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Juvenile Hypogonadism Booklet

Juvenile Hypogonadism booklet

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Juvenile Hypogonadism

Hypogonadism, usually primary, identified early in life.

About the Condition

Hypogonadism occurs when the ovaries or testes (gonads) do not produce enough gonadal hormones (progesterone, estrogen, and testosterone). Hypogonadism may be present from birth due to genetic factors or may develop in adult life. The ovaries or testes atrophy (shrink), so they cannot produce enough germ cells (ova or sperm). Hypogonadism affects the growth of the brain, bone, muscle, fat, body hair, and breasts.

Primary hypogonadism is failure of the sex glands (testes or ovaries) to develop properly because they do not produce enough gonadal hormones (progesterone, estrogen, and testosterone). Consequently, the sex glands cannot produce enough sperm or ova. Untreated primary hypogonadism means the child will likely be sterile.

Hypogonadism also seriously affects the growth of the child’s brain, bone, muscle, fat, body hair, and breasts. The child will not develop age-appropriately.

Secondary hypogonadism is failure of the pituitary gland in the brain to stimulate the testes or ovaries adequately. This may be because the child inherited a gene called KISS, or the hypothalamus in the brain has a disturbance in gonadotrophin releasing-hormone (GnRH) secretion, or a lesion develops in the pituitary gland after birth.

If childhood hypogonadism is treated correctly with hormone replacement therapy, then affected men and women can go on to lead a normal life.

Next page: Symptoms of Childhood Hypogonadism

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Symptoms of Childhood Hypogonadism

  • High pitched voice
  • Absent facial hair
  • Decreased or absent body hair
  • Eunuchoidal body proportions
  • Female escutcheon
  • Testicular volume < 4 mL
  • Testicular length < 2.5 cm
  • Penile length < 5 cm
  • Smooth scrotum with no rugae, small prostate

Symptoms in Childhood

If a boy develops hypogonadism that goes unnoticed until age 4 or 5, then he probably has Klinefelter syndrome.

In 25% of boys, there is a telltale condition called clinodactyly, where the little finger curves toward the ring finger because the middle bone is wedge-shaped instead of rectangular. He is likely taller than 75% of his classmates, with long, thin limbs, sparse body hair, and weak skeletal muscles. This appearance is called a eunuchoid body type.

The school-age boy with hypogonadism from Klinefelter syndrome probably has characteristic knock-knees (genu valgum), high arches (pes cavus), protruding lips, a projecting jaw (prognathism), and very widely-spaced eyes (hypertelorism). If he is Caucasian or Black, epicanthal skin folds on his upper eyelids at the inner corner give him an oriental look. He exhibits poor coordination because fused bones in his forearms make it difficult for him to rotate his arms (radioulnar synostosis).

This boy has a quiet, tractable personality with occasional tantrums and aggression. About 5% of boys have a normal constitutional lag from an immature hypothalamus in the brain, which they eventually outgrow. Testosterone replacement therapy (TRT) can stimulate puberty in young boys.

Symptoms in Adolescence

If a young person develops hypogonadism, then he or she will be infertile and may be sexually dysfunctional. Be prepared for hypogonadism that results from cancer treatment, a secondary disease like diabetes, infections like mumps, or toxins like herbicides used on farms that run off into the water supply.

Adolescents with hypogonadism appear much more immature than their peers. Boys with adolescent-onset hypogonadism do not develop a beard, strong muscles, or voice deepening. They demonstrate low energy and show little interest in sex. Their sexual function is decreased and their genitals are smaller in comparison to other young men. They often develop female breasts (gynecomastia).

Testosterone replacement therapy (TRT) can initiate their secondary sex characteristics and make them appear normal.

Next page: Causes of Childhood Hypogonadism

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Causes of Childhood Hypogonadism

Primary Cause: Genetic eg Klinefelter syndrome (XXY), XX males with SRY gene

Hypogonadism has many possible causes that your doctor must investigate:

  • Alstrom syndrome
  • Anorexia nervosa (excessive dieting)
  • Cancer of the gonads or prostate gland
  • Cirrhosis of the liver
  • Chemotherapy
  • Cushing syndrome
  • Diabetes mellitus
  • Environmental toxins (2,4-D; atrazine; borax; butylate; and methoxychlor)
  • Gaucher disease
  • Genetic disorders
  • Hemochromatosis
  • Histiocytosis
  • HIV (the virus that causes AIDS)
  • Hyperprolactinemia (too much prolactin, the milk-producing hormone) from a pituitary tumor or medicine like metoclopramide
  • Idiopathic Hypogonadotropic Hypogonadism
  • Kallmann syndrome
  • Klinefelter syndrome (XXY syndrome)
  • Laurence-Moon Biedl syndrome
  • Liver and kidney diseasev
  • Malnutrition
  • Polycystic ovarian syndrome
  • Prader-Willi syndrome
  • Prescription drugs, like methadone, spironolactone, and ketokonazole
  • Radiation
  • Removal of the pituitary gland to treat a pituitary or breast tumor
  • Sarcoidosis
  • Sickle cell anemia
  • Street drugs, like heroin and marijuana
  • Stress
  • Trauma to the gonads or head
  • Tuberculosis
  • Turner’s syndrome
  • Viral orchitis if a male gets mumps after puberty
  • XYY syndrome

If the pituitary gland in the brain does not produce enough luteinizing hormone (LH) in a boy, then the Leydig cells in his testes do not secrete testosterone. If the pituitary does not produce enough follicle stimulating hormone (FSH), then the tubules where sperm should be manufactured do not develop. The boy’s blood tests usually show low or low-normal FSH and LH and low testosterone.

Can progress onwards to Oestoporosis, infertility, chronic fatigue, psychosocial factors.

Palpation of testes, physical examination.

Diagnosis

Genetic testing.

Next page: Treatment and Management of Childhood Hypogonadism

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For men

AndroForte® 2% Testosterone Cream is specially formulated for men with low blood testosterone levels.

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Treatment and Management of Hypogonadism in Childhood

Klinefelter, Turner and Kallmann syndromes have no cures.

They are permanent genetic disorders. Hormone replacement therapy controls the symptoms.

Affected children do not need a special diet, or to restrict their activities. Hospital care is not required. Treatment is on an out-patient basis.

Boys receive testosterone replacement therapy (TRT) beginning at 11 or 12 years old. Testosterone reduces the gonadotropin level to high normal and gradually virilizes the affected boy, giving him male secondary sex characteristics, like a beard, body hair, and a male-pattern fat distribution.

Testosterone is the treatment of choice for Klinefelter syndrome. Testosterone can reduce the gonadotropin level to high normal. Gradually, testosterone will virilize the boy, giving him male secondary sex characteristics, like a beard, body hair, and a male-pattern fat distribution.

When your son is 11 or 12 years old, the doctor will commence testosterone supplementation. Testosterone treatment options include injections, gels and scrotal cream.

Your son needs blood drawn (venipuncture) to check the gonadotropin hormone levels. The doctor closely monitors your son’s growth and the development of male secondary sex characteristics. Testosterone is sometimes called exogenous androgen. Boys with short AR CAG repeats in their gene sequence respond better to androgen therapy than boys with long CAG repeats.

Adult males with Klinefelter syndrome visit the doctor every two or three weeks to receive an intramuscular injection of 200 milligrams of either testosterone enanthate or cyprionate. Ask your doctor if AndroForte® 5% cream is a suitable alternative for you. Unlike intramuscular injections, the cream is painless. The daily dose you receive with cream is even. Injections produce uneven levels because they wear off and have to be replenished every 7 to 22 days.

Next page: Further information about Childhood Hypogonadism

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Topics discussed in the Understanding Childhood Hypogonadism booklet include:

  • What is childhood hypogonadism?
  • What causes hypogonadism?
  • What hormones are involved?
  • When does hypogonadism occur?
  • How many people have hypogonadism?
  • How dangerous is hypogonadism?
  • How is hypogonadism treated?
  • What are my treatment options?
  • How can I decrease the symptoms?
  • Who is most likely to have hypogonadism?
  • What is a normal appearance?
  • What can I expect during my doctor’s visit?
  • What are the pros and cons of natural hormone treatments versus synthetics?

Testosterone for Men FAQ's

AndroForte® 5

AndroForte 5 testosterone cream packaging

For men

AndroForte® 5% Testosterone Cream is specially formulated for men with low blood testosterone levels.

Downloadable Resources

AndroForte® 2

AndroForte 2 testosterone cream packaging

For men

AndroForte® 2% Testosterone Cream is specially formulated for men with low blood testosterone levels.

Downloadable Resources

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