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Ivemark syndrome with asplenia in siblings
1First Department of Obstetrics and Gynecology, Semmelweis University, Budapest, Hungary.
The Journal of Pediatrics
|May 1, 1997
Summary
Ivemark syndrome, a rare condition, involves absent spleen, symmetric liver, and cardiac defects. Prenatal diagnosis is possible, and these cases support an autosomal recessive inheritance pattern.
Area of Science:
- Pediatric Genetics
- Medical Diagnostics
- Congenital Malformations
Background:
- Ivemark syndrome, also known as Ivemark kidney and spleen dục, is a rare congenital disorder.
- It is characterized by asplenia (absent spleen), polysplenia, or heterotaxy, often accompanied by complex cardiac malformations and visceral heterotaxy.