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KMID : 0367419770180060054
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Journal of Korean Pediatric Society
1977 Volume.18 No. 6 p.54 ~ p.58
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Ivemark´s Syndrome 4 Cases
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이순일/Lee, Soon Il
이우길/강부경/이선희/이승규/진동식/윤덕진/Liegh, Woo Gill/Kang, Bu Kyung/Lee, Sun Hee/Lee, Sung Kyu/Chin, Dong Shik/Yun, Duk Jin
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Abstract
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Ivemark´s syndrome is rare disease and characterized by congenital absence of the spleen associated with characteristic group of anomalies of the cardiovascular and gastroenteric systems.
We had experienced 4 cases of Ivemark´s yndrome: Case 1 had asplenia, situs inversus, dextrocardia, ASD, and pulmonary stenosis; Case 2 had asplenia, bilobulated liver, centraly located stomach but deviated to right side, single ventrilce, and transposition of the great vessels with pulmonary atresia; Case 3 had asplenia, bilobulated liver, rentraly located stcmach but deviated to left side, tricuspid atresia, high VSD, hypoplasia of right ventricle, and right sided aortic arch; Case 4 had asplenia, bilobulated liver, dextrocardia, single ventricle, and truncus arteriosus.
We had diagnosed these cases by EKG, chest -K-ray, cardiac catheterization with cardiac angiogram. and liver and spleen scanning.
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KEYWORD
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