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All about congenital adrenal hyperplasia types of congenital adrenal hyperplasia causes of congenital adrenal hyperplasia symptoms of congenital adrenal hyperplasia diagnosis of congenital adrenal hyperplasia treatments for congenital adrenal hyperplasia

What is congenital adrenal hyperplasia?

Congenital adrenal hyperplasia (CAH) refers to any of several autosomal recessive diseases resulting from defects in steps of the synthesis of cortisol from cholesterol by the adrenal glands. Most of these diseases involve excessive or defective production of sex steroids and can pervert or impair development of primary or secondary sex characteristics in affected

infants, children, and adults. Only a small minority of people with CAH can be said to have an intersex condition, but this attracted American public attention in the late 1990s and many accounts of varying accuracy have appeared in the popular media.

The human body has two adrenal glands, one on top of each kidney. These glands form part of the endocrine system, which works in conjunction with the nervous system and the immune system to help the body cope with different events and stresses. Adrenaline is the most well known hormone secreted by the adrenal glands. Other important hormones include cortisol, aldosterone and the sex hormone androgen. All these hormones are synthesised from cholesterol, which is an oily substance manufactured by the body and found in animal, and some vegetable, products. Particular enzymes are needed to help convert cholesterol into the adrenal hormones. The underlying problem in CAH is that that not enough of these enzymes are produced. This is due to a genetic or inherited 'fault'. The symptoms of the disorder depend on the particular enzymes affected. There is no cure for CAH, but the disorder can be managed very well with medications.

Congenital adrenal hyperplasia (CAH) is a form of adrenal insufficiency in which the enzyme that produces two important adrenal steroid hormones, cortisol and aldosterone, is deficient. Because cortisol production is impeded, the adrenal gland instead overproduces androgens (male steroid hormones). Females with CAH are born with an enlarged clitoris and normal internal reproductive tract structures. Males have normal genitals at birth. CAH causes abnormal growth for both sexes; patients will be tall as children and short as adults. Females develop male characteristics, and males experience premature sexual development.

In its most severe form, called salt-wasting CAH, a life-threatening adrenal crisis can occur if the disorder is untreated. Adrenal crisis can cause dehydration, shock, and death within 14 days of birth. There is also a mild form of CAH that occurs later in childhood or young adult life in which patients have partial enzyme deficiency. CAH, a genetic disorder, is the most common adrenal gland disorder in infants and children, occurring in one in 10,000 total births worldwide. It affects both females and males. It is also called adrenogenital syndrome.

More information on congenital adrenal hyperplasia

What is congenital adrenal hyperplasia? - Congenital adrenal hyperplasia (CAH) refers to any of several autosomal recessive diseases resulting from defects in steps of the synthesis of cortisol from cholesterol by the adrenal glands.
What types of congenital adrenal hyperplasia are there? - In all its forms, congenital adrenal hyperplasia due to 21-hydroxylase deficiency accounts for about 95% of diagnosed cases of CAH.
What causes congenital adrenal hyperplasia? - Congenital adrenal hyperplasia is an inherited disorder. This particular group of genes contains instructions that the adrenal glands need in order to produce 21-hydroxylase.
What are the symptoms of congenital adrenal hyperplasia? - Female children with severe CAH might be born with male genitals. All children with severe CAH have masculine features. The adult onset form is far less severe than the childhood version.
How is congenital adrenal hyperplasia diagnosed? - Congenital adrenal hyperplasia is diagnosed by a careful examination of the genitals and blood and urine tests that measure the hormones produced by the adrenal gland.
What're the treatments for congenital adrenal hyperplasia? - The goal of treatment for congenital adrenal hyperplasia is to return the androgen levels to normal. Patients with CAH should see a pediatric endocrinologist frequently. 
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Topics in endocrine disorders

Adrenal insufficiency
Addison's disease
Congenital adrenal hyperplasia
Conn's syndrome
Cushing's syndrome
Nelson's syndrome
Pheochromocytoma
Bartter's syndrome
Neuroblastoma
Adrenocortical carcinoma
Hypoglycemia
Insulinoma
Rickets
Osteomalacia
Hyperparathyroidism
Hypoparathyroidism
Pituitary gland disorders
Thyroid gland disorders


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