Journal of the Korean Pediatric Society 1990;33(3):367-372.
Published online March 31, 1990.
A case of Beckwith-Wiedemann Syndrome.
Jin Soo Choi, Byung Hee Kim, Young Soo Chon, Hwa Il Kwang, Young Youn Choi, Tai Ju Hwang
Department of Pediatrics, Chonnam University Medical School, Kwangju, Korea
Beckwith -Wiedemann 증후군 1례
최진수, 김병희, 전영수, 곽화일, 최영륜, 황태주
전남대학교 의과대학 소아과학교실
Received: 15 June 1989   • Accepted: 7 October 1989
Abstract
The Beckwith-Wiedemann syndrome (exomphalos-macroglossia-gigantism: E M G Syndrome) is characterized by the presence of a wide variety of anomalies such as omphalocele, muscular macroglossia, gigantism, neonatal hypoglycemia, cytomegaly of the adrenal fetal cortex, hyperplasia of gonadal interstitial cells, and hyperplastic visceromegaly, particularly of pancreas and kidneys. Other abnormalities may include hemihypertrophy, microcephaly and mental retardation, prominent occiput, facial flame nevus, neonatal polycythemia, and linear indentations of the ear lobes. Since the original descriptions of this syndrome by Beckwith (1963, 1964) and Wiedemann, (1964) numerous examples of the association of these anomalies have been reported. We experienced a case of Beck with-Wiedemann in 1 day male baby who showed macroglossia, omphalocele, facial flame nevus, linear indentations of the ear lobes, hypoglycemia and prominent occiput. A brief review of related literatures is also presented.
Key Words: Omphalocele, Macroglossia, Hypoglycemia




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