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Wolf's Syndrome in Twins — Translocation in The Mother

Published online by Cambridge University Press:  27 January 2025

M. Lévy ,
B. Noel  and
D. Viola
M. Lévy*
Affiliation:
Department of Pediatrics (Péd. A), Centre Hospitalier de Metz and Laboratory of Cytogenetics, Chambéry, France
B. Noel
Affiliation:
Department of Pediatrics (Péd. A), Centre Hospitalier de Metz and Laboratory of Cytogenetics, Chambéry, France
D. Viola
Affiliation:
Department of Pediatrics (Péd. A), Centre Hospitalier de Metz and Laboratory of Cytogenetics, Chambéry, France
*
Service de Pédiatrie A, Centre Hospitalier, 57000 Metz, France

Abstract

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A case of MZ twins, both affected by Wolf's syndrome, is described. Their mother, of subnormal look and low intellectual level is translocated. The children, born with a weight and size much below the average, show a very special morphotype: a hook-nose, an abnormal conformation of the back edge of the nostrils (a protrusion in the shape of a horn overhanging the filtrum), hypertelorism, microcephaly. Great asynchronism in the maturation of the bones and a somatoschisis of the body of the cervical vertebrae are noted. Deletion of the short arm chromosome 4 is juxtacentromeric. The study of blood and tissue groups corroborates monozygosity. Dermatoglyphs are little abnormal and identical in the two children. The mother's family is phenotypically normal. At 19 months of age, measuring is still below 4, psychomotor progress is extremely weak, and convulsions are frequent.

Type
Twin Studies in Medical and Clinical Genetics
Information
Acta geneticae medicae et gemellologiae: twin research , Volume 25 , January 1976 , pp. 267 - 270
Copyright
Copyright © The International Society for Twin Studies 1976

References

REFERENCES

Arrighi, F.E., Tsu, T.C. 1971. Localizations of heterochromatin in human chromosomes. Cytogenetics, 10: 81–86.CrossRefGoogle ScholarPubMed
Dutrillaux, B., Lejeune, J. 1971. Sur une nouvelle technique d'analyse du caryotype humain. C.R. Acad. Sci. (Paris), 272: 2638.Google Scholar
Dutrillaux, B., Laurent, C., Couturier, J., Lejeune, J. 1973. Coloration des chromosomes humains par l'acridine orange après traitement par le 5 bromodeoxyuridine. C.R. Acad. Sdi. (Paris), 276: 3179.Google ScholarPubMed
Nance, W.E., Uchida, I. 1964. Turner's syndrome, twinning and unusual variant of glucose-6-phosphate dehydrogenase. Am. J. Hum. Genet., 16: 380–392.Google ScholarPubMed
Nielsen, J. 1969. Klinefelter Syndrome and the XYY Syndrome. Kobenhavn: Munksgaard.Google ScholarPubMed
Noel, B., Mottet, J., Quack, B. 1974. Identification des hommes 47,XYY dans la population générale par la coloration en fluorescence de l'hétérochromatine. J. Genet. Hum., 22: 31–43.Google Scholar
Stewart, A., Barber, R. 1963. Data on parental age, sibship size and twins. Ann. Hum. Genet., 27: 1–9.CrossRefGoogle ScholarPubMed
Wolf, U., Reinwein, H., Porsch, R. et al. 1965. Defizienz an den kurzen Armen eines chromosoms Nr 4. Humangenetik, 1: 397–413.Google Scholar