Journal of the Korean Pediatric Society 1975;18(6):468-472.
Published online June 30, 1975.
Ivemark’s Syndrome 4 Cases
Soon II Lee, Woo Gill Lee, Bu Kyung Kang, Sun Hee Lee, Sung Kyu Lee, Dong Shik Chin, Duk Jin Yun
Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
Ivemark 氏 症候群 4例
이순일, 이우길, 강부경, 이선희, 이승규, 진동식, 윤덕진
延世大學校 醫科大學 小兒科學敎室
Abstract
Ivemark’s syndrome is rare disease and characterized by congenital absence of the spleen as sociated 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, centraly located stomach 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 X-ray, cardiac catheterization with cardiac angiogram, and liver and spleen scanning.


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