Adrenal Disorders
Prepared by
Dr Hussein Noori Ali
Lecturer of Internal Medicine
College of Medicine
University of Garmian
Nov 2025
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ADRENAL ANATOMY AND DEVELOPMENT
The normal adrenal glands weigh 6–11 g each. They are located above
the kidneys and have their own blood supply.
Arterial blood flows initially to the subcapsular region and then
meanders from the outer cortical zona glomerulosa through the
intermediate zona fasciculata to the inner zona reticularis and
eventually to the adrenal medulla.
The right suprarenal vein drains directly into the vena cava, while the
left suprarenal vein drains into the left renal vein.
During early embryonic development, the adrenals originate from
the urogenital ridge and then separate from gonads and kidneys at
about the sixth week of gestation. Concordant with the time of sexual
differentiation (seventh to ninth week of gestation) adrenal cortex,
starts to produce cortisol and the adrenal sex steroid precursor DHEA.
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Cortex: The adrenal cortex produces glucocorticoids (primarily
cortisol), mineralocorticoids (primarily aldosterone), and androgens
(primarily dehydro-epiandrosterone and androstenedione).
Glucocorticoids promote and inhibit gene transcription in many
cells and organ systems.
Prominent effects include anti-inflammatory actions and increased
hepatic gluconeogenesis.
Mineralocorticoids regulate electrolyte transport across epithelial
surfaces, particularly renal conservation of Na in exchange for K.
Adrenal androgens' chief physiologic activity occurs after
conversion to testosterone and dehydro-testosterone.
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Medulla
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.
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Addison's Disease
(Primary or Chronic Adrenocortical Insufficiency)
Addison's disease is an insidious, usually progressive hypofunctioning
of the adrenal cortex.
It causes various symptoms, including hypotension and
hyperpigmentation, and can lead to adrenal crisis with cardiovascular
collapse.
Addison's disease develops in about 4/100,000 annually. It occurs in all
age groups, about equally in each sex, and tends to become clinically
apparent during metabolic stress or trauma.
Onset of severe symptoms (adrenal crisis) may be precipitated by
acute infection (a common cause, especially with septicemia). Other
causes include trauma, surgery, and Na loss from excessive sweating.
With treatment, Addison's disease should not typically reduce life
expectancy.
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Etiology
About 70% of cases in the US are due to idiopathic atrophy of the
adrenal cortex, probably caused by autoimmune processes.
The remainder result from destruction of the adrenal gland by
granuloma (eg, TB), tumor, amyloidosis, hemorrhage, or inflammatory
necrosis.
Hypoadrenocorticism can also result from administration of drugs that
block corticosteroid synthesis (eg, ketoconazole, the anesthetic
etomidate).
Addison's disease may coexist with diabetes mellitus or hypothyroidism
in autoimmune polyglandular failure (schmidt syndrome)
In children, the most common cause of primary adrenal insufficiency is
congenital adrenal hyperplasia
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Pathophysiology
Both mineralocorticoids and glucocorticoids are deficient.
Mineralocorticoid deficiency:
Because mineralocorticoids stimulate Na reabsorption and K
excretion, deficiency results in increased excretion of Na and
decreased excretion of K, chiefly in urine but also in sweat,
saliva, and the GI tract.
A low serum concentration of Na and a high concentration of
K result.
Inability to concentrate the urine, combined with changes in
electrolyte balance, cause severe dehydration, plasma
hypertonicity, acidosis, decreased circulatory volume,
hypotension, and, eventually, circulatory collapse.
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Glucocorticoid deficiency:
Glucocorticoid deficiency contributes to hypotension and causes severe
insulin sensitivity and disturbances in carbohydrate, fat, and protein
metabolism.
In the absence of cortisol, insufficient carbohydrate is formed from
protein; hypoglycemia and diminished liver glycogen result. Weakness
follows, due in part to deficient neuromuscular function.
Resistance to infection, trauma, and other stress is diminished.
Myocardial weakness and dehydration, reduce cardiac output, and
circulatory failure can occur.
Decreased blood cortisol results in increased pituitary ACTH production
and increased blood β-lipotropin, which has melanocyte-stimulating
activity and, together with ACTH, causes the hyperpigmentation of skin
and mucous membranes characteristic of Addison's disease.
Thus, adrenal insufficiency secondary to pituitary failure does not cause
hyperpigmentation.
