Ivemark syndrome


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pol·y·sple·ni·a

(pol'ē-splē'nē-ă), [MIM*208530]
A condition in which splenic tissue is divided into nearly equal masses or totally absent; congenital heart disease and malposition and maldevelopment of abdominal organs are common; may be related to situs inversus. Most cases are sporadic, although some suggest autosomal recessive inheritance.
See also: bilateral left-sidedness.
[poly- + G. splēn, spleen]

Ivemark syndrome

A heterotaxy syndrome (OMIM:208530) in which both right and left atria of the heart are morphologically right. Other associated anomalies include asplenia, mid-line liver, small intestine malrotation and two morphological right lungs.
  
Clinical findings
In keeping with congenital heart defect—i.e., cyanosis, breathlessness, lethargy, poor feeding.

pol·y·sple·ni·a

(pol'ē-splē'nē-ă)
A condition in which splenic tissue is divided into nearly equal masses; there are several small spleens; congenital heart disease and malposition and maldevelopment of abdominal organs are common; may be related to situs inversus. Most cases are sporadic, although some suggest autosomal recessive inheritance.
Synonym(s): Ivemark syndrome.
[poly- + G. splēn, spleen]

Ivemark,

Björn, Swedish pathologist, 1925–.
Ivemark syndrome - a possibly heritable disorder in which organs of the left side of the body are a mirror image of their counterpart on the right side, with associated splenic agenesis and cardiac malformations.
References in periodicals archive ?
Situs ambiguous with asplenia (Ivemark syndrome), also referred to as right isomerism or bilateral right sidedness, is characterized by ambiguous location of the abdominal organs and congenital absence of most or all of normal splenic tissues.
McKenzie Wallace died just five days after being born because of a heart defect linked to rare genetic disorder Ivemark syndrome.
Ivemark syndrome with agenesis of the corpus callosum: a case report with a review of the literature.
The Ivemark syndrome: prenatal diagnosis of an uncommon cystic renal lesion with heterogeneous associations.