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The Clinical Study on Marfan Syndrome

Journal of the Korean Pediatric Society 1998;41(10):1411-1416.
Published online October 15, 1998.
The Clinical Study on Marfan Syndrome
Jung Sim Kim1, Dong Kyu Jin1, See Hwan Ko1, Jee Yeon Min1, I-Seok Kang2, Kye Won Jeon3, Yung Lan Choi2, Heung Jae Lee2, Seung Woo Park4, Won Ro Lee4, Chong Suh Lee5, Sei Yeul Oh5, Chang Won Ki6, Han Wook Yoo6, In Sook Park6, Jae Kon Ko6
1Department of Pediatrics, Sung Kyun Kwan University, College of Medicine, Seoul, Korea
2GUCH Clinic, Sung Kyun Kwan University, Collage of Medicine, Seoul, Korea
3Congenital and Metabolic Disorder Clinic, Sung Kyun Kwan University, Collage of Medicine, Seoul, Korea
4Department of Division of Cardiology, Sung Kyun Kwan University, Collage of Medicine, Seoul, Korea
5Department of Orthopedics, Sung Kyun Kwan University, Collage of Medicine, Seoul, Korea
6Department of Pediatrics, Asan Medical Center, Seoul, Korea
Marfan 증후군 환자의 임상적 고찰
김정심1, 진동규1, 고시환1, 민지연1, 강이석2, 전계원3, 최영란2, 이흥재2, 박승우4, 이원로4, 이종서5, 오세열5, 기창원6, 유한욱6, 박인숙6, 고재곤6
1성균관대학교 의과대학 소아과학교실
2심혈관센터 GUCH클리닉
3삼성서울병원 선천성대사클리닉
4성균관대학교 의과대학 순환기내과학교실, 심혈관센터 GUCH클리닉
5성균관대학교 의과대학 정형외과학교실
6울산대학교 의과대학 소아과학교실
Heung Jae Lee, Email: 1
: The aim of this study was to assess the involvement of several organs patients with Marfan syndrome in Korea. Also the clinical features in childhood patients with Marfan syndrome were assessed.
: Thirty-eight cases of Marfan syndrome were enrolled in this study. Clinical evaluations of the musculoskeletal, cardiovascular and occular system were performed in all cases.
: The musculoskeletal system was involved in 32 cases(84.2%) and occular system in 24 cases(63.1%). Cardiovascular abnormalities were found in 19 cases(50.0%) at initial evaluation. Family history was involved in 21 cases(55.2%). Ectopia lentis was found in 17 cases(70.8%). Severe myopia and iris abnormalities were also present in 14 cases(58.2%). The ascending aorta was dilated in 13 cases(34.2%). Emergency operation was performed in 3 cases(7.9%) because of a dissecting aorta. Mitral regurgitation and prolapse were found in 29 cases(76.4%) and other valve insufficiency was accompainied in 5 cases(13.1%). Of the 38 cases, 29 patients(79.3%) were less than 15 years of age and their major manifestations were occular problems in 23 cases (79.3%), and family history in 17 cases(58.6%). In one infant, severe heart failure was the predominant clinical feature.
: The clinical features of Korean patients with Marfan syndrome were summarized in this report. Heart failure was the main manifestaton in infantile Marfan syndrome. Early treatment with beta-blocker and valvular replacement can prevent fatality, i.e. aortic dissection, in this disease, concern and management should be advocated in the early detection of Marfan syndrome.
Key Words: Marfan syndrome

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