Eruption delay in a 47 XXY male: A case report

Department of Dental Science, Dental School, Alma Mater Studiorum, University of Bologna, Bologna, Italy.
European Journal of Paediatric Dentistry (Impact Factor: 0.45). 06/2012; 13(2):159-60.
Source: PubMed


The 47,XXY syndrome, or Klinefelter syndrome, though it is a rare occurrence, it is the most common sex choromosome disorder affecting male subjects. This syndrome is underdiagnosed and seldomly before puberty. In this case, diagnosis was made before birth, through chorion villus sampling.

Case report:
A 16 month-old Italian male with 47 XXY syndrome showed the absence of primary teeth, with a delay of about 8-10 months, whereas during the first 15 months of life the auxological development has been normal both in weight and height (about 50th percentile). We assumed that this delay may be linked with Klinefelter syndrome, as sexual chromosomes play an important role in the dental development.

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    ABSTRACT: Chromosome 47, XYY syndrome is usually diagnosed late. Some of the clinical characteristics of XYY syndrome may be perceptible in dental care. The slow development of cognitive and motor activities and tall stature is common in XYY patients. The aim of this article was to relate the oral, physical, and behavioral aspects of a 6-year-old patient with the chromosome 47, XYY syndrome, diagnosed by means of karyotyping. The patient presented motor difficulty, which led to a fall and traumatism in the anterior region. In the radiography, agenesia of the permanent maxillary lateral incisors, presence of taurodontism in the primary molars, and macrodontia of the maxillary central incisors and permanent molars could be observed. Once the diagnosis was made, it was possible to understand his difficulty at school, and make available appropriate monitoring by a suitable multidisciplinary team to stimulate, control, and minimize the day-to-day difficulties found by patients with this syndrome.
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