management

Last reviewed 08/2021

Management of KS is a multidisciplinary task which should involve speech therapist, psychologist, endocrinologist, general practitioners and infertility specialists (1).

Treatment options for patients with KS include:

  • testosterone treatment
    • should begin around puberty (age 12 years) and the dose should increase with increasing age to maintain appropriate serum concentrations of testosterone, estradiol, FSH, and LH
    • although it does not treat infertility, gynecomastia, and small testes; it helps in normalization of body proportions (muscle bulk and bone structure )or/and development of normal secondary sex characteristics and general improvement in behavior and work performance
    • long term benefits of testosterone may include reduction in the risk of osteoporosis, autoimmune disease, and breast cancer (2)

  • speech therapy
    • important to refer boys to a speech therapist in case of delay in their speech development (1)
    • early therapy is important to boys with 47,XXY to maximize skills in the  understanding and production of more complex language (2)  

  • physical therapy
    • to avoid affecting the muscle tone, balance, and coordination in boys with hypotonia or delayed gross motor skills (3)

  • occupational therapy
    • recommended in cases of fine motor dyspraxia
    • may also benefit 47,XXY infants with feeding problems or difficulty with latching on or sucking (2)

  • fertility treatment
    • assisted reproduction techniques could be used such as testicular sperm extraction and subsequent intracytoplasmic sperm injection (1)

  • psychological  support

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