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Adrenal gland disorders
ADRENAL GLAND
•
Either of two small, dissimilarly shaped endocrine
glands, one located above each kidney, consisting of the
cortex, which secretes several steroid hormones, and
the medulla, which secretes epinephrine.
• The adrenal cortex :produces corticosteroids which
include glucocorticoids (primarily cortisol),
mineralocorticoids (primarily aldosterone), and
androgens (primarily dehydroepiandrosterone and
androstenedione).
•
Function is regulated by the neuroendocrine hormones
from the pituitary, hypothalamus and renin-angiotensin
system.
•
Adrenal androgens--chief physiologic activity occurs after conversion
to testosterone.
• The adrenal medulla :is composed of chromaffin cells, which
synthesize and secrete catecholamines (mainly epinephrine and lesser
amounts of norepinephrine).
•
•
Chromaffin cells also produce bioactive amines and peptides (eg,
histamine, serotonin, chromogranins, neuropeptide hormones
Epinephrine and norepinephrine(( the major effector amines of the
sympathetic nervous system)), are responsible for the “flight or fight”
response (ie, chronotropic and inotropic effects on the heart;
bronchodilation; peripheral and splanchnic vasoconstriction with
skeletal muscular vasodilation; metabolic effects including
glycogenolysis, lipolysis, and renin release).
•
Excess quantities of epinephrine and norepinephrine are produced in
pheochromocytoma .
Function of the gland
•
HORMONE
FUNCTION
• ALDOSTERONE: Renal : Na & Cl reabsorption; K excretion
•
GI : Na absorption
• GLUCO-CORTICOIDS:
Increase serum glucose by
gluconeogenesis & glycogenolysis especially during STRESS
•
Blocks inflammation(lymphokines)
•
Counteracts effect of histamine-inflammation
• sex HORMONE: Physiologically insignificant
•
Becomes useful during menopause in women
SYMPTOMATOLOGY
ALDOSTERONE DEFICIENCY
Decrease in plasma volume leading to dehydration.
Hypotension to shock.
Increased K.
CORTISOL DEFICIENCY
ANOREXIA, N/V, ABDOMINAL PAIN, WT LOSS, LETHARGY
HYPOGLYCEMIA
HYPOTENSION
HYPERKALEMIA, WEAK PULSE
HYPERPIGMENTATION
IMPAIRED STRESS TOLERANCE
SEX HORMONE DEFICIENCY
LOSS OF BODY HAIR
LOSS OF LIBIDO OR IMPOTENCE
MENSTRUAL & FERTILITY DISORDER
ADRENAL CORTEX DISORERS
ADRENAL INSUFFICIENCY
ADRENAL CRISIS
CUSHING’S SYNDROME
ALDOSTERONISM
ADDISON’S DISEASE
•
Is a hormone deficiency caused by damage to the outer layer of
the adrenal gland (adrenal cortex).
•
Addison's disease is a disorder that results in the body producing
insufficient amounts of certain hormones produced by the adrenal
glands.
•
Sometimes, Addison's disease also involves insufficient production
of aldosterone, one of the mineralocorticoid hormones.
•
•
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Insufficiency of corticosteroids can be total when the operation of
all hormones drops out, and partial fallout of activity of one adrenal
hormone.
Addison's disease can be life-threatening.
Addison's disease can occur at any age, but is most common in
people ages 30 to 50.
ETIOLOGY
•
Autoimmune processes -80% of cases-corticosteroid,
aldost,androgen-all
•
Pituitary failure( atrophy, necrosis)-no
HYPERKALEMIA/PIGMENTATION
•
TB, Syphilis, coccid mycosis ,Histoplasmosis, CMV(AIDS)
•
Tumor ,Metastases , Amyloidosis, Hemochromatosis
•
Hemorrhage-warfarin, shock, major surgery, antiphospholipid.
•
Drugs that block corticosteroid synthesis (eg, ketoconazole).