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Symptoms and Signs
Weakness, fatigue, and orthostatic hypotension are early symptoms
and signs.
Hyperpigmentation is characterized by diffuse tanning of exposed and,
to a lesser extent, unexposed portions of the body,
Black freckles are common on the forehead, face, neck, and shoulders.
Areas of vitiligo develop, as do bluish black discolorations of the
areolae and mucous membranes of the lips, mouth, rectum, and
vagina.
Anorexia, nausea, vomiting, and diarrhea often occur.
Decreased tolerance to cold, with hypometabolism, may be noted.
Dizziness and syncope may occur.
Weight loss, dehydration, and hypotension are characteristic of the
later stages of Addison's disease.
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Diagnosis
The best screening test is the cortisol response 60 min after
250 μg ACTH (cosyntropin) IV or IM.
Cortisol levels should exceed 18 μg/dL 30–60 min after the
ACTH.
If the response is abnormal, then primary and secondary
deficiency may be distinguished by measurement of
aldosterone from the same blood samples.
In secondary adrenal insufficiency, the aldosterone increment
from baseline will be normal (≥5 ng/dL).
Furthermore, in primary adrenal insufficiency, plasma ACTH is
elevated, whereas in secondary adrenal insufficiency, plasma
ACTH values are low or inappropriately normal.
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TREATMENT
Hydrocortisone, at 15–25 mg/d divided into 2/3 in the
morning and 1/3 in the afternoon, is the mainstay of
glucocorticoid replacement.
other glucocorticoids may be given at equivalent doses.
Mineralocorticoid supplementation is usually needed for
primary adrenal insufficiency, with administration of 0.05–0.1
mg fludrocortisone PO qd and maintenance of adequate Na
intake.
Doses should be titrated to normalize Na and K levels and to
maintain normal blood pressure without postural changes.
Mineralocorticoid replacement is not needed in pts with
secondary adrenal insufficiency.
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Adrenal crisis
Adrenal crisis is characterized by profound asthenia;
severe pain in the abdomen, lower back, or legs;
peripheral vascular collapse; and, finally, renal shutdown with
azotemia.
Body temperature may be low,
although severe fever often occurs, particularly when crisis is
precipitated by acute infection.
A significant number of patients with partial loss of adrenal function
(limited adrenocortical reserve) appear well, but experience adrenal
crisis when under physiologic stress (eg, surgery, infection, burns,
critical illness). Shock and fever may be the only signs.
During adrenal crisis, high-dose hydrocortisone (10 mg/h continuous IV or
100-mg bolus IV three times a day) should be administered along with
normal saline.
Thereafter, if the pt is improving and is afebrile, the dose can be tapered by
20–30% daily to usual replacement doses.
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Secondary adrenal insufficiency
Secondary adrenal insufficiency is adrenal hypofunction due to a lack
of ACTH.
Symptoms are the same as for Addison's disease, but there is usually
less hypovolemia.
-Secondary adrenal insufficiency may occur in panhypopituitarism,
- in isolated failure of ACTH production,
- in patients receiving corticosteroids,
-or after corticosteroids are stopped.
Inadequate ACTH can also result from failure of the hypothalamus to
stimulate pituitary ACTH production, which is sometimes called tertiary
adrenal insufficiency.
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Patients receiving corticosteroids for > 4 wk may have insufficient
ACTH secretion during metabolic stress to stimulate the adrenals to
produce adequate quantities of corticosteroids, or they may have
atrophic adrenals that are unresponsive to ACTH.
These problems may persist for up to 1 year after corticosteroid
treatment is stopped
Symptoms and signs are similar to those of Addison's disease.
Differentiating clinical or general laboratory features include the
absence of hyperpigmentation and relatively normal electrolyte
and BUN levels; hyponatremia, if it occurs, is usually dilutional.
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Diagnosis
• Serum cortisol • Serum ACTH , • ACTH stimulation testing
CNS imaging
Patients with confirmed secondary adrenal insufficiency should have CT
or MRI of the brain to rule out pituitary tumor or atrophy.
Adequacy of the hypothalamic-pituitary-adrenal axis during tapering or
after stopping long-term corticosteroid treatment can be determined
by injecting cosyntropin 250 μg IV or IM. After 30 min, serum cortisol
should be > 20 μg/dL (> 552 nmol/L).
An insulin stress test to induce hypoglycemia and a rise in cortisol is
the gold standard for testing integrity of the
hypothalamic-pituitary-adrenal axis.
