pritchett JA, Scimpff CR. The epidemiology of cancer therapy. II. The clinical course: data, decisions, and temporal demarcations. Arch Intern Med 1969; 123: 323–344.
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