Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.


Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Aagenaes syndrome

Other Names for this Disease
  • CHLS
  • Cholestasis lymphedema syndrome
  • LCS
  • LCS1
  • Lymphedema cholestasis syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.


Aagenaes syndrome, or lymphedema cholestasis syndrome (LSC1), is a form of idiopathic familial intrahepatic cholestasis associated with lymphedema of the lower extremities.[1][2] At least some cases of Aagenaes syndrome have been attributed to mutations in the LSC1 gene on chromosome 15q.[3] This condition is inherited in an autosomal recessive manner and is found mainly in individuals of Norwegian descent.[2]
Last updated: 9/13/2012


  1. Dang S, Sigal Y, Davies D. Recurrent cellulitis in a case of Aagenaes syndrome. Clin Pediatr (Phila). 2009; Accessed 9/13/2012.
  2. Drivdal M, Trydal T, Hagve TA, Bergstad I, Aagenaes O. Prognosis, with evaluation of general biochemistry, of liver disease in lymphoedema cholestasis syndrome 1 (LCS1/Aagenaes syndrome). Scand J Gastroenterol. 2006; Accessed 9/13/2012.
  3. Cholestasis-Lymphedema Syndrome. Online Mendelian Inheritance in Man (OMIM). 2007; Accessed 9/13/2012.
Your Questions Answered
by the Genetic and Rare Diseases Information Center

Please contact us with your questions about Aagenaes syndrome. We will answer your question and update these pages with new resources and information.
On this page

In Depth Information

  • The Online Mendelian Inheritance in Man (OMIM) is an catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Aagenaes syndrome. Click on the link to view a sample search on this topic.