Treatment
• Hydrocortisone or prednisone
• Fludrocortisone not indicated
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Cushing's syndrome
Cushing's syndrome is a constellation of clinical abnormalities caused
by chronic high blood levels of cortisol or related corticosteroids.
Cushing's disease is Cushing's syndrome that results from excess
pituitary production of ACTH, usually secondary to a pituitary
adenoma.
Epidemiology Cushing’s syndrome is generally considered a rare
disease. It occurs with an incidence of 1–2 per 100,000 population per
year. However, it is debated whether mild cortisol excess may be more
prevalent among patients with features of Cushing’s such as centripetal
obesity, type 2 diabetes, and osteoporotic vertebral fractures,
recognizing that these are relatively nonspecific and common in the
population.
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Etiology
The most common cause of Cushing’s syndrome is iatrogenic, due to
administration of glucocorticoids for therapeutic reasons.
Endogenous Cushing’s syndrome results from production of excess
cortisol (and other steroid hormones) by the adrenal cortex.
The major cause is bilateral adrenal hyperplasia secondary to
hypersecretion of adrenocorticotropic hormone (ACTH) by the
pituitary (Cushing’s disease 60-70%) or from
ectopic sources(10-15%) such as small cell carcinoma of the lung;
carcinoids of the bronchus, thymus, gut and ovary, medullary
carcinoma of the thyroid; or pheochromocytoma.
Adenomas or carcinomas of the adrenal gland account for about
15–20% of endogenous Cushing’s syndrome cases.
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Clinical Features
Some common manifestations (central obesity,
hypertension, osteoporosis, psychological disturbances,
acne, hirsutism, amenorrhea, and diabetes mellitus) are
relatively nonspecific.
More specific findings include easy bruising, purple
striae, proximal myopathy, fat deposition in the face and
nuchal areas (moon faces and buffalo hump), and rarely
androgenization.
Thin, fragile skin, and plethoric moon faces also may be
found.
Hypokalemia and metabolic alkalosis are prominent,
particularly with ectopic production of ACTH.
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Diagnosis
• Dexamethasone suppression test
• Urinary free cortisol (UFC) level
• ACTH levels; if detectable, provocative testing
In some centers, testing begins with measurement of UFC, the best assay for urinary
excretion (normal, 20 to 100 μg/24 h [55.2 to 276 nmol/24 h]).
UFC is elevated > 120 μg/24 h (> 331 nmol/24 h) in almost all patients with Cushing's
syndrome.
However, many patients with UFC elevations between 100 and 150 μg/24 h (276 and
414 nmol/24 h) have obesity, depression, or polycystic ovaries but not Cushing's
syndrome.
For initial screening, measurement of 24-h urinary free cortisol,
the 1mg overnight dexamethasone test (8:00 a.m. plasma cortisol <1.8 μg/dL [50
nmol/L]),
or late night salivary cortisol measurement is appropriate
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Definitive diagnosis is established in equivocal cases by
inadequate suppression of urinary cortisol (<10 μg/d [25
nmol/d]) or plasma cortisol (<5 μg/dL [140 nmol/L]) after 0.5
mg dexamethasone every 6 h for 48 h.
Once the diagnosis of Cushing’s syndrome is established,
further biochemical testing is required to localize the source.
Low levels of plasma ACTH levels suggest an adrenal
adenoma, bilateral nodular hyperplasia, or carcinoma;
inappropriately normal or high plasma ACTH levels suggest a
pituitary or ectopic source.
In 95% of ACTH-producing pituitary microadenomas, cortisol
production is suppressed by high-dose dexamethasone (2 mg
every 6 h for 48 h).
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MRI of the pituitary should be obtained but may not
reveal a microadenoma because these tumors are
typically very small.
Imaging of the chest and abdomen is required to localize
the source of ectopic ACTH production.
Pts with chronic alcoholism, depression, or obesity may
have false-positive results in testing for Cushing’s
syndrome—a condition named pseudo-Cushing’s
syndrome.
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Treatment
Transsphenoidal surgery for pituitary ACTH-secreting
microadenomas is curative in 70–80% when performed
by a highly experienced surgeon, but long-term follow-up
is required because these tumors may recur.
• Radiation therapy may be used when a surgical cure is
not achieved.
• Therapy of adrenal adenoma or carcinoma requires
surgical excision; stress doses of glucocorticoids must
be given pre- and postoperatively.
