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 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.
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