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Adrenal

disorders
Cortex Hipersekresi

Medulla
Hiposekresi
Disorders of adrenocortical
function
Adrenocortical hyperfunction
Glucocorticoids  Cushing’s syndrome
Aldosteronism  Conn’s disease

Adrenocortical hypofunction
Hypopituitarism  Glucocorticoids Addison’s
Hypoaldosteronism Mineralocorticoids disease
Hypopituitarism  Androgens
ADRENAL
HYPERFUNCTION

Cushing’s
Syndrome
CUSHING’S SYNDROME
Chronic glucocorticoid excess  symptoms &
physical features  Cushing’s Syndrome

Iatrogenic CS  Chronic glucocorticoid therapy :


most commonly

Spontaneous CS :
 Pituitary (Cushing disease)
 Adrenal adenoma
 ACTH secrt non pituitary tumor (ectopic ACTH Syndrome)
CUSHING’S SYNDROME
Classification

ACTH-dependent
Pituitary adenoma (Cushing disease) 70%
Nonpituitary neoplasma (ectopic ACTH)

ACTH-independent
Iatrogenic (glucocorticoid, megestrol acetat)
Adrenal neoplasma (adenoma, carcinoma)
Hyperplasia adrenal
Factitious
Indicative of
Cushing
Syndrome
Cushing disease

Adrenal tumor

Tumor producing
ACTH
CUSHING’SSYNDROME
CUSHING’S SYNDROME
Treatment :
Cushing’s disease
Microsurgery, Radiation therapy
Adrenal tumors
Unilateral adrenalectomy
Ectopic ACTH syndrome
Benign  surgical treatment
Malignant  Ketokonazole, metyrapone
Iatrogenic (Cushingoid)
Tapering off
Alternate day regimen
PSEUDO CUSHING’S SYNDROME

Obesity
Chronic alcoholism
Depression
Hirsutism Post adrenalectomy
Adrenal Hyperfunction

Hyperaldosteronism
ACTH

Aldosterone

K Angiotensin II

Angiotensin I
EBV
Renin

Renin substrate

Major factors regulating aldosterone secretion; EBF(Effective Blood Volume)


Primary Hyperaldosteronism
• Accounts for about 0.7% of cases of hypertension
• Insidens : Women >>
• Etiology :
• Unilateral adrenocortical adenoma (Conn’s
syndrome - 73%)
• Bilateral (27%)
• Hyperaldosteronism :
– HYPERTENSION
– HYPOKALEMIA
– ALKALOSIS
Primary Hyperaldosteronism
Clinical finding : Hypertension, muscular
weakness, paresthesias, headache,
polydipsia, polyuria, moderate hypertension
(malignant is rare)

Laboratory finding : Serum potassium low,


24 hours urine collection aldosterone

Imaging : CT-scan
Primary Hyperaldosteronism

Treatment :
Laparoscopic adrenalectomy
Pharmaco : spironolactone and antihyper
tensive agent
Complication : Renal damage
Prognosis :
Improved by early diagnosis and treatment,
only 2% malignant
Secondary Hyperaldosteronism
• Defiition : an appropriately increased production of
aldosterone in response to activation of the renin-
angiotensin system

• production rate of aldosterone is often higher in patients


with secondary than in those with primary aldosteronism

• characterized by
- hypokalemic alkalosis,
- moderate to severe increases in plasma renin
- moderate to marked increases in aldosterone levels
Adrenal Insuffiency

Addison’s Disease
Disorders of adrenocortical insufficiency

Deficient adrenal production of glucocorticoid


and mineralocorticoid  Adrenocortical
insufficiency

@ Primary adrenocortical insufficiency


(Addison’s disease)
@ Secondary - deficient pituitary ACTH secretion,
glucocorticoid therapy (most common)
Addison’s Disease
• Etiologi:
Tbc (prior 1920)
Autoimmune adrenalitis  adrenal atrophy (80%)
Associated other immunologic and autoimmune endocrine disorders
AIDS
malignant disease
• Incidens :
Rare, female >>, 30-50 years
• Clinical features:
weakness, fatigue, anorexia, weight loss, hyperpegmentasi,
hypotension
Addison’s Disease
Addison’s Disease
• Laboratorium :
Hiponatrimia- hiperkalemia (classic)
Radiologis /CT Scan
• Diagnosis
Basal adrenokortical steroid Normal
Rapid ACTH stimulation test
ACTH plasma
• Treatment:
Replacement therapy cortisol
Diseases of adrenal medulla

Pheochromocytoma
Pheochromocytoma
o Rare (< 0,2% of hypertensive)
o Cathecolamine-producing tumor of neuro
chromaffin cells.
o Incidence 3-4th decades
o autosomal dominat hereditary
o malignant 10-15% cases
o Hypertension is caused by excessive plasma level
epinephrine by tumor located unilateral or both
adrenals & anywhere along sympathetic nervus
chain ( 90% adrenal)
Pheochromocytoma
• Symptoms and Signs
Usually lethal unless diagnosed and treated
severe headache, perspiration, palpitation,
anxiety, tremor, tachycardia
Attack  cyanosis, facial pallor
Classical symptomatic triad : headache,
sweating, palpitations
• Laboratory finding
Urinary cathecolamines, metanephrine,
creatinine, urinary VMA
Common Symptoms in Patients with Hypertension Due
to Pheochromocytoma.
Symptoms during or following paroxysms
Headache
Sweating
Forceful heartbeat with or without tachycardia
Anxiety or fear of impending death
Tremor
Fatigue or exhaustion
Nausea and vomiting
Abdominal or chest pain
Visual disturbances
Dyspnea
Symptoms between paroxysms
Increased sweating
Cold hands and feet
Weight loss
Constipation
Pheochromocytoma
Localization
• CT scanning
– Overall accuracy 90%-95% for adrenal tumours
– Less accurate for extra adrenal tumours
• Isotope scintigraphy (MIBG scanning)
– 131I-MIBG stored in chromaffin granule
– Sensitivity 99%
– False negative 11%
– False positive 2%
Blood and Urine analysis
– Plasma catecholamine levels > 1000
micrograms
– Urinary VMA and Metanephrine levels
Pheochromocytoma
Treatment
• Surgery
• Preoperative preparation
To control hypertension & prevent CVS comp/
Alpha adrenergic blockade
– Phenoxybenzamine 10 mg qds 1-2 weeks before
surgery
– Beta blockade propanolol 10 mg qds 2-3 days
• Intraoperatively
Phentolamine
Sodium nitroprusside
Pheochromocytoma
• Treatment
Laparoscopic removal of the tumor 
treatment of choice,
open laparatomy
• Prognosis
Depends early diagnosis is made
ADRENAL
INCIDENTALOMA
ADRENAL INCIDENTALOMAS

• Masses found incidentally during radiographic


imaging of the abdomen
• Incidence : 0.35-4.36% in general population

In evaluation such mass:


• Is the mass benign or malignant ?
• Does the mass secrete hormones or mass
disfunction
ADRENAL INCIDENTALOMAS
Etiology
• Benign:
Non hormone secreting
lipoma, cyst, ganglioneuroma, adenoma
Hormone secreting
pheochromocytoma, aldosteronism, subclinical Cushing’s syndrome
• Malignant :
Adrenocortical carcinoma
Metastatic neoplasm
Lymphoma
ADRENAL INCIDENTALOMAS
• Size is important:
Adrenal masses > 4cm  more likely malignant
 surgical resection should be consideration

• The great majority (+ 89%) are benign, non


functioning masses

• A full biochemical workup should be completed


before surgery is done

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