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Asplenia with cardiovascular anomalies

Field	Value
name	Asplenia with cardiovascular anomalies
synonyms	Ivemark syndrome
image	Autosomal recessive - en.svg
caption	This condition is inherited in an autosomal recessive manner

Asplenia with cardiovascular anomalies, also known as Ivemark syndrome and right atrial isomerism, is an example of a heterotaxy syndrome. These uncommon congenital disorders are characterized by defects in the heart, spleen and paired organs such as the lungs and kidneys. Another name is "asplenia-cardiovascular defect-heterotaxy".

Right atrial isomerism is named for its discoverer, Swedish pathologist Biörn Ivemark.

Presentation

In right atrial isomerism, both atria of the heart are morphological right atria leading to associated abnormalities in the pulmonary venous system. In addition, individuals with right atrial isomerism develop asplenia, a midline liver, malrotation of the small intestine and the presence of two morphologic right lungs. Individuals with left atrial isomerism, by comparison, have two morphologic left atria, polysplenia, intestinal malrotation and two morphologic left lungs.

Causes

The cause of heterotaxy is unknown.

The Ivemark Syndrome Association, which is based in Dorset, is one of the organisations dedicated to helping patients and funding research.

Diagnosis

Treatment

References

(2008-11-10). "Ivemark Syndrome Association". Patient UK.
(Aug 2008). "Pancreatic aplasia in a fetus with asplenia-cardiovascular defect-heterotaxy (Ivemark syndrome).". Birth Defects Research Part A: Clinical and Molecular Teratology.
(November 1955). "Implications of agenesis of the spleen on the pathogenesis of conotruncus anomalies in childhood; an analysis of the heart malformations in the splenic agenesis syndrome, with fourteen new cases". Acta Paediatrica Supplement.
"Ivemark Syndrome". National Organization for Rare Diseases.
(2009-05-22). "'Jigsaw Kid' lives with jumbled up internal organs". The Telegraph.

Info: Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

rare-diseases congenital-disorders

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