klinefelter syndrome teeth
Klinefelter syndrome may adversely affect testicular growth resulting in smaller than normal testicles which can lead to lower production of testosterone. A simple screening method for detection of Klinefelter syndrome and other X-chromosome aneuploidies based on copy number of the androgen receptor gene.
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Klinefelter syndrome is a genetic condition that occurs in males when they have an extra X chromosome.
. This was directly related to effect of X and Y chromosomes on tooth development. Previous studies on the sex chromosome. It is suspected that Klinefelter syndrome is underdiagnosed because mild cases may not be identified.
It wasnt until I was in my mid 30s that I started having dental problems. Klinefelter syndrome is an under diagnosed chromosomal disorder with important challenges for health and medical management. The permanent tooth crowns in 47XXY males or individuals with an extra X or Y chromosome show increased size compared to normal men which is mainly due to increased enamel thickness the dentin thickness is somewhat reduced.
There are 2 types of chromosome called the sex chromosomes that determine the genetic sex of a baby. Das Klinefelter-Syndrom auch Klinefelter-Reifenstein-Albright-Syndrom 1 mit dem Karyotyp 47XXY ist eine der häufigsten Formen angeborener Chromosomenanomalien im männlichen Geschlecht und die häufigste Ursache von Hypogonadismus. It can affect physical and mental development.
This results in a thinner layer of the hard enamel which leads to an increased risk of tooth decay xxvii. Klinefelter syndrome KS also known as 47XXY is a syndrome where a male has an additional copy of the X chromosome. In this variant of the multirooted human teeth in which the body of the tooth is increased at the expense of the roots as regularly seen in cattle the teeth characteristically have a prismatic shape Heider and Wedl 1869.
We seldom ate sweets and never drank fizzy pop soda. The 2 sex chromosomes X and Y determine if you become a boy or a girl. Kohler S Gargano M Matentzoglu N et al The Human Phenotype Ontology in 2021 Nucleic Acids Research Volume 49 Issue D1 8 January 2021 Pages D1207D1217.
Often symptoms are subtle and subjects do not realize they are affected. Download data from HPO. I was brought up to take good care of my teeth with regular brushing and frequent check ups.
The syndrome may also cause reduced muscle mass reduced body and facial hair and enlarged breast tissue. Chromosomes contain all of your genes and DNA the building blocks of the body. There is some evidence of increased mesio-distal tooth crown size also in their primary dentition.
Klinefelter syndrome KS 47 XXY is the most abundant sex-chromosome disorder and is a common cause of infertility and hypogonadism in men. Klinefelter syndrome is a genetic condition affecting males and it often isnt diagnosed until adulthood. Chromosomes are packages of genes found in every cell in the body.
Klinefelter syndrome sometimes called Klinefelters KS or XXY is where boys and men are born with an extra X chromosome. The leading symptom in both syndromes is gonadal dysgenesis. The patient had mild mental retardation learning and speaking difficulties taurodontic teeth missing premolars and incisor teeth.
Keywords Cranio-facial Anomalies Hypodontia Klinefelter Syndrome Taurodontism. The aim of the present study was to. By now various dental features in these patients have been reported but to our knowledge this is the first report of multiple impacted teeth in the Klinefelter syndrome.
Due to the wide discrepancy of incidence of taurodontism reported in literature 004-480 in normal. Data from the National Center for Biotechnology Informations MedGen is used to provide genetic testing information available for a disease. Some common additional symptoms for several poly-X Klinefelter syndromes are listed below.
48XXYY Long legs Little body hair Lower IQ average of 60 to 80 normal IQ is 90 to 110 Leg ulcers and other vascular disease symptoms Extreme shyness but also sometimes aggression and impulsiveness 48XXXY or tetrasomy Eyes set further apart. Our results confirm the higher prevalence of taurodontism in Klinefelters patients compared to the normal population 125 vs. TS occurs in females with complete or partial absence of the second X chromosome while KS affects males with an extra X chromosomes.
Dental Health - Klinefelter Syndrome Dental Health Many KS adults do describe having poor dental health. This paper emphasizes the importance of. Request an Appointment at Mayo Clinic.
The primary features are infertility and small poorly functioning testicles. Almost one-half of men with KS have taurodontism pronounced tawr-oh-DAWNT-iz-uhm a dental problem in which the teeth have larger-than-normal chambers for holding pulp the soft tissue that contains nerve endings and blood vessels and shorter-than-normal tooth roots both of which make it easier for tooth decay to develop. Taurodont teeth were observed in 2 of the 16 Klinefelters patients 125 and in 2 of 100 normal males 20.
For example in patients with Turner syndrome is characterized by a reduction of the mesial-distal diameter and the vestibule-oral teeth respectively 61 and 26 while in male patients with Klinefelter syndrome shows that an increase of 18 and 17 of these diameters. In this condition the dental pulp - the living part of the tooth which contains a nerve occupies a greater area than normal. Variants of Klinefelter syndrome such as 48XXXY 49XXXXY are much rarer occurring in 1 in 50000 to 1 in 85000 or fewer newborns.
Klinefelter syndrome usually occurs as a. The effects of long-term hypogonadism may be hypogonadism in klinefelter s syndrome and teeth cult to discern from the gene dose effect of the extra X-chromosome. These are named either X or Y.
The average teth produces 4 to 7 mg of testosterone per day in a circadian pattern that peaks in the morning and is minimal in the evening. Turner syndrome TS and Klinefelter syndrome KS represent the two most common disorders due to aneuploidy of the sex chromosomes. A case of a boy with only half the complement of normal teeth and taurodontism is reported in association with Klinefelter syndrome 47 XXY.
The extra X chromosome that occurs in Klinefelter syndrome causes kkinefelter development of the testicles which in turn results in underproduction of testosterone. Sometimes symptoms are more evident and may include weaker muscles greater height poor motor. Girls normally have 2 X chromosomes.
Vertical enlargement of the molar pulp chamber was termed taurodontism bull-like teeth by Keith 1913. Causes Most people have 46 chromosomes. Klinefelter syndrome also known as XXY syndrome is a condition in boys and men thats caused by an extra X chromosome.
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