Klinefelter's Syndrome with Congenital Heart Disease: Report on case

Abstract

Due to an abnormality of one of the sex chromosomes (XXY-type chromosomes), the development of the muscular issue is poor. Klinelfelter's symptom is characterized in males by feminine breasts, a growth imperfection of the testicles, and a high value in the follicle stimulation hormone (FSH) and luternic hormone (LH).
In 1971, Daito reported a case of Klinefelter's Syndrome in a patient who exhibited Taurodont teeth. This was the first reported case in Japan.
Generally, a primary molar tooth or body of molar is long, but for Taurodont teeth, the marrow space gets longer, and the teeth have short roots.
We experienced a case of Klinefelter's Syndrome with congenital heart disease in a 3-year,8month old boy.
1. In the countenance of the patient, both eyes were far apart from each other. He had thick eyebrows, and a poor development of the face when it was measured.
2. According to clinical test, on the hormone value in the blood, the FSH and LH values were normal.
3. All the milk teeth showed enamel hypoplasia. In the examination, we noticed the imperfection of the face. We also noticed that the crowns in the primary molar teeth had grown imperfectly and they had a brown discoloration.
4. According to the orthopantomography and the dental X-ray, the Taurodont teeth were found among the primary molars in the maxilla and the mandible.
5. The mesio-distal width of the present teeth and the length and width of the dental arch were smaller than the standard average.
6. As a result of the cephalometric analysis, the crossbite was clearly noticeable.