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Disorders of adrenal gland
1. 1 MAGDI AWAD SASI 2013 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.((NOT THE MEDULLA))
Glucocorticoid-- Prominent effects include anti-inflammatory actions and
increased hepatic gluconeogenesis and help body respond to stress. .
Mineralocorticoids regulate electrolyte transport across epithelial
surfaces, particularly renal conservation of Na in exchange for K.
Glucocorticoid and Mineralocorticoids are essential for life .
2. 2 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
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).
Hypofunction may be primary (malfunction of the adrenal gland itself, as
in Addison's disease) or secondary (due to lack of adrenal stimulation by
the pituitary ((2ry)) or hypothalamus((3ry)).
Hyperfunction produces distinct clinical syndromes.
Hypersecretion of androgens results in adrenal virilism;
of glucocorticoids, Cushing's syndrome;
And of aldosterone, hyperaldosteronism (aldosteronism).
These syndromes frequently have overlapping features.
Hyperfunction may be compensatory, as in congenital adrenal
hyperplasia, or due to acquired hyperplasia, adenomas, or
adenocarcinomas.
Excess quantities of epinephrine and norepinephrine are produced in
pheochromocytoma .
3. 3 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
FUNCTION OF THE GLAND:
Cortex -Stimulated by ACTH
Hormone precursor:
Cholestrol
Secretes:
Cortisol
Aldosterone
Sex hormones: Androgen ,Estrogen
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(IL6,PG,lymphokines)
Counteracts effect of histamine-inflammation
Required for ANG2-maintain vasculature
SEX HORMONE Physiologically insignificant
Becomes useful during menopause in women
4. 4 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
SYMPTOMATOLOGY:
ALDOSTERONE DEFICIENCY
Decrease in plasma volume leading to dehydration.
Hypotension to shock.
Increased K.
Metabolic acidosis .
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
5. 5 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
ADRENAL INSUFFICIENCY
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.
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.
Polyglandular atrophy
Addison's disease may coexist with diabetes mellitus or hypothyroidism.
6. 6 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
SIGNS AND SYMPTOMS:
Signs and symptoms of Addison's disease usually develop slowly, often over
several months, and may include:
Mineralocorticoid deficiency
↑ in urinary excretion of Na,
↓ in urinary excretion of K
Inability to concentrate urine,
with electrolyte imbalance
severe dehydration
plasma hypertonicity, acidosis,
hypotension
circulatory collapse
Glucocorticoid
deficiency
Disturbances in
carbohydrate, fat and
protein metabolism,
decrease liver glycogen
Hypoglycaemia and
anorexia
Destruction of
adrenal gland
Mineralocorticoid &
glucocorticoid deficiency
• Symptoms & Signs usually do not appear until ~90% of adrenal gland has
been compromised.
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.
7. 7 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
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.
6) Distal canales acidosis. It is connected with disorders of acidogenesis in
distal nephron canals.
7)HYPEREMESIS- Gastro-intestinal disorders (nausea, vomiting, diarrhea).
Loss of sodium (osmotic diarrhea) and intoxication have significant
meaning. This disorders without appropriate correction result to death.
Think of Addison’s in all those with unexplained abdominal symptoms.
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
(HYPOGLYCEMIA)
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
8. 8 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Irritability
Fever ,myalgia, arthralgia,vitiligo 10%.
Sometimes, however, the signs and symptoms of Addison's disease may
appear suddenly.
10. 10 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
• 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
11. 11 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
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.
BUSE
• Low Na
• High K
• Low HCO3
• Azotemia
FBC
• Elevated
hematocrit
• Low WBC
count
• Relative
lymphocytosis
• Increased
eosinophils
RBS / DXT
• Hypoglycemia
(< 3 mmol/L)
12. 12 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Blood chemistry • Low serum Na (< 135 mEq/L)
• High serum K (> 5 mEq/L)
• Ratio of serum Na:K: < 30:1
• Low fasting blood glucose (< 50 mg/dL [< 2.78 mmol/L])
• Decreased plasma HCO3 (< 20 mEq/L)
• Elevated BUN (> 20 mg/dL [> 7.1 mmol/L])
ACTH stimulation test
This test involves measuring the level of cortisol in the blood before and
after an injection of synthetic ACTH.
