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DISORDERS
LEARNING OBJECTIVES
Define the different disorders of adrenal
gland.
Discuss these disorders with its etiology,
features.
Enlist the diagnostic techniques for these
disorders.
Make a therapeutic plan.
Adrenal cortex
ADRENAL DYSFUNCTION
Decrease function
Adrenal insufficiency
Eg Addison disease
Increase function
Cushing syndrome
Hyperaldosteronism
Pheochromocytoma
CUSHINGS SYNDROME
CLINICAL PRESENTATION
Central obesity
Face , trunk , neck, abdomen
involvement
Moon face with plethora
Peripheral obesity and fat
accumulation
Buffalo hump
Myopathies or muscular
weakness
Striae along lower abdomen, red
to purple colour
Hypertension
Exophthalmus
CHF
Osteopenia & osteoporosis
Glucose intolerance
Psychiatric changes
Gonadal dysfunction with
amenorrhea
Malaise
Hirsuties
Striae, acne, skinthinning, bruising
Polyuria, nocturia
decreased libido and
impotence in males
Cushing's syndrome
Occurrence
%
Sign
or
symptom
Occurrence
%
Central obesity
94
Easy bruisability
60
Hypertension
82
Osteoporosis
60
Glucose intolerance
80
Personality changes
55
Hirsutism
75
Acne
50
Amenorrhea or impotency
75
Edema
50
Purple striae
65
Headache
40
Plethoric faces
60
40
DIAGNOSIS
Hypercortisolism
24 hour urinary free cortisol test UFC
Night time salivary cortisol test (>0.25 g/dl )
Low dose dexamethasone suppression test.
Plasma ACTH , CRH test.
CT scans
MRI
THERAPEUTIC PLAN
Depends on etiology.
Symptoms resolve over several weeks to months with
rational therapy.
Iatrogenic dose tapering.
Transphenoidal adenectomy.
ACTH production decreases temporarily after surgery.
Glucocorticoid replacement therapy for 3 12 months.
Pituitary irradiation.
Ectopic ACTH secreting tumors , adrenal adenomas
surgery / radiation / chemotherapy
Cushing's syndrome
SURGERY
RADIATION
CHEMOTHERAPY
CORTISOL-INHIBITING DRUGS
PHARMACOTHERAPY
Steroidogenic inhibitors
Neuromodulators of ACTH secretion
Glucocorticoid receptor antagonist.
STEROIDOGENIC
INHIBITORS
Ketoconazole
Metyrapone
Aminoglutethimide
Inhibits conversion of cholesterol to pregnelone.
Short term effects due to compensatory rise in ACTH.
Effects are long lasting when given in combination.
ADRENOLYTIC AGENTS
Mitotane
Inhibits 11 hydroxylation in adrenal cortex.
Reduced synthesis of cortisol.
Cell degeneration of all zones except glomerulosa in acute
therapy .
NEUROMODULATORS
Not very efficacious when used alone.
Combination therapy is sometimes effective.
Cyproheptadine
Lower ACTH secretion
For non surgical patients.
Relapse can occur.
Bromocriptine
Valproic acid
Octreotide
Roseglitazone
GLUCOCORTICOID
RECEPTOR BLOCKER
Mifepristone
Progesterone , androgen glucocorticoid receptor blocker
Inhibits Dexamethsone suppression
Increases endogenous cortisol & ACTH
Pre treatment
Post treatment
ADRENAL
INSUFFICIENCY
ADDISONS DISEASE
Adrenal insufficiency
Autoimmune mediated destruction of
adrenal cortex.
Primary and secondary adrenal
insufficiency.
PRIMARY
destruction of adrenal
cortex
- autoimmune disorders
- chronic infection
CORTISOL
ALDOSTERONE
SECONDARY
Lack of ACTH
- drugs
- tumors and
infections of
pituitary gland
CORTISOL
CLINICAL FEATURES
Weight loss
Muscle weakness
Fatigue
Hyperpigmentation of skin
Alopecia . vitiligo
hypotension
Hyponatremia
Salt craving
Hypoglycemia
Elevated creatinine
Elevated ESR
hyperkalemia
DIAGNOSIS
Basal serum cortisol < 3 g/dl
Cosyntropin stimulation test (>19 g/dl ruled out)
Metyrapone test (plasma cortisol <8 g/dl & plasma 11
deoxycortisol <7 g/dl )
Insulin induced Hypoglycemia test
2 day ACTH Stimulation test (<18 g/dl serum cortisol)
Plasma aldosterone
Adrenal CT
MRI
PHARMACOTHERAPY
Dexamethasone - 0.5 mg od
Hydrocortisone 15 mg
Cortisone 20 mg
Prednisone 2.5 - 5 mg
Follow up in 6 8 weeks
Fludricortisone acetate 0.05 mg po od
For hyperkalemia deoxycorticosterone trimethylacetate
in oil
Stress related dose adjustment 5 10 mg hydrocort.
Alternative therapy licorice may be harmful
ADDISONIAN CRISIS
ACUTE ADRENAL INSUFFICIENCY
Hypotension
Loss of consciousness
ADDISONIAN CRISIS
Establish IV access.
Serum electrolytes, glucose, cortisol, ACTH
Rapidly infuse 2 3 l N/S or 5% dextrose.
Inject hydrocortisone 100 mg IV Q6h.
Taper the dose over next 2 3 days if patient stable.
Start fludricortisone 0.1 mg daily
Pregnancy continue the usual glucocorticoid and mineralocorticoids ,
some women need high doses in third trimester.
Unable to take orally dexamethasone IM
daily
1 2 mg deoxycorticosterone
25 mg IV hydrocort Q6h
Adequate saline hydration
During labor increase the dose
REFERENCES
Dipiro clinical practice
Herfindal clinical pharmacy and
therapeutic.
Roger walker
Davidson clinical medicine