Tuesday, 24 January 2017
Monday, 23 January 2017
Klinefelter Syndrome ( Male with some female characteristics)
KLINEFELTER SYNDROME
A chromosomal condition (Non Dysjunction of X chromosome during meiosis)that affects male physical and cognitive development, results from presence of one or two extra 'X' Chromosomes.Also called as XXY(47xxy)Syndrome.
Karyotype
This syndrome is a chromosomal-sexual disorder most common with an rate of 1 in 500 males, with prevalence of any ethnic group and is characterized by significant testicular dysfunction, azoospermia, gynecomastia, decreased libido, increased plasma gonadotropins in males with two or more chromosome X
Causes:
Dental And Facial Manifestations
Diagnosis
⧫ Usually in the late adulthood when patient fail to develope sufficient secondary sexual characteristics
⧫ Only 10% of Klinefelter cases are found by Prenatally through genetic tests on cells collected from amniocentesis or chorionic villus sampling (CVS)
⧫ Others Based on Past History and Genetic Karyotyping
⧫ About 64% individuals are never recognized
Treartment
→ Supportive therapy
→ Testosterone replacement
→ Speech therapy and educational support can help boys who have language or learning problems
→ Interceptive Orthodontics
A chromosomal condition (Non Dysjunction of X chromosome during meiosis)that affects male physical and cognitive development, results from presence of one or two extra 'X' Chromosomes.Also called as XXY(47xxy)Syndrome.
Karyotype
This syndrome is a chromosomal-sexual disorder most common with an rate of 1 in 500 males, with prevalence of any ethnic group and is characterized by significant testicular dysfunction, azoospermia, gynecomastia, decreased libido, increased plasma gonadotropins in males with two or more chromosome X
Causes:
- The presence of an extra X chromosome in males most often occurs when the genetic material in the egg splits unevenly. But it can also occur when the genetic material in the sperm splits unevenly
- Even though Klinefelter syndrome is a genetic disorder, it is not passed down through families( Not hereditary).
- Occurs only as a result of a random genetic error after conception.
- Weaker muscles and reduced strength
- Males willl have less facial and body hair, and broader hips
- XXY males may develop breast tissue and
- Weaker bones, and a lower energy level than other males
- Affected males are often infertile, or may have reduced fertility
- Delayed Puberty
- Low serum testosterone level but high serum follicle-stimulating hormone (FSH) and luteinizing hormone(LH) levels
Dental And Facial Manifestations
- Skeletal Disproportion
- Mandibular Prognathism
- Taurodontism ( Bull Tooth )
- Hypertelorism,Strabismus
- Upward slant of the Palpabral fissure
- Cleft Palate
Diagnosis
⧫ Usually in the late adulthood when patient fail to develope sufficient secondary sexual characteristics
⧫ Only 10% of Klinefelter cases are found by Prenatally through genetic tests on cells collected from amniocentesis or chorionic villus sampling (CVS)
⧫ Others Based on Past History and Genetic Karyotyping
⧫ About 64% individuals are never recognized
Treartment
→ Supportive therapy
→ Testosterone replacement
→ Speech therapy and educational support can help boys who have language or learning problems
→ Interceptive Orthodontics
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