Search tips
Search criteria 


Logo of jrsocmedLink to Publisher's site
J R Soc Med. 1992; 85(Suppl 19): 20–24.
PMCID: PMC1295449

Liver and biliary problems in cystic fibrosis.

Full text

Full text is available as a scanned copy of the original print version. Get a printable copy (PDF file) of the complete article (1.0M), or click on a page image below to browse page by page. Links to PubMed are also available for Selected References.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.
  • Tanner MS. Current clinical management of hepatic problems in cystic fibrosis. J R Soc Med. 1986;79 (Suppl 12):38–43. [PMC free article] [PubMed]
  • Setchell KD, Smethurst P, Giunta AM, Colombo C. Serum bile acid composition in patients with cystic fibrosis. Clin Chim Acta. 1985 Sep 30;151(2):101–110. [PubMed]
  • Fondacaro JD, Heubi JE, Kellogg FW. Intestinal bile acid malabsorption in cystic fibrosis: a primary mucosal cell defect. Pediatr Res. 1982 Jun;16(6):494–498. [PubMed]
  • Thompson GN, Davidson GP. In vivo bile acid uptake from terminal ileum in cystic fibrosis. Pediatr Res. 1988 Mar;23(3):323–328. [PubMed]
  • de Rooij FW, van den Berg JW, Sinaasappel M, Bosman-Jacobs EP, Touw-Blommesteijn AC. Bile acid malabsorption in cystic fibrosis; membrane vesicles, a tool for revealing the role of the ileal brush border membrane. Acta Paediatr Scand Suppl. 1985;317:28–30. [PubMed]
  • Thompson GN. Excessive fecal taurine loss predisposes to taurine deficiency in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1988 Mar-Apr;7(2):214–219. [PubMed]
  • Weizman Z, Durie PR, Kopelman HR, Vesely SM, Forstner GG. Bile acid secretion in cystic fibrosis: evidence for a defect unrelated to fat malabsorption. Gut. 1986 Sep;27(9):1043–1048. [PMC free article] [PubMed]
  • Robb TA, Davidson GP, Kirubakaran C. Conjugated bile acids in serum and secretions in response to cholecystokinin/secretin stimulation in children with cystic fibrosis. Gut. 1985 Nov;26(11):1246–1256. [PMC free article] [PubMed]
  • Nathanson MH, Boyer JL. Mechanisms and regulation of bile secretion. Hepatology. 1991 Sep;14(3):551–566. [PubMed]
  • Ishikawa T, Müller M, Klünemann C, Schaub T, Keppler D. ATP-dependent primary active transport of cysteinyl leukotrienes across liver canalicular membrane. Role of the ATP-dependent transport system for glutathione S-conjugates. J Biol Chem. 1990 Nov 5;265(31):19279–19286. [PubMed]
  • Keppler D, Hagmann W, Rapp S, Denzlinger C, Koch HK. The relation of leukotrienes to liver injury. Hepatology. 1985 Sep-Oct;5(5):883–891. [PubMed]
  • Gaskin KJ, Waters DL, Howman-Giles R, de Silva M, Earl JW, Martin HC, Kan AE, Brown JM, Dorney SF. Liver disease and common-bile-duct stenosis in cystic fibrosis. N Engl J Med. 1988 Feb 11;318(6):340–346. [PubMed]
  • Nagel RA, Westaby D, Javaid A, Kavani J, Meire HB, Lombard MG, Wise A, Williams R, Hodson ME. Liver disease and bile duct abnormalities in adults with cystic fibrosis. Lancet. 1989 Dec 16;2(8677):1422–1425. [PubMed]
  • Gaskin KJ, Waters DL, de Silva M, Martin H, Howman-Giles R, Dorney S. Liver disease and bileduct stenosis in cystic fibrosis. Lancet. 1990 May 5;335(8697):1098–1098. [PubMed]
