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Diseases

Genetic and Rare Diseases Information Center (GARD)

Ivemark syndrome


Other Names for this Disease
  • Asplenia with cardiovascular anomalies
  • Asplenia syndrome
  • Bilateral right-sidedness sequence
  • Splenic agenesis syndrome
Related Diseases
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Overview

Ivemark syndrome is a rare congenital condition that affects multiple organ systems of the body. Ivemark syndrome is classified as a heterotaxy disorder or a laterality disorder. These terms refer to the failure of the internal organs of the chest and abdomen to be arranged in the proper location within the body. It is characterized by the absence (asplenia) or underdevelopment (hypoplasia) of the spleenheart malformations, and the abnormal arrangement of the internal organs of the chest and abdomen. Symptoms vary greatly depending on the specific abnormalities present; however if heart malformations are complex, the prognosis is often poor. The exact cause of Ivemark syndrome is not known. Most cases are sporadic (isolated and seemingly random). Unlike some other heterotaxy disorders, causative gene mutations have not been identified. There is no cure for Ivemark syndrome. Treatment might include surgical repair of heart malformations when appropriate and prophylactic antibiotic therapy to reduce the incidence of infection due to the absence or poor function of the spleen.[1][2][3]

Last updated: 2/23/2016

References

  1. Ivemark Syndrome. National Organization for Rare Disorders (NORD). 2010; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/740/viewAbstract.
  2. Kenneth Lyon Jones. Smith's Recognizable Patterns of Human Malformation. Elsevier Saunders; 2006; 6:698-700.
  3. Adrian Hrusca, Andreea Liana Rachisan, Bogdan Lucian, Simona Oprita, Simona Manole, Simona Cainap. Ivemark syndrome-a rare entity with specific anatomical features. Rev Med Chile. March 2015; 143(3):383-386. http://www.ncbi.nlm.nih.gov/pubmed/?term=26005826.
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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. You may need to register to view the medical textbook, but registration is free.
  • 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 Ivemark syndrome. Click on the link to view a sample search on this topic.
Other Names for this Disease
  • Asplenia with cardiovascular anomalies
  • Asplenia syndrome
  • Bilateral right-sidedness sequence
  • Splenic agenesis syndrome
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.