Sexual Development and

Hormone Replacement Therapy

Sex Hormone Replacement Therapy 

 

Around 90% of those with TS will experience ovarian failure resulting in a deficiency of the sex hormones estrogen and progesterone since the sex hormones are produced by the ovaries. Thankfully, these important hormones can be replaced with medications.  The term for underdeveloped or improperly functioning ovaries is hypogonadism.  Hormone replacement therapy (HRT) is recommended for girls and women with TS who experience ovarian failure.  An endocrinologist is best suited to determine when to begin hormone replacement therapy (HRT) for the sex hormones and once HRT is established, an adult endocrinologist, gynecologist or general practitioner may oversee HRT. 

 

Determining if HRT is Needed

Interestingly, girls with TS may develop pubic hair, body odor, underarm hair, and acne develop under the influence of hormones from the adrenal glands. Around 11-12 years of age, an endocrinologist will use two lab tests to determine ovarian function. The tests are FSH (Follicle Stimulating Hormone) and AMH (Anti-Mullerian Hormone).  While a small percentage of girls with TS will show some signs of breast development in their early teens and may menstruate on their own, these usually stop sooner than usual. Girls with mosaicism are more likely to enter puberty spontaneously, in rare instances some with classic TS may have ovaries producing estrogen.  Some women with TS are actually diagnosed during fertility testing, even though they have menstrual cycles or their cycles stopped in their 20’s or 30’s.

The goal of HRT is to mimic natural puberty so estrogen begins with a very low dose and gradually increase over 2-3 years to a typical young adult dose, allowing for natural breast development and maturation of the uterus. Higher doses of estrogen limit growth potential, whether or not growth hormone has been started. Girls may remain on a lower dose if growing taller is important.

 

 

HRT Options

 

It is recommended to use a small patch that adheres to the skin on the abdomen, allowing the estrogen to enter directly into the bloodstream, bypassing the liver. Current studies suggest the body utilizes estrogen better before it is broken down by the liver so it may:

 

  • decrease the risk of blood clots

  • improve blood pressure control

  • result in better bone mass

  • improve the effectiveness of growth hormone

 

Some patch dosages are applied weekly, while some are applied twice per week. For very small dose delivery, you may need to cut the patch, although the packaging states “never cut” the patch, pharmacists suggest using a small blade to avoid contact on your fingers.

Estrogen may also be taken orally (in pill form) and may be more convenient, comfortable, affordable and available.

 

Progestin for Starting a Menstrual Cycle

After two years of estrogen, or when “breakthrough bleeding” occurs, another hormone, progestin, is added as a pill or combined with estrogen in an oral contraceptive. • Progestin taken 10 days per month, results in a monthly menstrual period and maintaining a healthy uterus. Some girls and women choose to menstrual cycles every other month or every three months. Discuss options with your doctor.

 

Stopping HRT

Estrogen is essential for a healthy body and it is recommended you should receive estrogen until 50 years of age. If you have had trouble with side effects of ERT, don’t give up, studies suggest ERT is important for strong bones, preventing depressive symptoms,

 

Important Note: estrogen replacement therapy for girls and women with TS is not the same as hormone treatment in a woman without TS who takes birth control pills or estrogen replacement in postmenopausal women. Discuss your concerns with safety and family history of cancers with your doctor

 

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Growth Hormone & Estrogen Replacement Therapies in Turner Syndrome 

Turner Syndrome a Guide for Families TSSUS

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