  • Bilton D, Fox R, Webb AK. Liver disease and biliary tract abnormalities in cystic fibrosis. Lancet. 1990 Feb 10;335(8685):357–357. [PubMed]
  • Bilton D, Fox R, Webb AK, Lawler W, McMahon RF, Howat JM. Pathology of common bile duct stenosis in cystic fibrosis. Gut. 1990 Feb;31(2):236–238. [PMC free article] [PubMed]
  • Johansen HK, Nir M, Høiby N, Koch C, Schwartz M. Severity of cystic fibrosis in patients homozygous and heterozygous for delta F508 mutation. Lancet. 1991 Mar 16;337(8742):631–634. [PubMed]
  • Scott-Jupp R, Lama M, Tanner MS. Prevalence of liver disease in cystic fibrosis. Arch Dis Child. 1991 Jun;66(6):698–701. [PMC free article] [PubMed]
  • Maurage C, Lenaerts C, Weber A, Brochu P, Yousef I, Roy CC. Meconium ileus and its equivalent as a risk factor for the development of cirrhosis: an autopsy study in cystic fibrosis. J Pediatr Gastroenterol Nutr. 1989 Jul;9(1):17–20. [PubMed]
  • McHugo JM, McKeown C, Brown MT, Weller P, Shah KJ. Ultrasound findings in children with cystic fibrosis. Br J Radiol. 1987 Feb;60(710):137–141. [PubMed]
  • Vergesslich KA, Götz M, Mostbeck G, Sommer G, Ponhold W. Portal venous blood flow in cystic fibrosis: assessment by Duplex Doppler sonography. Pediatr Radiol. 1989;19(6-7):371–374. [PubMed]
  • Patriquin H, Lafortune M, Burns PN, Dauzat M. Duplex Doppler examination in portal hypertension: technique and anatomy. AJR Am J Roentgenol. 1987 Jul;149(1):71–76. [PubMed]
  • Patriquin H, Tessier G, Grignon A, Boisvert J. Lesser omental thickness in normal children: baseline for detection of portal hypertension. AJR Am J Roentgenol. 1985 Oct;145(4):693–696. [PubMed]
  • Gleeson D, Ruppin DC, Saunders A, Murphy GM, Dowling RH. Final outcome of ursodeoxycholic acid treatment in 126 patients with radiolucent gallstones. Q J Med. 1990 Jul;76(279):711–729. [PubMed]
  • Colombo C, Setchell KD, Podda M, Crosignani A, Roda A, Curcio L, Ronchi M, Giunta A. Effects of ursodeoxycholic acid therapy for liver disease associated with cystic fibrosis. J Pediatr. 1990 Sep;117(3):482–489. [PubMed]
  • Cotting J, Lentze MJ, Reichen J. Effects of ursodeoxycholic acid treatment on nutrition and liver function in patients with cystic fibrosis and longstanding cholestasis. Gut. 1990 Aug;31(8):918–921. [PMC free article] [PubMed]
  • Marteau P, Chazouilléres O, Myara A, Jian R, Rambaud JC, Poupon R. Effect of chronic administration of ursodeoxycholic acid on the ileal absorption of endogenous bile acids in man. Hepatology. 1990 Nov;12(5):1206–1208. [PubMed]
  • Nakagawa M, Colombo C, Setchell KD. Comprehensive study of the biliary bile acid composition of patients with cystic fibrosis and associated liver disease before and after UDCA administration. Hepatology. 1990 Aug;12(2):322–334. [PubMed]
  • Mieles LA, Orenstein D, Teperman L, Podesta L, Koneru B, Starzl TE. Liver transplantation in cystic fibrosis. Lancet. 1989 May 13;1(8646):1073–1073. [PubMed]
  • Cox KL, Ward RE, Furgiuele TL, Cannon RA, Sanders KD, Kurland G. Orthotopic liver transplantation in patients with cystic fibrosis. Pediatrics. 1987 Oct;80(4):571–574. [PubMed]

Articles from Journal of the Royal Society of Medicine are provided here courtesy of Royal Society of Medicine Press