Plasma ACTH -INCREASED 1RY , DECREASED 2RY
ACTH signals – adrenal glands to produce
If adrenal glands are damaged, the ACTH stimulation test shows that the
output of cortisol in response to synthetic ACTH is blunted or nonexistent.
Preinjection
plasma cortisol
level (N= 138 –
690 nmol/L)
IV
Tetracosactide
250 µg
Plasma cortisol
level at 30 – 90
minutes post
injection (≥ 500
nmol/L
Failure to
respond →
adrenal
insufficiency (?
Primary /
secondary)
13. 13 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Insulin-induced hypoglycemia test
Occasionally, doctors suggest this test if pituitary disease is a possible
cause of adrenal insufficiency (secondary adrenal insufficiency).
The test involves checking the blood sugar (blood glucose) and cortisol
levels at various intervals after an injection of insulin.
In healthy people, glucose levels fall and cortisol levels increase.
Imaging tests
Abdominal x ray- Calcifications in the adrenal areas - PTB
Chest x ray----Small heart
Computerized tomography (CT) scan of the abdomen to check the size
of adrenal glands and look for other abnormalities that may give
insight to the cause of the adrenal insufficiency.
And may also suggest a CT scan or magnetic
resonance imaging (MRI) scan of the pituitary
gland if testing indicates the patient have
secondary adrenal insufficiency.
Negative
Synacthen
test
High plasma
ACTH level
ADDISON’S
DISEASE
14. 14 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
TREATMENT
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 well-being, 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 facing a stressful situation, such as an operation, an infection or a minor
illness, doctor will suggest a temporary increase in dosage.
If ill with vomiting and can't retain oral medications, may need
corticosteroid injections.
COPING SKILLS:
Carry a medical alert card and bracelet at all times.
Stay in contact with doctor.
Keep extra medication handy
15. 15 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Addisonian crisis
An addisonian crisis is a life-threatening situation that results in
HYPOTENSION, HYPOGLYCEMIA and HYPERKALEMIA.
This situation requires immediate medical care.
Treatment typically includes intravenous injections of:
1. Hydrocortisone
2. Saline solution
3. Sugar (dextrose)
ADRENAL CRISIS IS :
Acute episodes from stress that taxes
the adrenal cortical function beyond its
capabilities.
Examples of acute insufficiency are:
а) State after removal of adrenals
b) Hemorrhage in adrenals which arises
during sepsis, meningococci infection
(syndrome Waterhouse-Friderixan);
chronic anticoagulant therapy, DIC.
c) Rapid withdrawal of steroids (from
previous treatment).
• Fast falling of the adrenals
function causes development of
collapse and the patients can die
during the first day.
16. 16 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Occur in :
1.Following stress , infection, surgery ,trauma.
2. Prolonged fasting in patient with latent insufficiency.
3. Sudden with drawl of adrenocortical hormone in pt with insufficiency
4. Following bilateral adrenlectomy or removal of functioning adrenal tumor
that has suppressed other adrenal.
5. Following sudden destruction of pituitary gland( pituitary necrosis) OR
when the thyroxine is given to a patient with hypoadrenalism.
6. Following injury to both adrenals by:
TRAUMA ANTICOAGULANT THERAPY
INFECTION HEMORRHAGE
THROMBOSIS 2RY CARCINOMA
ABDOMINAL DISCOMFORT
EMOTIONAL UPSET HIGH TEMPERATURE
C/F:
CNS-headache , confusion ,lassitude , coma.
GIT- nausea , vomiting , diarrhea , abdominal pain
Fever with fatigubilty.
SIGNS:
FLUID LOSS/DEHYDRATION
HYPOTENSION
CYANOSIS
17. 17 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
ANY PATIENT WITH GIT SYMPTOMS AND SHOCK IN STEROID WITHDRAWL.
Skin hyperpigmentation , meningococcimeia with purpura
LAB:
Hyponatremia ,Hyperkalemia, Hypoglycemia ,Hypercalcemia, High eosinophil.
