disorders

1. ADRENAL GLAND DISORDERS
This include those related to hypo function, which can lead to acute or chronic insufficiency
and those related to hyper function (over activity) which often leads to overproduction of
androgen or cortisol.
ACUTE ADRENOCORTICOL INSUFFICIENCY
- In acute adrenocorticol insufficiency, the function of the entire cortical adrenal glad
suddenly becomes insufficient. Usually this occurs in association with severe
overwhelming infection, typically involving hemorrhagic destruction of the adrenal
glands. (Meningococcemia) It also occurs when corticosteroid therapy that has been
maintained at high levels for long periods is abruptly stopped.
Signs and Symptoms:
1. The bp drops to extremely low levels; the child appears ashen gray ad may be pulseless.
2. Temperature becomes elevated; dehydration and hypoglycemia (abnormally low
concentration of blood glucose) are marked.
3. Sodium and chloride blood levels are very low but serum level is elevated because there is
inverse relationship between sodium and potassium values.
4. The child is prostrate and seizure may occur.
Management:
1. Immediate replacement of cortisol.
2. Administration of deoxycorticosterone acetate (synthetic equivalent of aldosterone)
3. IV 5% glucose in NSS- to restore bp, sodium and blood glucose level.
4. A vasoconstrictor may be necessary- to elevate bp further.
CONGENITAL ADRENAL HYPERLASIA
- -Is a syndrome that is inherited as an autosomal recessive trait. The primary defect is an
ability of the adrenal glands to synthesize cortisol from its precursor. Because of this, the
level of ACTH increases, stimulating the adrenal glands to improve function. Although the
adrenal enlarges, they still can’t produce cortisol, but they overproduce androgen.
Signs and Symptoms:
Female:
1. The female infant is born with clitoris which appears more like a penis- due to excessive
androgen production at early fetal life.
2. Sinus between the urethra may present.
3. The girl resembles a boy with undescended testes and hypospadias.
4. If not treated, pubic and axillary hair and acne will appear precociously and deep
masculine voice will develop.
5. At puberty, there will be no breast development and menstruation.
Male:
1. By 3 or 4 years of age, these boys have pubic hair and enlargement of the penis, scrotum
and prostate.
2. Acne and deep, mature voice may be present.
3. Testes do not enlarge; they appear small in relation t the size of the penis.
4. Child will be infertile because spermatogenesis does not occur.
Management:
1. Both male and female are given corticosteroid agent (hydrocortisone) - to replace what
they can’t produce normally.
2. The child needs periodic analysis of serum cortisol levels and growth measurements- to
estimate effectiveness of therapy.
3. Chronic villi sampling (as early as 6-8 wks.) and amniocentesis (at 15 wks.) must be done to
identify the fetus with this kind of disorder.
SALT- LOSING FORM OF CONGENITAL ADRENOGENITAL HYPERPLASIA
- Salt as well as fluid is not retained by the body because there is inadequate aldosterone
due to the blockage of cortisol formation.
Signs and Symptoms:
1. Affected infants may begin to have vomiting, diarrhea, anorexia, lack of weight, extreme
dehydration. If symptoms remained untreated, the extreme loss of salt and fluid can lead to
collapse and death as early as 48-72 hours after birth.

2. 2. In boys, the first sign is inability to gain back their birth weight.
Management:
1. Taking of supplements hydrocortisone in conjunction with high amount of salt and DOCA
(synthetic aldosterone)- to maintain balance of fluid and electrolytes.
*long-acting DOCA- given once a month IM
*Capsules of DOCA- can be implanted SC as long therapy
2. Fludrocortisone (florinet), a mineral corticoid may be given orally to aid salt
Retention.
CUSHING SYNDROME
- Is caused by overproduction of adrenal hormone cortisol results from increase ACTH
production due to a pituitary tumor. The peak age of occurrence is 6 or 7 years old but
can occur as early as infancy.
Signs and Symptoms:
1. Overproduction of cortisol results in increased glucose production.
2. Fat accumulation on cheeks, chin and trunk can cause moon faced, stocky appearance.
3. Extremities appear to be thin due to muscle wasting.
4. Immuno compromised child
5. Child’s face tends to be red, especially the cheeks due t hyperpigmentation
6. Purple striae resulting from collagen deficit appear on the child’s hips, abdomen and
thighs.
7. Polyuria develops as the body tries to excrete increased glucose levels.
8. Hypertension (arteriosclerosis) due to increased salt and water retention.
Management:
1. Treatment of Cushing syndrome is surgical removal of the causative tumor. The prgnosis
depends on whether the tumor is benign or malignant; carcinoma of this type tends to be
metastasizing rapidly.
2. If a major part of adrenal gland is removed, the child will need cortisol therapy.
3. If major portion of pituitary gland is removed, replacement of pituitary hormone is
needed.
4. Observe the child for signs of shock after surgery.