Hypogonadotropic hypogonadism is a health condition developed because of absent or decreased function of the ovaries due to a problem with the pituitary or hypothalamus gland – decreased production of a hormone called “Gonadotropin-Releasing Hormone (GnRH)”. The Gonadotropin-Releasing Hormone is needed for stimulation of ovaries to produce sex hormones. Absence and/or deficit of GnRH is the main cause for development of all clinical symptoms of hypogonadotropic hypogonadism – during this disease ovaries produce little or no sex hormones.
Hypogonadotropic hypogonadism sometimes could be called Gonadotropin deficiency, Secondary hypogonadism and/or Kallmann syndrome.
In general girls with hypogonadotropic hypogonadism do not reach puberty, have small size of ovaries and are not able to become pregnant (primary infertility).
Females with hypogonadism have abnormal ovaries that do not produce normal levels of sex hormones which are needed for normal puberty development and for normal menstrual cycles. Actually all girls with Gonadotropin deficiency have primary amenorrhea (absence of periods).
Most cases of Gonadotropin deficiency are inherited.
It is well known that in healthy females the hypothalamus in the brain releases Gonadotropin-Releasing Hormone (GnRH) which stimulates the pituitary gland to release hormones called “Luteinizing Hormone (LH)” and “Follicle-Stimulating Hormone (FSH)”. These hormones are responsible for hormone production in ovaries (mainly sex hormones). And ovarian sex hormones are responsible for normal sexual development in puberty including development of the regular menstrual cycle. The disruption in this chain of the step-by-step hormones production causing the deficiency of the sex hormones and preventing normal sexual maturity.
Failure of the hypothalamus is most common cause of so called “Kallmann syndrome” which is an inherited form of hypogonadotropic hypogonadism that can be associated with a loss of smell.
If the hypogonadism inherited or develops before puberty, it prevents normal sexual maturity.
If the hypogonadism occurs after puberty, sexual development may be normal, but the client could have symptoms of low sex hormones – similar like menopause symptoms.
Lack of development during puberty is a main cause of all symptoms typical for hypogonadotropic hypogonadism. Main symptom is AMENORRHEA (absence of periods) which is a result of incomplete hormonal development during puberty.
Unfortunately amenorrhea is not only symptom of this disease. Most common symptoms include the following:
- Amenorrhea (absence of menstrual periods);
- Incomplete sexual maturation;
- Absence of secondary sexual characteristics such as pubic and underarm hair;
- Underdeveloped breasts and/or shrinking of breasts;
- Short stature (in some cases);
Young woman with hypogonadotropic hypogonadism
- Inability or reduced ability to smell (in some cases);
- Pain during sexual intercourse;
- Underdeveloped and dry vagina (lack of lubrication);
- Loss of bone mass (osteoporosis);
- Decreased libido;
- Hot flashes;
- Sleep disturbances;
- Symptoms of urinary bladder discomfort (frequent urination, frequent urinary infections);
- Heart disease (sometimes).
Diagnosis of the hypogonadotropic hypogonadism is not complicated and should be done in specialized clinics. Few tests could be enough for final diagnosis:
- Blood tests for main female hormones;
- So called “LH test” (Luteinizing hormone test) – LH response to Gonadotropin-releasing hormone (GnRH)
- Magnetic resonance imaging (MRI) of the head for excluding the noncancerous growth in the pituitary gland
Only solution for hypogonadotropic hypogonadism is hormonal replacement therapy which includes all needed female hormones which can insure proper development. As soon hormonal replacement starts, sooner and better the results could be.
Modern treatment of the hypogonadotropic hypogonadism could include hormonal pills, injections and/or slow-release skin patches.
In case of pituitary tumors surgical treatment would be required.
With the right in-time hormonal treatment, girls with hypogonadotropic hypogonadism could have almost normal puberty development and sometimes fertility could be restored. But permanent treatment monitoring and management would be needed as treatment could be long (months or years).
Possible negative consequences of the hypogonadotropic hypogonadism could include:
- Delayed puberty (if not treated);
- Infertility (if not treated);
- Sexual dysfunction (if not treated);
- Social conflicts, emotional stress and low self-esteem (because of short high, lack of puberty development and absence of menstrual periods).
Genetic counseling and genetic tests would be recommended to girls and women with history of inherited hypogonadotropic hypogonadism.
It is important to mention that prevention of serious head injuries could reduce the risk of pituitary injury-associated hypogonadotropic hypogonadism.
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