Hyperuricemia , lymphocytosis, Hyperuremia
IF YOU FIND HYPERPIGMENTATION LOOK FOR OTHER (( H))= CRISIS
Adrenal hormone assay : hydroxycorticoid &17ketsteroid in 24HR urine
detected
Cosyntropin stimulation test :
Cosyntropin(ACTH) 0.25mg I/V
S.cortisol 30 , 60 min
Normally raises to 20 micgm/dl
Stope hydrocortisone before 8hr of the test
Plasma ACTH is markedly elevated in 1ry adrenal disease ((200pg/dl))
GOALS OF CARE:
TO REVERSE SHOCK
REPLENISH NEEDED STEROID
TREATMENT:
Dxtose 5% , Normal saline
ADRENAL CORTICAL HORMONE REPLACEMENT: INJECTABLE
HIGH SALT DIET
ANTIBIOTICS.
18. 18 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
CUSHING’S SYNDROME
CAUSE:
Sustained over production of glucocorticoids by adrenal gland from
ACTH by pituitary tumor.
20. 20 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Truncal obesity
Buffalo hump
Moon- face
Weight gain
Sodium retention with hypertension
Thinning of extremities – from loss of muscle tissue due to protein
catabolism
Purple striae – from thinning of skin
Echymosis from slight trauma
poor wound healing
hypertension, hyperglycaemia
ANDROGENIC EFFECTS:
Oligomenorrhea
Hirsutism
Gynaecomastia
Decreased libido
TREATMENT & NURSING CARE:
PSYCHOLOGICAL SUPPORT
PREVENT INFECTION – INFLAM & IMMUNE RESPONSE ARE SUPPRESSED
PROMOTE SAFETY
SURGERY – SUB/TOTAL ADRENALECTOM
22. 22 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Aldosterone is a hormone that controls sodium and potassium levels in the
blood. Hyperaldosteronism leads to retention of salt and loss of potassium,
which then leads to hypertension (high blood pressure).
PRIMARY – CONN’S SYNDROME(73%)unilateral adenoma
-B/L CORTICAL HYPERPLASIA
A.D .,defect allowing ACTH stimulation of Aldosterone production.
SECONDARY
Who is affected by Hyperaldosteronism?
People in their 30’s-50’s (adulthood), with prevalence increasing with age
Females are more likely to be affected than males
African Americans have a significantly greater risk of fatality from the
disease than other races.
CONN’S SYNDROME
PRIMARY ALDOSTERONISM
CAUSE:
ADRENAL ADENOMA
S/SX:
HYPOKALEMIA
FATIGUE
HYPERNATREMIA, HPN, TETANY
MANAGEMENT:
SURGERY
ALDACTONE – ALDOSTERONE ANTAGONIST
23. 23 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Conn’s disease
Usually caused by a benign tumor of the adrenal gland or bilateral adrenal
hyperplasia (enlargement of an organ caused by an increase in the
reproduction rate of its cells) .
Features:
- Hypertension (0.7%HTN PTS) - headache
Malignant HTN RARE, typically moderate, diastolic only w out S&S.
- Hypokalemia metabolic alkalosis - tateny ¶sthesia
- Fatigue - polyuria and polydipsia
- Nocturia escape phenomenon prevents edema
- Muscle weakness
-Paralysis simulating periodic paralysis.
Secondary Hyperaldosteronism is when the excess aldosterone is caused
by something outside the adrenal gland and mimics the primary
condition.
The problem is outside the adrenal gland:
e.g. RENIN – ANGIOTENSIN SYSTEM
Causes of Secondary Hyperaldosteronism:
Increased renin production, commonly caused by the ingestion of oral
contraceptives and toxemia from pregnancy.
Renin - An enzyme secreted by and stored in the kidney area that
stimulates aldosterone and therefore, raises blood pressure
24. 24 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Conditions like congestive heart failure, liver failure, kidney disease,
cirrhosis, and dehydration
Certain medicines like diuretics and fludrocortisone.
