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Genetic and Rare Diseases Information Center (GARD)

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Ivemark syndrome

Other Names for this Disease
  • Asplenia syndrome
  • Asplenia with cardiovascular anomalies
  • Bilateral right-sidedness sequence
  • Splenic agenesis syndrome
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Ivemark syndrome is a rare condition that affects multiple organ systems of the body. It is characterized by the absence (asplenia) or underdevelopment (hypoplasia) of the spleen, heart malformations, and the abnormal arrangement of the internal organs of the chest and abdomen (situs inversus). Symptoms vary greatly depending on the specific abnormalities present. The exact cause of Ivemark syndrome is not known.[1]

Last updated: 9/20/2011


  1. Ivemark Syndrome. National Organization for Rare Disorders (NORD). 2010; Accessed 9/20/2011.
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Basic Information

  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. Click on the link to view this information. You may need to register to view the medical textbook, but registration is free.
  • 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 Ivemark syndrome. Click on the link to view a sample search on this topic.