Pathology of the genital adrenal cortex

Reason

Adrenal hyperplasia

Hyperplasia, adenoma, carcinoma.

Congenital adrenal hyperplasia due to a lack of enzymes

Enzyme hydroxylase P450C21, P450C18, P450C17, P450C11β. Men 3 β hydroxysteroid dehydrogenase (3β HSD).

Clinical

Symptoms vary depending on the illness originating from the foetus, infant, child or adult. Often think of the diagnosis of hypercritical adrenal cortex when there is hypertension, hypokalaemia associated with hirsutism, amenorrhea, acne and masculinization.

Influence from the foetus

Male foetus: a few symptoms, only genital hypertrophy. Pregnancy: masculinization of the genitals.

Effects on young children

The growth of cartilage is premature, so children stop growing in height early. Boy: the scrotum has many dark wrinkles.

Girls: symptoms of masculinity, hirsutism, developed muscles; thick skin, sweaty, acne; raucous; large clitoris.

Affect puberty

Male children: Early puberty, pubic hair, large penis but small testicles, no sperm and ejaculation activity.

Female children: Breast not growing, uterus small, small ovaries, no period.

Influence on adults

Men: No specific symptoms.

Females: Symptoms vary from diminished femininity with amenorrhea, less or less period. Atrophy, infertility (no ovulation). If severe, there will be masculinizing symptoms: growing beard, chin; more hair on hands, genitals; oily skin, hoarse voice; large clitoris, enlarged lips.

Subclinical

17-cetosteroid in 24 h urine increased. Male> 20mg / 24h (normal <5mg); female> 14 mg / 24h (normal <3mg).

Dehydroepiandrosterone sulfate in plasma and in urine increased.

Testosterones in plasma and urine increased.

Treatment

K of the adrenal epithelium

Surgery. If this is not possible, use Mitotane.

Adrenal hyperplasia

Hydrocortisone dose 1-1.5 times the amount of hydrocortisone secreted per day (10-13mg) per square meter of body. Or can be estimated 10-26 mg Hydrocortisone / day; or Dexamethasone 0.5-1 mg / day.

The dose is adjusted according to 17-cetosteroid in urine, DHEA in plasma and concentration of precursors of cortisol.

If there are symptoms of salt loss: Syncortyl 1 mg/kg intramuscularly or maintenance dose 9α- Fluorohydrocortison 25-50μg / day.