SYMTOMS:
Symptoms are similar to those of Primary Hyperaldosteronism
Moderate hypertension (high blood pressure)
Most individuals have no other specific symptoms. However, some may
have:
◦ Muscle weakness ,Fatigue
◦ Temporary paralysis ,Cramping
◦ Headaches
◦ Low potassium level
◦ Tingling
◦ Muscle spasms
25. 25 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
DIAGNOSIS:
High NA intake and low k
Elevated aldosterone levels can be measured in the blood or urine.
24 HR urine collection is assayed for Aldosterone ,Cortisol , Creatinine.
Plasma assayed for Aldosterone at 8 AM while PT is supine after
overnight recumbency and again after 4 HR upright.
Adrenal Adenoma ---more than 20mg/dl not raise
Adrenal hyperplasia----less than 20mg/dl raise upright
Low plasma rennin less than 5mg/dl with 24 hr urine Aldosterone more
than 20mg/dl indicates HYPERALDOSTERONISM.
Urine Ald. less 20mg /24hr seen in gonadal/adrenal enzyme defect.
Once hyperaldosterone is diagnosed, plasma assayed for 18hydroxy-
corticosterone—above 85mg/dl—adrenal neoplasm.
◦ In a blood test, PRA (Plasma renin activity), is used to distinguish
between primary (low PRA) and secondary Hyperaldosteronism
(high PRA).
Abdominal CT scans can show adrenal masses
Electrocardiograms (ECGs) can show abnormalities in heart rhythm that
are often associated with low potassium level.
It is likely that many cases of secondary Hyperaldosteronism are never
detected.
Treatments:
Secondary Hyperaldosteronism is treated by treating the underlying
cause.
Typically, medication and diet (but not surgery) are used
Spironolactone is the treatment of choice.
26. 26 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
When untreated, the disease can lead to uncontrolled hypertension
(which can become a risk factor for stroke and heart disease).
The outlook for the patient depends on the cause of the condition
Adrenocortical Neoplasms
• May be responsible for a variety of hyperadrenalisms
a) functional adenomas most commonly associated with
hyperaldosteronism and Cushing syndrome
• A virilizing neoplasm high incidence to be carcinogenic
• Functional and non-functional adrenocortical neoplasms cannot be
differentiated solely on basis of morphology
a) Hormone measurements
b) Most adrenocortical adenomas do not cause hyperfunction
• Adrenocortical carcinomas are rare
a) two rare inherited adrenal cortical carcinomas
i) Li-Fraumeni syndrome
- autosomal dominant
- predisposition to develop other cancers due to mutations in p53
ii) Beckwith-Wiedemann syndrome
• Generally, adrenal adenomas are small (1-2cm) whereas adrenal
carcinomas are large, invasive lesions
a) adrenal carcinomas metastasize via lymphatics and inferior vena cava
i) mean survival is ~2 yrs.
27. 27 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Adrenal Medulla
• Most important diseases of adrenal medulla are the neoplasms
a) neuronal
i) Neuroblastoma
ii) Mature ganglionic cell tumors
b) Chromaffin cells
i) pheochromocytoma
• Pheochromocytoma
a) neoplasm composed of chromaffin cells
i) synthesize and release catecholamine’s.
b) familial syndromes (~10%)
• i) MEN2A and MEN2B
ii) type I neurofibromatosis
iii) Von Hippel-Lindau disease
iv) Sturge-Weber syndrome
c) Extra-adrenal source (~10%)
i) carotid body
ii) organ of Zuckerkandl
• d) are bilateral (~10%)
i) may be as high as 50% in familial cases
e) malignant (~10%)
i) more common when arise in extra-adrenal sites
HORMONES: EPINEPHRINE
NOREPINEPHRINE
PHEOCHROMOCYTOMA:
This is a rare tumor of chromaffin tissue which secrets
catecholamine’s & is responsible for less than 0.1% of causes of
H.T.
28. 28 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
The tumors are usually benign /10% malignant //in over 90% of cases
the tumor found in the adrenal medulla, while it may arise elsewhere
in the body in sympathetic ganglia (paragangliomas).
There is a useful rule of tens’ in this condition:
10% are malignant , 10% in children
10% are extraadrenal , 10%extreedrenal are extraabdominal
10% are familial , 10% bilateral adrenal tumors
10% are non hypertensive
It is associated with MEN type II.