•
All groves syndrome-achalasia, alacrima,neurologic disease.
• Addison's disease may coexist with diabetes mellitus or
hypothyroidism.
Destruction of
adrenal gland
Mineralocorticoid &
glucocorticoid deficiency
Mineralocorticoid deficiency
↑ in urinary excretion of Na,
↓ in urinary excretion of K
Inability to concentrate urine,
with electrolyte imbalance
Glucocorticoid
deficiency
Disturbances in
carbohydrate, fat and
protein metabolism,
decrease liver glycogen
severe dehydration
plasma hypertonicity, acidosis,
hypotension
circulatory collapse
Hypoglycaemia and
anorexia
SIGNS & SYMPTOMS
• A. Manifestation, connected with the falling of mineral
corticoids:
• 1) DEHYDRATION develops owing to loss of sodium ions (decreases
rearbsortion) with the loss of water (polyuria).
• 2) HYPOTENSION - by decrease of circulating blood volume.
• 3) HEMOCONCENTRATION is connected with liquid loss, results to
disorders of microcirculation and hypoxia.
• 4) Decreasing of kidney blood circulation is stipulated by increase of
arterial pressure with disturbances of glomerular filtration and
development of intoxication (nitrogenemia);
• 5) HYPERKALEMIA -- by decrease secretion of potassium ions and
their output from the damaged cells.
• B. Manifestations by disorders of glucocorticoid
function of adrenal:
•
Muscle weakness and fast tiredness
•
Decrease of ability to remove water during water load (water poisoning).
•
Weight loss and decreased appetite
•
HYPOTENSION -- (permissive reaction on catecholamine’s)90%
•
Darkening of the skin (HYPERPIGMENTATION-- bronzed
disease)
•
Diffuse tanning over nonexposed as well as exposed areas over knuckles,
elbows, knees, nail beds, palmer creases, and posterior neck.
•
Nipples and areolar darken, new scars pigmented ,skin in pressure areas
•
Depression , emotional changes, mental irritability.
•
Salt craving
EXAMINATION &DIAGNOSIS
• Examine for hyperpigmentation:
 Hand: palmar creases
 Mouth and lips
 Areas usually covered by clothing: nipple
 Areas irritated by belts, straps, collars or rings
• Look for vitiligo
• Look for sparse axillary hair and pubic hair
• Examine the abdomen for adrenal scar
• Examine blood pressure for postural hypotension
• Signs of critical deterioration (Addisonian crisis):
– Shock (low BP, tachycardia)
– hypothermia
•
SCREENING AND DIAGNOSIS
•
Doctor will talk first about the medical history and the signs and symptoms.
•
If doctor thinks that patient may have Addison's disease, He may undergo
some of the following tests:
•
Blood test.
•
ACTH stimulation test.
•
Insulin-induced hypoglycemia test.
•
Imaging tests.
• BLOOD TEST:
•
Measuring blood levels of sodium, potassium, cortisol and ACTH gives the
doctor an initial indication of whether adrenal insufficiency may be causing
the signs and symptoms.
•
A blood test can also measure antibodies associated with autoimmune
Addison's disease.
ACTH stimulation test
This test involves measuring the level of cortisol in the blood before
and after an injection of synthetic ACTH.
If adrenal glands are damaged, the ACTH stimulation test shows that
the output of cortisol in response to synthetic ACTH is blunted or
nonexistent.
TREATMENT
•
•
•
•
•
•
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If patient receive an early diagnosis of Addison's disease,
treatment may involve taking prescription corticosteroids.
Because the body isn't producing sufficient steroid hormones,
doctor may have recommend to take one or more hormones to
replace the deficiency.
Cortisol is replaced using hydrocortisone (Cortef), prednisone or
cortisone. Fludrocortisone (Florinef) replaces aldosterone, which
controls the body's sodium and potassium needs and keeps the
blood pressure normal.
