Adrenarche

Premature adrenarche or pubarche is the early appearance of pubic hair, traditionally before the age of 8 years in females and 9 years in males. Androgens are "male like" hormones that are released from the adrenal gland in males and females. These hormones cause the development of underarm hair, acne, body odor and an increase in growth and bone maturation. Premature adrenarche occurs when the adrenal gland "turns on" a little early. It is not serious and does not usually advance to true early puberty.

Premature adrenarche is usually considered a variation of normal growth and development, and it is caused by the earlier than normal secretion of androgens.

The development of pubic hair, underarm hair, body odor or acne can be embarrassing for children. To minimize the visibility of these changes underarm hair may be shaved and a mild deodorant may be used. It is important to reassure your child that all children experience these changes at some point, and for them changes have occurred a little early. It is always important to talk to your child about their concerns and feelings.

It is important to differentiate premature adrenarche from true precocious (early) puberty because the causes and treatments are very different. True precocious puberty would also Include breast development in girls, enlargement of testes in boys, and hormone levels that are increased for age. It is also important to make sure there is not an abnormality in the adrenal glands. Initially, blood tests to check puberty and adrenal hormones may be done. A hormone stimulation test called an ACTH stimulation test may be done to measure these hormones more precisely. The test takes about one hour. During this test, your child will be given a medicine to stimulate production of hormones. Blood will be drawn at different times during the test to measure the amount of hormones present. An X-ray of the left wrist may be done to measure your child's bone age. Bone age is one measure of proper growth. Magnetic resonance imagery (MRI) of the brain and/or an ultrasound of the pelvis/abdomen may be done to provide information about the cause of the early development.

Congenital Adrenal Hyperplasia (CAH) is an inherited disorder of the adrenal glands. The adrenal glands, located on the top of the kidneys, produce three types of hormones called cortisol, aldosterone, and androgens. The life essential hormones are cortisol and aldosterone. Cortisol is  an energy and blood sugar regulating hormone. Aldosterone is a sail and water-regulating hormone. Androgens are "masculine hormones" that cause pubic hair growth and masculine changes in puberty, called virilization. Normally, there is an enzyme fa protein in the body that causes a chemical change) that is necessary to make cortisol and aldosterone. In CAH the enzyme is either missing. partially inactive, or totally inactive which leads to a 

"roadblock" in the "hormone production highway". If the enzyme is missing or severely inactive, both cortisol and aldosterone will not be produced. lf the enzyme is partially inactive, the cortisol produced will be reduced. In addition, the other hormones which are in the same or different pathway "highway" to make cortisol and aldosterone are also effected and may be may be abnonnally low, or abnormally high. ACTH, produced from the pituitary gland, which is located at the base of the brain, stimulates the adrenals to make the above mentioned hormones. 

CAH, depending on the missing or inactive enzyme, can be the virilizing (masculinizing) or the feminizing type, as well as, saltwasting or nonsaltwasting type. The virilizing type is caused by the overproduction of androgens, which develops male characteristics. The feminizing type is caused by lack of necessary male hormones in the males that result in a more female appearance of the genitalia. Salt wasting applies to children whol ose saltf rom the kidney due to lack of aldosterone. Non-saltwasting type applies to children who produce enough aldosierone, but not enough cortisol. Usually people with non-saltwasting CAH have a milder degree of CAH.

CAH is a congenitally inherited disorder. This means that your child was born with the disorder. The "pattern" for the baby is contained in the chromosomes (genes) inherited from the parents. The chromosome for the missing or inactive enzyme was passed on to the child from both parents. In general, parents of children with CAH are healthy, but each parent carried the partial trait for CAH. 

Depending on it's severity the salt wasting form of CAH can produce poor feeding, vomiting dehydration, low blood pressure, weight loss and poor growth in the newborn.

In children with the milder form symptoms may not appear until later and  the androgens produced in excess may cause rapid growth, advanced bone age, and early develoipent of acne and body hair. Even though these children are taller than their same aged friends, they may end up as short adults due to the rapid closing or maturation of the growth plates. In teens cystic acne, excess body hair and in girls menstrual irregularities. 

CAH is diagnosed by a blood sample test from your child. Cortisol and the other adrenal hormone levels will be measured. Your physician may also request an adrenal stimulation called an ACTH stimulation test, (a blood test that requires multiple samples). The genetic cause of CAH can be determined by doing a blood test that looks directly at the structure of the DNA gene. Other family members may find out if they are carriers of CAH by having similar blood tests. 

Your physician may order additional tests such as ultrasounds, boneage X-rays or other X-rays of the abdomen and pelvis, to visualize the adrenal glands or the structure of the internal genitalia. 

If you have a child with CAH, and have another pregnancy, tests may be done to determine the baby's sex and whether the unborn baby has CAH. If the fetus is a female and has CAH, prenatal medication may be given 10 prevent the development of male-like external genitalia.