Tetra-X症候群の1例

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  • A Case of Tetra-X syndrome

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A group of numerical abnormalities among sex chromosomal aberrations found in females, which is characterized by an excessive number of X chromosomes such as females with XXX chromosomes (triple-X females), females with XXXX (tetra-X females), or females with XXXXX (penta X females) is collectively called the poly-X syndrome. Since Jacobs et al. (1959) referred to- a patient with this syndrome as a “super female”, various cases have been reported. In Japan, however, there has been no report on the oral findings relating to this syndrome but a case of triple-X female was reported by Miura et al.<BR>The patient of this case was a female child at the age of 16 years and 5 months who had been diagnosed to be a female with XXXX chromosomes (tetra-X female) within the poly-X syndrome. She was shown proved to have congenital heart disease, microseme, a shorter body height, the mental retardation and was underweight. As to the oral findings, congenital absence of maxillary bilateral lateral incisors, maxillary left lateral second premolar and mandibular bilateral second premolars, and large mesiodistal tooth width, as well as lower palate and progenia were observed.

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