247
Views
30
CrossRef citations to date
0
Altmetric
ORIGINAL ARTICLE

Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome)

, , , &
Pages 465-471 | Received 20 Apr 2005, Published online: 26 Aug 2009

References

  • Aagenaes O, Hagen van der CB, Refsum R. Hereditary recurrent intrahepatic cholestasis from birth. Arch Dis Child 1968; 43: 646–57
  • Bull LN, Roche E, Song EJ, Pedersen J, Knisely AS, Der Hagen C, et al. Mapping of the locus for cholestasis-lymphoedema syndrome (Aagenaes syndrome) to a 6.6-CM interval on chromosome 15q. Am J Hum Genet 2000; 67: 994–9
  • Aagenaes O. Hereditary cholestasis with lymphoedema (Aagenaes syndrome, cholestasis-lymphoedema syndrome): new cases and follow-up from infancy to adult age. Scand J Gastroenterol 1998; 33: 335–45
  • Sigstad H, Aagenaes O, Bjorn-Hansen RW, Rootwelt K. Primary lymphoedema combined with hereditary recurrent intrahepatic cholestasis. Acta Med Scand 1970; 188: 213–9
  • Aagenaes O, Medbo S. Arvelig intrahepatisk gallestase med Lymfødem. Tidsskr Nor Lægeforen 1993; 30: 3673–7
  • Joven J, Clivelle X, Camps J, Espinel E, Simo J, Vilella E, et al. Plasma protein abnormalities in nephrotic syndrome: effect on plasma colloid osmotic pressure and viscosity. Clin Chem 1997; 43: 1223–31
  • Knisely AS. Progressive familial intrahepatic cholestasis: a personal perspective. Pediatr Dev Pathol 2000; 3: 113–25
  • Sveger T, Eriksson S. The liver in adolescents with alpha(1)-antitrypsin deficiency. Hepatology 1995; 22: 514–7
  • Lykavieris P, Hadchouel M, Chardot C, Bernard O. Outcome of liver disease in children with Alagille syndrome: a study of 163 patients. Gut 2001; 49: 431–5
  • Hoffenberg EJ, Narkewicz MR, Sondheimer JM, Smith DJ, Silverman A, Sokol RJ. Outcome of syndromic paucity of interlobular bile ducts (Alagille syndrome) with onset of cholestasis in infancy. J Pediatr 1995; 127: 220–4
  • Emerick KM, Rand EB, Goldmuntz E, Krantz ID, Spinner NB, Piccoli DA. Features of Alagille syndrome in 92 patients: frequency and relation to prognosis. Hepatology 1999; 29: 822–9
  • Quiros-Tejeira RE, Ament ME, Heyman MB, Martin MG, Rosenthal P, Hall TR, et al. Variable morbidity in Alagille syndrome: a review of 43 cases. J Pediatr Gastroenterol Nutr 1999; 29: 431–7
  • Whitington PF, Freese DK, Alonso EM, Schwarzenberg SJ, Sharp HL. Clinical and biochemical findings in progressive familial intrahepatic cholestasis. J Pediat Gastroenterol Nutr 1994; 18: 134–41
  • Bourke B, Goggin N, Walsh D, Kennedy S, Setchell KDR, Drumm B. Byler-like familial cholestasis in an extended kindred. Arch Dis Child 1996; 75: 223–7
  • Nielsen IM, Ornvold K, Brock Jacobsen B, Ranek L. Fatal familial cholestatic syndrome in Greenland Eskimo children. Acta Paediatr Scand 1986; 75: 1010–6
  • Drouin E, Russo P, Tuchweber B, Mitchell G, Rasquin-Weber A. North American Indian cirrhosis in children: a review of 30 cases. J Pediatr Gastroenterol Nutr 2000; 31: 395–404
  • Shneider BL. Genetic cholestasis syndromes. J Pediatr Gastroenterol Nutr 1999; 28: 124–31

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.