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Metastatic and unresectable adrenal carcinomas are treated with
mitotane in doses gradually increased to 6 g/d in three or four
divided doses.
On occasion, debulking of lung carcinoma or resection of carcinoid
tumors can result in remission of ectopic Cushing’s syndrome.
If the source of ACTH cannot be resected, medical management
with ketoconazole (600–1200 mg/d), metyrapone (2–3 g/d), or
mitotane (500–1000 mg/d) may relieve manifestations of cortisol
excess.
In some cases, bilateral total adrenalectomy is required to control
hypercorticism.
• Pts with unresectable pituitary adenomas who have had bilateral
adrenalectomy are at risk for Nelson’s syndrome (aggressive
pituitary adenoma enlargement).
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Nelson’s syndrome
Nelson’s syndrome is increased pigmentation (because of
high levels of ACTH) associated with an enlarging pituitary
tumour, which occurs in about 20% of cases after bilateral
adrenalectomy for Cushing’s disease.
Currently this syndrome is rare , because adrenalectomy is an
uncommon primary treatment, and its incidence may be reduced by
pituitary radiotherapy soon after adrenalectomy.
The Nelson’s adenoma may be treated by pituitary surgery and/or
radiotherapy (unless given previously).
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INCIDENTAL ADRENAL MASSES
Adrenal masses are common findings on abdominal CT or MRI (1–7%
prevalence with increasing age).
The majority of such “incidentalomas” are clinically nonfunctional, and
the probability of an adrenal carcinoma is low (<5%).
The first step in evaluation is to determine the functional status by
measurement of plasma free metanephrine to screen for
pheochromocytoma.
In a pt with a known extraadrenal malignancy, there is a 30–50%
chance that the incidentaloma is a metastasis.
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-Additional hormonal evaluation should include 24-h urine
for urinary free cortisol or overnight 1-mg dexamethasone
suppression testing,
-aldosterone/renin ratio in hypertensive patients,
- DHEAS in women with signs of androgen excess, and
estradiol in males with feminization.
-Fine-needle aspiration is rarely indicated and absolutely
contraindicated if a pheochromocytoma is suspected.
Adrenocortical cancer is suggested by large size (>4–6 cm),
irregular margins, tumor inhomogeneity, soft tissue
calcifications, and high unenhanced CT attenuation values
(>10 HU).
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Hyperaldosteronism
Hyperaldosteronism is a syndrome associated with hypersecretion of the major adrenal
mineralocorticoid, aldosterone.
The normal function of aldosterone is to reabsorb sodium and excrete potassium and acid
(H+).
Hyperaldosteronism can be divided into the following:
The most common cause of primary hyperaldosteronism is a unilateral adrenal adenoma
(Conn’s syndrome) (70%).
Bilateral hyperplasia accounts for 25–30%.
Excessive black licorice ingestion can mimic this effect. Licorice has aldosterone-like
qualities.
Secondary aldosteronism, in which the stimulus is extraadrenal
Secondary aldosteronism is caused by reduced renal blood flow, which stimulates the
renin-angiotensin mechanism with resultant hypersecretion of aldosterone.
Causes of reduced renal blood flow include obstructive renal artery disease (eg, atheroma,
stenosis), renal vasoconstriction (as occurs in accelerated hypertension), and edematous
disorders (eg, heart failure, cirrhosis with ascites, nephrotic syndrome).
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Clinical Presentation
Primary hyperaldosteronism is characterized by hypertension and low
potassium levels.
Most of the other symptoms, such as muscle weakness, polyuria, and
polydipsia, are from the hypokalemia.
Metabolic alkalosis occurs because aldosterone increases hydrogen ion
(H+) excretion.
Aldosterone causes alkalosis.
Edema is uncommon with primary hyperaldosteronism because of
sodium release into the urine.
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Diagnosis
Diagnosis is suspected in patients with hypertension and hypokalemia.
Initial laboratory testing consists of plasma aldosterone levels and PRA.
Ideally, tests are done after the patient has not taken any drugs
that affect the renin-angiotensin system (eg, thiazide diuretics, ACE inhibitors,
angiotensin antagonists, β- blockers) for 4 to 6 wk.
PRA is usually measured in the morning with the patient recumbent.
Patients with primary aldosteronism typically have plasma aldosterone > 15 ng/dL (>
0.42 nmol/L) and low levels of PRA, with a ratio of plasma aldosterone (in ng/dL) to
PRA (in ng/mL/h) > 20.
