KLINEFELTER 'S SYNDROME. Klinefelter's syndrome is a chromosomal condition that affects male physical and cognitive development. It is named after Dr HARRY KLINEFELTER who first described the syndrome in 1942.T he syndrome is comprised of TESTICULAR DYSGENESIS,MICRO ORCHIDISM,EUNUCHOIDISM,GYNECOMASTIA,ELEVATED URINARY GONADOTROPINS AND AZOOSPERMIA. It is the most common chromosomal disorder associated with male hypogonadism and infertility. Prevalence is 1 in 500 males. KS can be diagnosed prenatally from fetal cytogenetic analysis performed on chorionic villus or amniocytes. Post natal diagnosis is done by *Karyotype analysis on peripheral blood lymphocytes. *XCAT -KS buccal swab test *FISH fluorescence in -situ hybridization. Management is based on three major facets of the syndrome *Hypogonadism. *-Gynecomastia. *Psychosocial problems. CLINICAL PRESENTATION. *Eunuchoid body proportions. *Female distribution of adipose tissue. *Sparse /absent facial.axillary.pubic and body hair. *-Gynecomastia. *Decreased muscle mass and strength. *Decreased physical endurance. *Small testes and penis. *Diminished libido. *Loss of functional seminiferous tubules and Sertoli cells. *Altered HYPOTHALAMIC -pituitary gonadal axis. *Tall stature with long arms and legs. *Erectile dysfunction *Speech and language deficits. *Learning disabilities. *Poor self esteem. *Behavioral problems They are also at increased risk for *Diabetes *Osteoporosis. *SLE. *Rheumatoid arthritis. *Sjogren syndrome. 47 XXY karyotype of KS is due to Mon disjunction of chromosomes during meiosis. Echo cardiography is done to assess mitral valve prolapse. Radiography is performed to assess bone density. Testicular biopsies are characterised by extensive fibrosis and hyalinisation of seminiferous tubules and hyperplasia of the interstitium.sometimes you can find residual foci of spermatogenesis TREATMENT. *Testicular replacement therapy. *Speech and behavioral therapy. *For infertility TESE and ICSI enables to retrieve sperms for IVF. *Mastectomy for -gynecomastia. LABORATORY FINDINGS. * Reduced testosterone. *High LH & FSH levels. *Elevated estradiol levels. 47 XXY

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MADAM , ONCE AGAIN , YOUR ARTICLE TOOK ME TO MY O.B.G.CLASS ROOM WITH A SENSATION OF EUPHORIA. VERY CLEARLY EXPLAINED MAM. THANKS A LOT ..

Thank you doctor
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Good morning vasundhara
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Very very informative and nicely described important topic. Thanks

Welcome doctor
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Wow.. Beautiful depiction. Thanks for sharing.

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Good morning madam, ,really nice info, ,thanx

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complete lack of fusion of mesonephric ducts

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