The Japanese Journal of Pediatric Dentistry
Online ISSN : 2186-5078
Print ISSN : 0583-1199
ISSN-L : 0583-1199
A Case of XXXXY Syndrome
Naoto OsugaTamami SaitoMasanori TamuraSachiyo TeramotoHiroshi IwasakiHiroo Miyazawa
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1999 Volume 37 Issue 5 Pages 1047-1054

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Abstract
A case of the XXXXY syndrome, in which 3 excess X chromosomes are present in the male, is described with dental findings.
1. The patient had a history of retarded intrauterine growth and was born with vaginal cephalic delivery after a pregnancy of 39 weeks and 4 days. The state at birth was neonatal asphyxia with Apgar scores of 3 (1 minute) and 5 (5 minutes).
2. The neonate showed pulmonary valve stenosis, hypospadias, split scrotum, pes varus of the left foot, retarded mental and physical development, and poor body weight gains as complications.
3. The state of ossification of the carpal bones was nearly in agreement with the chronological age. X-ray films revealed taurodontism of the first deciduous molars.
4. Analysis of lateral roentgenographic cephalometry of the head indicated a generally small skeleton, a slightly large length of the mandibular body, a small height of the rami of mandible, and clockwise rotation of the mandible.
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© The Japanese Society of Pediatric Dentistry
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