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Low levels of both PRA and aldosterone suggest nonaldosterone
mineralocorticoid excess (eg, due to licorice ingestion, Cushing's
syndrome, or Liddle syndrome).
High levels of both PRA and aldosterone suggest secondary
hyperaldosteronism (Renal artery stenosis , OCP consumption)
Patients with findings suggesting primary hyperaldosteronism
should undergo CT or MRI to determine whether the cause is a
tumor or hyperplasia.
Aldosterone levels measured on awakening and 2 to 4 h later
while standing also may help make this distinction;
in adenoma, levels decline and in hyperplasia, levels increase.
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Treatment
• Surgical removal of tumors
• Spironolactone or eplerenone for hyperplasia
complete remission occurs in 50 to 70%.
(With adrenal hyperplasia, 70% remain hypertensive after
bilateral adrenalectomy; thus, surgery is not recommended).
Hyperaldosteronism in these patients can usually be controlled
by spironolactone, starting with 300 mg po once/day and
decreasing over 1 month to a maintenance dose, usually around
100 mg once/day; or by amiloride 5 to 10 mg po once/day or
another K-sparing diuretic.
The newer more specific drug eplerenone may be used because,
unlike spironolactone, it does not block the androgen receptor.
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Syndromes of adrenal androgen excess
Syndromes of adrenal androgen excess result from excess
production of dehydroepiandrosterone (DHEA) and
androstene-dione, which are converted to testosterone in
extraglandular tissues.
The elevated testosterone accounts for most androgenic
effects.
Clinical Signs and Symptoms. Hirsutism, oligomenorrhea,
acne, and virilization.
Etiology includes congenital adrenal hyperplasia(CAH) ,
adrenal adenomas (rare), and adrenal carcinomas.
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Congenital adrenal hyperplasia
Congenital adrenal hyperplasia (CAH) (most common adrenal disorder of infancy and
childhood) is associated with increased adrenal androgen production due to
enzymatic defects.
CAH arises from autosomal recessive mutations, which produce deficiencies of
enzymes necessary for the synthesis of cortisol.
C-21 hydroxylase deficiency
occurs in 95% of all cases of CAH.
(two-third classic form and one-third Late form)
In this disorder, due to the lack of 21-hydroxylation, hydroxyprogesterone and
progesterone cannot be converted into 11-doxycortisol and 11-doxycortisone , and as
a result, there is a failure in the production of cortisol and aldosterone. Cortisol
deficiency leads to increased ACTH production
It increases 17-hydroxyprogesterone and progesterone.
Also, the increase in ACTH production leads to an increase in the production of
androstenadione and DHEA, which can be converted to testosterone.
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Classic form of C-21 hydroxylase deficiency
(Absolute or severe deficiency)
Adrenal virilization occurs with or without an associated salt-losing
tendency, owing to aldosterone deficiency, which leads to
hyponatremia, hyperkalemia, dehydration, and hypotension.
Patients are female at birth with ambiguous external genitalia
(female(46XX) pseudohermaphroditism), enlarged clitoris, and partial
or complete fusion of the labia. Postnatally CAH is associated with
virilization.
Patients may be male(46XY) at birth with macrogenitosomia;
postnatally this is associated with precocious puberty.
About 35% of those patients also see a reduction in aldosterone
secretion.
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diagnosis
In case of suspicion of 21alpha-hydroxylase deficiency, the most
useful diagnostic test is to check 170HP (17-hydroxyl progesterone)
that if it was more than 200ng/dL the diagnosis is definite.
If the diagnosis is not definite, it can be tested by using stimulation
with ACTH administration, which caused an increase of -17 OHP.
Imaging; abdominal CT Scan that reveals the adrenal cortex
hyperplasia
Classical treatment
In the classic form, three treatment measures should be performed
1- treat cortisol deficiency by replacing cortisol.
2- alpha fludrocortisone.
3- Treatment of increased sex hormones with flutamide or
Piglutamide (anti-androgen) or testolactone (aromatase inhibitor)
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Late form of C-21 hydroxylase deficiency
(mild or relative deficiency)
In this type, 21-hydroxylase deficiency is mild.
The most common AR disease in humans and is very common in Ashkenazi Jew
because enzyme deficiency is relative So Salt Wasting does not occur and puberty
appear with Virilization and menstrual disorder .
