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Clinical Study on the Dilated Cardiomyopathy in Children.

Journal of the Korean Pediatric Society 1983;26(11):1081-1094.
Published online November 30, 1983.
Clinical Study on the Dilated Cardiomyopathy in Children.
Jong Jin Seo, Byong Gwan Sohn, Jung Yeun Choi, Yong Soo Yoon, Chang Yee Hong
Department of Pediatrics, College of Medicine,Seoul National University
小兒期 擔張型心筋症의 臨床的 考察
徐宗振, 孫炳瓘, 崔正衍, 尹龍洙, 洪影義
서울大學校 醫科大學 小兒科學敎室
Abstract
Forty five cases of dilated cardiomyopathy who were admitted, to Seoul National University Hospital from June, 1980 to May, 1983 were studied on the clinical features and laboratory findings. 1) Twenty two cases (44.4%) were primary dilated cardiomyopathy, and the other 25 case (55.6%) were secondary. Nineteen cas&s(95.0%) of primary dilated cardiomyopathy were diagnosed within 3 year of age, but sacondary dilated cardiomyopathy did not show any age prevalence during childhood. 2) Secondary dilated cardiomyopathies were late sequelae of myocarditis, or due to uremia and/or hypertension, the toxicity of adriamycin, or endocardial fibroelastosis. ' 3) The older the age at diagnosis and the better initial echocardiographic findings including shortening fraction, LVSTI&EPSS and the higher incidence of cardiac murmur probably secondary to mitral regurgitation, the longer the survival in the group (Group 1) of patients with primary dilated cardiom yopathy. These findings are suggestive of possible relationship with longer survival in primary dilated cardiomyopathy. 4) Echocardiographic examination was a most important and useful noninvasive method for diagnosis, evaluation of clinical course & prognosis of dilated carpiomyopathy in children. LVIDd, shortening fraction, LVSTI & EPSS were good echocardiographic index of left ventricular function, and LVSTI was the most sensitive & useful method in children. 5) General prognosis of primary dilated cardiomyopathy is poor, but active treatment with digoxin, diuretics & vasodilator significantly improved the clinical status in many patient with primary dilated, cardiomyopathy eventually extending their survival time. 6) Active management of treatable underlying disease in secondary cardiomyopathy also improved the the clinical status eventually extending the survival time.
Key Words: Dilated cardiomyopathy, Children, Echocardiography


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