According to Cohen (2008), congenital Adrenal Hyperplasia is a genetic defect of the adrenal glands.

People with this condition do not produce enough of the hormones cortisol and aldosterone, and produce too much of androgen. It affects both males and females. In children and adults with congenital adrenal hyperplasia, the adrenal glands, a pair of walnut-sized organs above your kidneys, typically don't produce enough of the hormone cortisol.

Some forms of inborn adrenal hyperplasia are more serious and induce adrenal crisis in the newborn payable to salt atrophy. In this salt-losing kind of inborn adrenal hyperplasia, newborns produce serious symptoms soon after birth, including vomiting, dehydration, electrolyte changes, and cardiac arrhythmias. Untreated, this circumstance can head to death within 1 to 6 weeks after birth.

About 1 in 10,000 to 18,000 children are born with congenital adrenal hyperplasia. Congenital adrenal hyperplasia occurs among people of all races. Mild forms of the disease result in symptoms such as severe acne, excess facial and/or body hair, early development of pubic hair, receding scalp hairline, menstrual disturbances in females. More severe forms of the disorder can result in ambiguous genitalia in a newborn girl, as well as severe salt and hormonal imbalances in both girls and boys.

Congenital adrenal hyperplasia are usually treated by use of medication. Oral corticosteroid therapy corrects the endocrine deficiency. Prenatal therapy with a synthetic hormone called dexamethasone throughout pregnancy can allow proper development of the external genitalia in female fetuses. Reconstructive surgery for girls with masculine external genitalia is usually performed between the ages of 1 and 3 months to correct the abnormal

appearance. A mineralocorticoid is required in the salt-losing form. Genetic counseling is indicated for parents with a family history of congenital adrenal hyperplasia.

http://www.articlesfactory.com/articles/health/complete-information-on-congenital-adrenalhyperplasia.html

Congenital adrenal hyperplasia refers to a group of inherited disorders of the adrenal gland, also known as adrenogenital syndrome and 21-hydroxylase deficiency. Aldosterone is a hormone released by the adrenal glands. It is part of the complex mechanism used by the body to regulate blood pressure. Aldosterone increases the reabsorption of sodium and the excretion of potassium in the distal tubules of the kidneys. The reabsorption of sodium is accompanied by the reabsorption of water, which raises blood pressure.

Congenital adrenal hyperplasia can affect both boys and girls. People with congenital adrenal hyperplasia lack an enzyme needed by the adrenal gland to make the hormones cortisol and aldosterone. Without these hormones, the body produces more androgen, a type of male sex hormone.

The goal of treatment is to return hormone levels to normal. This is done by taking a form of cortisol (dexamethasone, fludrocortisone, or hydrocortisone) every day. People may need additional doses of medicine during times of stress, such as severe illness or surgery.The health care provider will determine the gender of a baby with ambiguous genitalia by checking the chromosomes (karyotyping). Girls with male-looking genitals will usually have surgery between ages 1 month - 3 months to correct the abnormal appearance (Elsevier et. al, 2007)

http://www.scripps.org/articles/1578-congenital-adrenal-hyperplasia

Congenital adrenal hyperplasia (CAH) represents a family of autosomal recessive disorders in which there is a deficiency of one of the enzymatic activities necessary for cortisol synthesis. The result is increased adrenocorticotropic hormone (ACTH) secretion, adrenal hyperplasia, overproduction of the adrenal steroids that do not require the deficient enzyme activity, and deficiency of the steroids distal to the disrupted enzymatic step. An abnormality in each of the enzymatic activities required for cortisol synthesis has been described. Because some of the disorders also result in defects in gonadal steroidogenesis, abnormalities in gonadal hormone production also may be present. The symptoms and signs of each enzymatic disorder depend on which hormones are deficient and which are produced in excess. Much has been learned in recent years about the enzymes of adrenal steroidogenesis, the genes encoding them, and the genetic mutations resulting in CAH (Levine, 2000)

http://pedsinreview.aappublications.org/content/21/5/159.short

The term congenital adrenal hyperplasia (CAH) encompasses a group of autosomal recessive disorders, each of which involves a deficiency of an enzyme involved in the synthesis of cortisol, aldosterone, or both. The clinical manifestations of each form of congenital adrenal hyperplasia are related to the degree of cortisol deficiency and/or the degree of aldosterone deficiency. In some cases, these manifestations reflect the accumulation of precursor adrenocortical hormones. When present in supraphysiologic concentrations, these precursors lead to excess androgen production with resultant virilization, or because of mineralocorticoid properties, cause sodium retention and hypertension (Wilson, 2012) http://emedicine.medscape.com/article/919218-overview#showall

Congenital adrenal hyperplasia is a group of autosomal recessive disorders resulting from the deficiency of one of the five enzymes required for the synthesis of cortisol in the adrenal cortex. The most frequent is steroid 21-hydroxylase deficiency, accounting for more than 90 percent of cases. Since the last Medical Progress article on this topic was published in the Journal in 1987,1 much has been learned about the genetics of the various clinical forms of 21-hydroxylase deficiency, and correlations between the genotype and the phenotype have been extensively studied. Gene-specific prenatal diagnosis is now feasible, and prenatal treatment has been more widely implemented. This discussion will be limited to the most common form of congenital adrenal hyperplasia ( White, 2003)

http://www.nejm.org/doi/full/10.1056/NEJMra021561