• Occasionally, this tumor produces other biogenic steroids or peptides
associated with Cushing’s Syndrome.
• Extra-medulla tumors – 1-3% in chest and neck (usually malignant)
• 20% multiple, 10% malignant
SYMPTOMS AND SIGNS:
Hypertension, may be paroxysmal or persistent. Due to secretion of one
or more of catecholamine hormones or precursors: norepinephrine,
epinephrine, dopamine or dopa.
Occasionally the pt. may be hypotensive ( dopamine – secreting tumors).
some pt. may present with a complication of the hypertension e.g.:
stroke, MI, LVF
There may be abdominal pain with vomiting, constipation, wt. loss,
glucose intolerance.
90% Presented with Palpitation, Hypertension,Sweating &Anxiety.
29. 29 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
During the attack the pt. presents with: pallor (some time flushing),
palpitation, sweating, headache, anxiety (feeling of death).
Tachycardia, sweating, postural hypotension, tachypnea, flushing, cold
and clammy skin, severe headache, angina, palpitation, dyspnoea
Paroxysmal attacks may be provoked by exercise, anaesthesia, palpation
of tumor, postural changes, urination, beta-blockers
Hyperglycemia
Cardiac arrhythmia and congestive heart failure.
DIAGNOSTIC TEST :
Urine Sample
Blood Work
Abdominal CT Scan
MRI of the Abdominal Region
Adrenal Gland Biopsy
Screening
- Two 24 h urines for catecholamines is the best screening investigation
- 24 h urine for VMA (15% false negative) and metanephrine (10% false
negative) - needs vanilla-free diet before collection
If the diagnosis is established, or strongly suspected
MIBG scan - meta-iodo-benzylguanidine labeled with 131
I
- Increased uptake by pheochromocytoma
30. 30 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
CT scan of adrenals - patient should be alpha- and beta-blocked to avoid
hypertensive episode after contrast administration
End products of catecholamine metabolism.
DRUGS & FOOD TO BE WITHHELD 24H B4 THE TEST:
COFFEE & TEA
BANANA
VANILLA
CHOCOLATES
MANAGEMENT:
SURGERY
MEDICAL : ADRENERGIC BLOCKING AGENTS: PHENTOLAMINE
NURSING CARE:
MONITOR BP IN SUPINE & STANDING
MONITOR URINE FOR GLUC & ACETONE
31. 31 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
This requires excision of the tumor or long term TX with α and
(usually β) adrenoceptor blockade.
Prior to surgery give α –antagonist drug such as phenoxybenzamine 10-20
mg orally 3-4 times / day for a minimum of 6 weeks to allow restoration
of normal plasma volume if tachycardia is there then β –
antagonist as propranolol (10- 20 mg ) 3 times daily should be added or
combined α and β-antagonist(e.g. labetalol)can be added.
β-antagonist should not be given before the α-antagonist as it may cause
a paradoxical rise in blood pressure due to unopposed α-mediated
vasoconstriction.
During surgery Na-nitropruside & the short acting α-antagonist
phentolamine are very useful in controlling hypertension episodes which
may occur during anesthesia or mobilization of the tumor.post-operative
hypotension may occur and require volume expansion and very
occasionally noradrenalin infusion.
After surgery, it is necessary to continually monitor all vital signs in an
intensive care unit. When the tumor cannot be surgically removed,
medication is needed to manage it. This usually requires a combination of
medications to control the effects of the excessive hormones. Radiation
therapy and chemotherapy have not been effective in curing this kind of
tumor.
a. Pre-op: Sympathetic blocking agents= Minipress (prazosin), Hytrin (terazosin),
Cardura (doxazosin) to reduce BP and other symptoms of of catecholamine excess
Since change in BP sudden, client may experience orthostatic hypotension
Use Beta blocking agents such as Inderal to dec. heart rate, BP and force of
contraction and calcium channel blocking agents also used.
