J Korean Pediatr Soc.
1998 Oct;41(10):1411-1416.
The Clinical Study on Marfan Syndrome
- Affiliations
-
- 1Department of Pediatrics, Sung Kyun Kwan University, Collage of Medicine, Korea.
- 2Division of Cardiology, Sung Kyun Kwan University, Collage of Medicine, Korea.
- 3Department of Orthopedics, Sung Kyun Kwan University, Collage of Medicine, Korea.
- 4Department of Opthalmology, Sung Kyun Kwan University, Collage of Medicine, Korea.
- 5Congenital and Metabolic Disorder Clinic, GUCH Clinic, Sung Kyun Kwan University, Collage of Medicine, Korea.
- 6Department of Pediatrics, Asan Medical Center, Seoul, Korea.
Abstract
-
PURPOSE: 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.
METHODS
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.
RESULTS
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.
CONCLUSION
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.