Aldosterone and Cortisol is normal and testosterone is a little high so severe Sexual
changes are not seen in this type of disease.
Note: If these patients are boys, they have no symptoms, but if they are girls , they
come with hirsutism, acne and amenorrhea during puberty , Because the level of
DHEA and androstenedione is high.
Also, hyperkalemia and metabolic acidosis are not seen.
Investigations; high17-hydroxy progesterone
To confirm it ,We use ACTH stimulation test . If 30 minutes after
administration of ACTH (0.25mg) ,17-OHP>1500ng the diagnosis is
confirmed.
Treatment in LATE form:
The conventional treatment is to use dexamethasone (0.5 mg per day) to suppress
ACTH, but since they only have symptoms of virilism, the use of antiandrogens such as
spironolactone (100-200 mg per day) or flutamide (125 mg) per day is more effective .
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Deficiency of 11beta hydroxylase
The cause of 5% of CAH cases is deficiency of 11 beta hydroxylase enzyme.
In this syndrome, the conversion of 11-deoxycortisol to cortisol and
11-deoxycorticosterone to corticosterone (precursor of Ald) is inhibited, so in
this disorder there is not production of aldosterone and cortisol, and instead
sex hormones increase and due to the increase of 11 Deoxycorticosterone
(DOC-11) patient will find symptoms of hyperaldosteronism, so in these
patients we will have hypertension, hypokalemia and metabolic alkalosis.
Symptoms appear in the form of pigmentation, weakness, fatigue and
lethargy and high blood pressure.
There are symptoms of virilization such as 21-alpha hydroxylase deficiency.
Diagnosis is based on recording the increase of -11-deoxycortisol ( basic form
or after ACTH injection)
Treatment;
Dexamethasone or prednisolone( In 11beta hydroxylase deficiency we treat
the hypertention with corticosteroid therapy)
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Diagnosis
CAH should be considered in all infants exhibiting failure
to thrive, especially those with episodes of acute adrenal
insufficiency, salt wasting, or hypertension.
The most useful measurements are of serum
testosterone, androstenedione,
dehydroepiandrosterone,
17-hydroxyprogesterone,
Management. Glucocorticoid (hydrocortisone)
replacement
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Phaeochromocytoma and paraganglioma
Its rare neuro endocrine tumor of sympathetic system.
it may secrete adrenalin/noradrenalin.
80% in medulla and 20% elsewhere
90% benign and 10% malignant
It may be part of other syndromes like VHL or neurofibromas or
MENII-A (MTC, PHEO, parethyroid Hyperplasia or Adenoma)
or MENII-B(MTC, PHEO, multiple neuromas, distinctive facial features,
marfanoid habitus)
In adults, approximately 80% of pheochromocytomas are unilateral and
solitary, 10% are bilateral, 10% are extraadrenal and about 10% are
malignant ,10% in children
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Investigations
• Plasma free metanephrine or urinary metanephrine
• Chest and abdomen imaging (CT or MRI) if catecholamine screen positive
• Possibly nuclear imaging with 123 I-meta-iodobenzylguanidine (MIBG)
Plasma free metanephrine is up to 99% sensitive
Urinary metanephrine is less specific than plasma free metanephrine, but
sensitivity is about95%.
The principal urinary metabolic products of epinephrine and norepinephrine
are the metanephrines {vanillylmandelic acid (VMA) and homovanillic acid
(HVA)}
Serum chromogranin A is often elevated and may be a useful tumour marker
in patients with non-secretory tumours and/or metastatic disease
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Treatments
In functioning tumours, medical therapy is required to prepare the patient for
surgery, preferably for a minimum of 6 weeks to allow restoration of normal
plasma volume.
The most useful drug in the face of very high circulating catecholamines is the
α-blocker phenoxybenzamine (10–20 mg orally 3–4 times daily) because it is
a non-competitive antagonist, unlike prazosin or doxazosin
If α-blockade produces a marked tachycardia, then a β-blocker such as
propranolol can be added.
B-blocker should not be given before alpha blockers due to paradoxical rise of
blood pressure.
During surgery, sodium nitroprusside and the short-acting α-antagonist
phentolamine are useful in controlling hypertensive episodes, which may
result from anaesthetic induction or tumour mobilisation.
Post-operative hypotension may occur and require volume expansion and,
very occasionally, noradrenaline (norepinephrine) infusion, but is uncommon
if the patient has been prepared with phenoxybenzamine.
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