Abstract
A pair of monozygous twins discordant for Wiedemann-Beckwith syndrome is described and published reports reviewed to establish the mode of inheritance. Single gene control seems unlikely and it is proposed that the condition arises from the interaction of "at risk" genes in the fetus and metabolic factors in the mother, which suggests multifactorial control.
Full text
PDFSelected References
These references are in PubMed. This may not be the complete list of references from this article.
- Ben-Galim E., Gross-Kieselstein E., Abrahamov A. Beckwith-Wiedemann syndrome in a mother and her son. Am J Dis Child. 1977 Jul;131(7):801–803. doi: 10.1001/archpedi.1977.02120200083019. [DOI] [PubMed] [Google Scholar]
- Cohen M. M., Jr, Gorlin R. J., Feingold M., ten Bensel R. W. The Beckwith-Wiedemann syndrome. Seven new cases. Am J Dis Child. 1971 Dec;122(6):515–519. doi: 10.1001/archpedi.1971.02110060085015. [DOI] [PubMed] [Google Scholar]
- Filippi G., Mckusick V. A. The Beckwith-Wiedmann syndrome. Medicine (Baltimore) 1970 Jul;49(4):279–298. doi: 10.1097/00005792-197007000-00002. [DOI] [PubMed] [Google Scholar]
- Forrester R. M. Wiedemann-Beckwith syndrome. Lancet. 1973 Jul 7;2(7819):47–47. doi: 10.1016/s0140-6736(73)91988-0. [DOI] [PubMed] [Google Scholar]
- Gardner L. I. Letter: Pseudo-Beckwith-Wiedemann syndrome: interaction with maternal diabetes. Lancet. 1973 Oct 20;2(7834):911–912. doi: 10.1016/s0140-6736(73)92044-8. [DOI] [PubMed] [Google Scholar]
- Irving I. M. Exomphalos with macroglossia: a study of eleven cases. J Pediatr Surg. 1967 Dec;2(6):499–507. doi: 10.1016/s0022-3468(67)80003-4. [DOI] [PubMed] [Google Scholar]
- Kosseff A. L., Herrmann J., Opitz J. M. The Wiedemann-Beckwith syndrome: genetic considerations and a diagnostic sign. Lancet. 1972 Apr 15;1(7755):844–844. doi: 10.1016/s0140-6736(72)90825-2. [DOI] [PubMed] [Google Scholar]
- Lubinsky M., Herrmann J., Kosseff A. L., Opitz J. M. Letter: Autosomal-dominant sex-dependent transmission of the Wiedemann-Beckwith syndrome. Lancet. 1974 May 11;1(7863):932–932. doi: 10.1016/s0140-6736(74)90383-3. [DOI] [PubMed] [Google Scholar]
- Matsuura N., Endo M., Okayasu T., Okuno A. Letter: Wiedemann-Beckwith syndrome. Lancet. 1975 Sep 13;2(7933):508–508. doi: 10.1016/s0140-6736(75)90588-7. [DOI] [PubMed] [Google Scholar]
- Sommer A., Cutler E. A., Cohen B. L., Harper D., Backes C. Familial occurrence of the Wiedemann-Beckwith syndrome and persistent fontanel. Am J Med Genet. 1977;1(1):59–63. doi: 10.1002/ajmg.1320010107. [DOI] [PubMed] [Google Scholar]
- WIEDEMANN H. R. COMPLEXE MALFORMATIF FAMILIAL AVEC HERNIE OMBILICALE ET MACROGLOSSIE--UN "SYNDROME NOUVEAU"? J Genet Hum. 1964 Sep;13:223–232. [PubMed] [Google Scholar]
- Wiedemann H. R. E.M.G. syndrome. Lancet. 1973 Sep 15;2(7829):626–627. doi: 10.1016/s0140-6736(73)92468-9. [DOI] [PubMed] [Google Scholar]