b. General management
Diet: high in vitamin, mineral, calorie, no caffeine
Sedatives; Monitor BP
Eliminate attacks; If attack- complete bedrest and HOB 45 degrees
c. Surgery via laparoscopic adrenalectomy or open abdominal incision; complete
removal of the tumor cures hypertension in 10-30% of the cases
34. 34 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Multiple Endrocrine Neoplasia Syndromes (MEN)
• Group of inherited diseases
a) hyperplasias, adenomas and carcinomas of multiple endocrine
organs
i) Occur at younger age vs. cancers
ii) Arise in multiple endocrine organs
iii) Even in one organ, they are multifocal
iv) Tumors preceded by hyperplasia
v) More aggressive and recur more frequently vs. sporadic
tumors
• MEN-1(( WEMERS SYNDROME))
a) Inherited as autosomal dominant
b) Is a tumor suppressor gene
i) Loss of MEN-1 therefore causes tumor genesis
- Parathyroid (95% involved)
- Pancreas (>40%)
- Pituitary (>30%)
I- Parathyroid gland
A) Primary hyperparathyroidism arising from hyperplasia is a common
feature MEN-1 or adenoma of several parathyroid gland.
B) Occurs in 90% of cases.
C) Presents with hypercalcemia.
II-Pancreas-pancreatic islet cell tumors
a) Leading cause of death in MEN-1
b) Aggressive
c) Often functional tumors
i) Gastrinomas (Zollenger-Ellison syndrome)-MOST COMMON
ii) Insulinomas and resultant hypoglycemia
35. 35 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
III- Pituitary adenoma
A) most common in MEN-1 is prolactinoma
B)May secret prolactin ,Growth hormone , ACTH.
C)May cause local pressure effects and hypopituitrism.
D)Usually non functional.
IV- Adrenal cortical adenoma
A)Occur in 70%
B)Can be adenoma or hyperplasia
C)Bilateral in about half
D) They are generally benign and nonfunctional
• MEN-2b (William syndrome)
a) Involve also the thyroid
b)Adrenal (medulla)
c) Major differences between MEN-2a and MEN-2b is
i) Do not develop primary hyperparathyroidism (MEN-2b)
ii) Develop at extra endocrine sites
- lips
- tongue
- GI tract
• MEN-2 carry RET Protooncogene
a) persons are advised to have prophylactic thyroidectomy to
prevent carcinoma.
•
Adrenogenital syndrome results from the hereditary stipulated blockade
of cortisole synthesis and amplified formation of androgens from general
intermediate products.
36. 36 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
• Depending on the level of blockade of cortisole synthesis there are three
variants of adrogenital syndrome.
• І. Disorders of early stages of synthesis – deficiency of glucocorticoides,
mineralcorticoides and androgens hyperproduction. Manifestations: signs
of insufficiency of gluco- and mineralocorticoidal functions of adrenal
cortex features of early sexual maturing in males, virilization in women
(appearance of man's sexual features).
• ІІ. Disorders of intermediate stages – deficiency of glucocorticoides,
surplus of androgens, formation of mineralocorticoides is not infringed
(classical androgenic syndrome). Manifestations are the same, as in the
first case, only without signs of insufficiency of mineralocorticoidal
function.
• ІІІ. Disorders at final stages of cortirol synthesis – deficiency of
glucocorticoides, hyperproduction of androgens and
mineralocorticoide. Features of hyperaldosteronism are connected with
manifestations of classical androgenital syndrome.
Adrenocortical neoplasms
Adrenocortical neoplasms associated with symptoms of excess of
androgen are more likely to be androgen secreting adrenal carcinomas
than adenomas.
It is also often assoc with hypercortisolism (mixed syndrome)
The tumour secretes androgen thus increasing in circulation and
converted to testosterone at the peripheral tissues.
May occur at any age , relatively rare.
should be suspected when the onset of androgenic symptoms is sudden
(i.e., generally <2 yr) and the pace of symptoms is rapid, and when they
lead to virilization and masculinization.
37. 37 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
may be associated with other systemic symptoms including weight loss,
anorexia, a feeling of abdominal bloating, back pain.
CAH Depends on the nature and severity of the enzymytic defect.
