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A Case of Beckwith-Wiedemann Syndrome.

Journal of the Korean Pediatric Society 1987;30(10):1166-1169.
Published online October 31, 1987.
A Case of Beckwith-Wiedemann Syndrome.
Hyo Sook Hong, Hong Chul Lee, Oh Kyung Lee, Myung Ho Lee
Department of Pediatrics. Chonju Presbyterian Medical Center
Beck with-Wiedemann 증후군 1례
홍효숙, 이홍철, 이오경, 이명호
전주예수병원 소아과
Abstract
Beckwith-Wiedemann syndrome constitutes a clinicopathologic entity characterized macroglossia, omphalocele or umblical hernia, visceromegaly, postnatal gigantism, microcephaly, Nevus flammeus, ear lobe grooves, hemihypertrophy and other abnormalities. The anomalies of the syndrome are facultative and not obligatory. We experience one case of Beck with-Wiedemann syndrome. This 1-day-aged female neonate showed macroglossia, gigantism, omphalocele and hypoglycemia. Review of literature was done briefly.
Key Words: Beck with-Wiedemanrx syndrome, Omphalocele


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