- Original Article
- A case of Beckwith-Wiedemann Syndrome.
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Jin Soo Choi, Byung Hee Kim, Young Soo Chon, Hwa Il Kwang, Young Youn Choi, Tai Ju Hwang
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Clin Exp Pediatr. 1990;33(3):367-372. Published online March 31, 1990
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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... |
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