Onset of clinical symptoms can occur in the
Perinatal period
Later childhood
Adulthood (less common)
Anterior pituitary
ACT
H
Cholesterol
Pregnolone
17- hydroxypregnenolone
17- hydroxyprogesterone
21
11– deoxycortisol
Cortisol
Glucocorticoids
Progesterone
21
Aldosterone
Corticosterone
11- deoxycortisone
Mineralocorticoids
Testosterone
Androstenedione
Dehydroxypiandrosterone
Sex steroids
Adrenal cortex (bilateral hyperplasia)
Congenital
adrenal hyperplasia
Autosomal recessive deficiency of an enzyme in the cortisol synthetic
pathways.
38. 38 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
◦ Cortisol secretion is reduced and feedback leads to increased ACTH
secretion to maintain adequate cortisol leading to adrenal
hyperplasia.
◦ Diversion of the steroid precursors into the androgenic steroid
pathways occurs. Thus, 17-hydroxyprogesterone, androstenedione
and testosterone levels are increased, leading to virilization.
Virilism==(excessive androgens may cause masculinization).
Male excessive estrogen cause feminisation—breast enlargement.
Androgens have important functions in women
◦ Essential in the production of E2 (in ovary & adipose tissue)
◦ Responsible for delivery. & maint. of axillary & pubic hair
◦ Important for libido.
Virilization:
The development of exaggerated
masculine characteristics, usually
in women, often as a result of
overproduction of androgens
So, if hyperandrogenism becomes
extreme, virilization occurs
Symptoms of virilization include
1. Excess facial and body hair
(hirsutism),
2. Baldness
3. Acne
4. Deepening of the voice
5. Increased muscularity
39. 39 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
6. An increased sex drive.
In women,
1. The uterus shrinks
2. The clitoris enlarges (clitoromegaly)
3. The breasts become smaller
4. normal menstruation stops (amenorrhea)
Clinical Manifestation:
• Cortisol deficiency – hypoglycemia, inability to withstand stress,
vasomotor collapse, hyperpigmentation, apneic spells, muscle weakness
& fatigue.
• Aldosterone deficiency – hyponatremia, hyperkalemia, vomiting, urinary
sodium wasting, salt craving, acidosis, failure to thrive, volume depletion,
hypotension, dehydration, shock, diarrhea.
• Androgen excess – ambiguous genitalia, virilization of external genitalia ,
hirsutism, early appearance of pubic hair, penile enlargement , excessive
height gain and skeletal advance.
*Late onset CAH – normal genitalia, have acne, hirsutism, irregular
menses/amenorrhea.
ANDROGEN
Testosterone, dehydroepiandrosterone sulfate (DHEAS),
dehydroepiandrosterone (DHEA), androstenedione, and androstenediol
The ovaries produce 50% of circulating testosterone, 50% of the
androstenedione and 20% of DHEA.
The adrenal glands produce all the DHEAS and 80% of the DHEA. The
adrenals also secrete 50% of androstenedione and 25% of testosterone.
40. 40 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
Adrenal androgens increase in response to ACTH stimulation
LH stimulates theca cells of the ovaries to secrete androgens
Hyperandrogenism
Excess of androgens may be caused by:
◦ primary gonadal disorders
◦ primary adrenal disorders
◦ iatrogenic
In practice though, the causes are restricted to a few conditions:
41. 41 MAGDI AWAD SASI 2013 ADRENAL GLAND DISORDERS
◦ PCOS
◦ Cushing’s syndrome
◦ CAH
◦ Tumours
Lab
Testosterone and Dehydroepiandrosterone sulphate (DHEAS)
◦ DHEAS hyperandrogenemia of adrenal origin
Serum prolactin
thyroid stimulating hormone (TSH)
Serum 17 hydroxyprogesterone (17-OHP) test –if suspect CAH
LH and FSH ( suggestive of PCOS if ratio >2)
Lipid profile
OGTT
◦ Relying on a fasting glucose level alone is inadequate as it is a poor
predictor of impaired glucose tolerance or diabetes
Effect of androgens
Fat deposition (small breast)
◦ Androgens inhibit the ability of some fat cells to store lipids
Muscle mass (heavy mascular mass)
◦ Androgens promote the enlargement of skeletal muscle cells
Brain
◦ Enhanced libido.