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Adrenogenital Syndrome

Adrenogenital Syndrome Definition

The more proper term for adrenogenital syndrome is congenital adrenal hyperplasia or CAH. This refers to numerous other autosomal recessive disor which result from biochemical paths of cortisol setoidogenesis from cholesterol done by the adrenal glands. Most number of these conditions could have lesser or greater sex steroid production and they can alter primary or secondary sex characteristic development among adults, children and infant patients. Only a few cases of people with CAH can be appropriated with intersex condition although this attracted the attention of the American public in the 90's and since then, there have been various accounts that have been circulated. It is estimated that 95% of CAH cases are caused by 21-hydroxylase deficiency. Conditions Caused by CAH The most common conditions that are caused by adrenogenital syndrome are: ambiguous genitals (in some female patients wherein it can be initially hard to know the exact sex of the infant); salt-wasting that causes vomiting (which may lead to dehydration and eventually—death); the early appearance of pubice or the occurrence failure or the delay of puberty (including sexual infantilism); too much facial hair; virilization; irregular menstrual periods during the adolescent stage; infertility caused by anovulation; and hypertension.

Adrenogenital Syndrome Historical Background

The earliest recorded description and observation made of adrenogenital syndrome was made by an Italian anatomist named Luigi De Crecchio. He stated that it is extremely hard to determine the sex of the patient at on the earliest stage of the patient’s life. He observed a cadaver that was taken to an anatomical theater of a certain hospital. He noted that the general physiognomy was male. He further noted that there were no feminine curves (to begin with); there was a penis (posteriorly curved and measured at 6 centimeters and at 10 centimeters when stretched); the corona was measured at 3 centimeters and its circumference was at 8 centimeters; the pubic hair distribution was obviously male but the feminine characteristics begin with lower extremities which are quite delicate but covered with hair. De Crecchio also observed that there was hypospadias (first grade). The top of the penis also showed two folds of skin which encircled the penis on either side. On the other hand, there were loose folds of skin which resembled the labia majora. The confirmation that the cadaver was female came up when the internal organs were examined. The patient had a uterus, normal vagina, ovaries and fallopian tubes. De Crecchio’s interviews with several witnesses have accounted that the patient had conducted himself as a male during his life. The patient’s causes of death were diarrhea and episodes of vomiting.

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