In addition, doctor may recommend treating androgen deficiency
with an androgen replacement called dehydroepiandrosterone.
Some studies indicate that, for women with Addison's disease,
androgen replacement therapy may improve overall sense of wellbeing, libido and sexual satisfaction.
These hormones are given orally in daily doses that mimic the
amount body normally would make, thereby minimizing side effects.
If ill with vomiting and can't retain oral medications, may need
corticosteroid injections.
CUSHING’S SYNDROME
Sustained over production
of glucocorticoids by
adrenal gland from ACTH by
pituitary tumor
Excessive glucocorticoid
administration.
• Cushing's Disease: Is the result of a
pituitary tumor and the inappropriate
production of ACTH.
Cushing's Syndrome: Causes the same
symptoms, but is a disorder marked by
overproduction of adrenal hormones,
Cushing’s syndrome is all other forms of
Cushing’s.
CLINICS
DIAGNOSIS:
• 24-hour urine cortisol: 24-hour urine cortisol
(or urine free cortisol) is often used to evaluate
overall cortisol production. One out of four 24-hour
urine samples may be normal and other testing
may have to be performed. Like the midnight
cortisol test, it is recommended that this test be
repeated if results are abnormal, to avoid false
positives.
• Midnight plasma cortisol and late-night
salivary cortisol measurements: Normally,
cortisol production is suppressed at midnight but
in Cushing syndrome, this does not happen.
Therefore, an elevated blood (plasma) level at
midnight suggests Cushing syndrome. Blood is
the preferred sample, but this usually requires a
hospital stay. Alternatively, a saliva sample can
be collected late at night at home and then
tested. It is recommended that a sample be
collected three nights in a row. If only a single
sample is collected and tested, the test should
be repeated if the result is outside the
established reference range, to confirm the
results and to avoid false positives.
• Dexamethasone suppression screening test:
Dexamethasone is a synthetic steroid that mimics cortisol
in the feedback inhibition of corticotropin-releasing
hormone (CRH) and adrenocorticotropin hormone (ACTH)
production. A normal response to dexamethasone is
suppression of cortisol secretion.
• Dexamethasone can be used in different dosages for
different purposes as a diagnostic test. One version
involves giving a fairly low dose (1 mg) at bedtime to
prevent the rise in ACTH and cortisol that normally occurs
during sleep. Between 8 and 9 am the next morning, a
blood sample is drawn and the cortisol level is measured.
In healthy individuals, the level will be low (suppressed); in
those with Cushing syndrome, the level will not be
suppressed
TREATMENT
• Reducing corticosteroid use.
• Surgery. If the cause of Cushing syndrome is a
tumor, your doctor may recommend complete
surgical removal. Pituitary tumors are typically
removed by a neurosurgeon, who may perform the
procedure through your nose.
• Radiation therapy: Radiation can be given in
small doses over a six-week period, or with a
technique called stereotactic radiosurgery
(Gamma Knife surgery). In the latter procedure,
administered as a single treatment, a large dose of
radiation is delivered to the tumor, and the
radiation exposure to surrounding tissues is
minimized.
• Medication:Medications to control excessive
production of cortisol at the adrenal gland include
ketoconazole (Nizoral), mitotane (Lysodren) and
metyrapone (Metopirone)
• Mifepristone (Korlym) is approved for people with
Cushing syndrome who have type 2 diabetes or
glucose intolerance. Mifepristone does not
decrease cortisol production, but it blocks the effect
of cortisol on your tissues.
• The newest medication for Cushing's is pasireotide
(Signifor), and it works by decreasing ACTH
production from a pituitary tumor. This medication
is given as an injection twice daily. It's
recommended if pituitary surgery is unsuccessful
or cannot be done
• Ketoconazole (600 to 1200 mg/day)
• metyrapone(2-3 g/day)
• Mitotane(2-3 G/day)- slow onset
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