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Rett syndrome: presumptive carriers of the gene defect. Sex ratio among their siblings
H O Akesson1, B Hagberg, J Wahlström
1Department of Psychiatry, Sahlgren University Hospital, Göteborg, Sweden.
European Child & Adolescent Psychiatry
|January 1, 1997
Abstract:
The live-born full siblings of 121 presumptive carriers of Rett syndrome belonging to eight pedigrees were examined as to sex ratio at birth. A total of 394 such siblings born in the period 1757-1966 were identified. 183 (46.4%) of them were male and 211 (53.6%) female, i.e., significantly fewer than in the average Swedish population (p = 0.03) were male. Our findings support the existence of one or more lethal alleles causing Rett syndrome.