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Bilateral Right-Sidedness Sequence

DA: 261

Developmental anomaly information

Definition: Ivemark syndrome is a rare progressive birth defect characterized by the absence of a spleen, malformations of the cardiovascular system and abnormal displacement of internal organs. In right isomerism, the liver is symmetrical, and each lung has three lobes and an epiarterial bronchus, (both atrial appendages are pyramidal in shape and the lungs are trilobed with short eparterial bronchi).


  • Asplenia syndrome
  • Ivemark syndrome
  • asplenia with cardiovascular anomalies
  • congenital absence of spleen syndrome
  • splenic agenesis syndrome, dextro-isomerism,

Developmental anomaly parent: Heterotaxy

Topology: Human body

System: Human body

Developmental anomaly category: Polytopic field defect

Case records MDB475

Humpath: 10310

Observed in MDB cases
Observed in combinaisons (MDB)
Observed in malformative diseases (MDB)
Associated genes in profile
Associated genes in malformative diseases
Associated chromosomal diseases
Associated signaling pathways
Associated developmental anomalies

Here is the list of developmental anomalies associated with this developmental anomaly, based on the cases' information :

Last modified: 2008-12-03 16:49:53 by farideh

Created: 2008-05-23 02:00:00 by phsaw

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