Adrenogenital Syndrome .
Adrenogenital Syndrome (AGS) is a common inherited form of adrenal insufficiency. AGS is a disease that affects the manufacture of the "stress" hormone, cortisol. Cortisol is released into the blood stream from the adrenal gland, a small organ near the kidney. This group of diseases is due to mutations (genetic defects) in the genes coding for several enzymes needed for the production of adrenal cortex hormones. This enzyme is necessary for efficient production of two vital adrenal steroid hormones: cortisol and aldosterone. .
Deficient production of these substances causes disruption in the delicate balance of hormones. Sensing low levels of cortisol, the adrenal, directed by the master hypothalamus and pituitary glands, goes into high gear. Because cortisol production is impeded, the adrenal cortex instead manufactures androgens, or male steroid hormones, an undesired by-product. Females with Adrenogenital Syndrome are born with an enlarged clitoris and normal internal reproductive tract structures. Males have normal genitals at birth. Adrenogenital Syndrome 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 short, while one part of the adrenal functions poorly, making inadequate amounts of cortisol and aldosterone, another portion of the gland over-produces.This group of conditions of similar sources. A family of autosomal recessive disorders of steroid. Hormone production in the adrenal glands leading to a deficiency of cortisol, the stress fighting hormone. The master hormonal regulatory gland, the pituitary, sensing the deficiency, secretes massive amounts of the stimulating hormone corticotropin to bring the cortisol levels up to normal. This hormone in turn causes the adrenal glands to overproduce certain intermediary hormones which have testosterone-like effects on the fetus and child, leading to so-called virilization.
