Other Names for this Disease
- Cholestasis lymphedema syndrome
- Lymphedema cholestasis syndrome
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intrahepatic cholestasis associated with lymphedema of the lower extremities. At least some cases of Aagenaes syndrome have been attributed to mutations in the LSC1 gene on chromosome 15q. This condition is inherited in an autosomal recessive manner and is found mainly in individuals of Norwegian descent.Aagenaes syndrome, or lymphedema cholestasis syndrome (LSC1), is a form of idiopathic familial
Last updated: 9/13/2012
- Dang S, Sigal Y, Davies D. Recurrent cellulitis in a case of Aagenaes syndrome. Clin Pediatr (Phila). 2009; http://www.ncbi.nlm.nih.gov/pubmed/19498211. Accessed 9/13/2012.
- 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; http://www.ncbi.nlm.nih.gov/pubmed/16635916. Accessed 9/13/2012.
- Cholestasis-Lymphedema Syndrome. Online Mendelian Inheritance in Man (OMIM). 2007; http://omim.org/entry/214900. Accessed 9/13/2012.
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- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Online Mendelian Inheritance in Man (OMIM) is a 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.