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DISORDERS OF THE ADRENOCORTICAL HORMONES. Dr. Ayisha Qureshi MBBS, Mphil. Hypoadrenalism- ADDISON’S DISEASE. ADDISON’S DISEASE. DEFINITION:

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disorders of the adrenocortical hormones

DISORDERS OF THE ADRENOCORTICAL HORMONES

Dr. Ayisha Qureshi

MBBS, Mphil

addison s disease
ADDISON’S DISEASE

DEFINITION:

Addison’s disease results from the inability of the adrenal cortices to produce the adrenal hormones & this is most frequently caused by the primary atrophy of adrenal cortex.

CAUSES:

  • Autoimmune disorder
  • Tuberculous destruction of the glands
  • Cancer
  • Secondary to impaired pituitary gland function leading to insufficient ACTH production.
signs symptoms
Signs & Symptoms

Mineralocorticoid deficiency

Glucocorticoid deficiency

Patient cannot maintain normal blood glucose levels as no gluconeogenesis b/w meals

Nausea, vomiting, fever, hypotension

All metabolisms effected

Patient highly susceptible to the deteriorating effects of stress.

Even mild infections can lead to death

  • Greatly decreased renal sodium reabsorption
  • Increased loss of Na, Cl & water in the urine
  • Decreased ECF/ hypovolemia
  • Hyponatremia
  • Hyperkalemia
  • Mild Acidosis
  • Increased RBC conc. Due to decreased ECF
  • Decreased CO
  • Shock
  • Death
signs symptoms1
Signs & Symptoms
  • Extreme melanin pigmentation of the mucous membranes & skin.
  • Usually deposited in blotches
  • Cause is increased ACTH secretion as well as increased MSH secretion

WHAT IS POMC & MSH?

Proopiomelanocortin (POMC) is a preprohormone which when cleaved causes the formation of:

  • MSH (melanocyte stimulating hormone) which causes darkening of the skin by stimulating formation of melanin & dispersing it to the epidermis
  • Beta Lipoprotein
  • Beta endorphin & few others

(ACTH also has 1/30 as much activity of MSH & so its hypersecretion also causes Hyperpigmentation of the skin.)

treatment
TREATMENT
  • A person with complete destruction of the adrenal may die within a few days to 2 weeks b/c of weakness & circulatory shock.
  • However, if small quantities of mineralo & glucocorticoids are administered daily, they can live for years.
slide8

DEFINITION:

Hypersecretion by the adrenal cortex causes a complex cascade of hormone effects called Cushing’s syndrome.

CAUSES:

  • Exogenous

- Factitious

- Iatrogenic

  • ACTH-Dependant

- Pituitary adenoma

- Ectopic ACTH syndrome

  • ACTH-Independant

- Adrenal adenoma

- Adrenal carcinoma

slide9

When Cushing’s syndrome is secondary to excess secretion of ACTH by the anterior pituitary, it is called CUSHING’S DISEASE.

signs symptoms2
Signs & Symptoms
  • Truncal Obesity
  • Supraclavicular & dorsal fat pad
  • Buffalo hump
  • Moon Facies
  • Purple striae due to decreased collagen proteins
  • Thin skin
  • Capillary fragility & easy bruising
  • Hirsuitism
  • Diabetes Mellitus also called Adrenal Diabetes
  • Thin extremities and severe muscle weakness due to increased protein catabolism
  • Infections due to suppressed immune system with impaired wound healing
  • Osteoporosis
  • Hypertension
diagnosis treatment
Diagnosis & Treatment

DIAGNOSIS:

  • Very challenging
  • Diagnose excess Cortisol (24 hour urine cortisol & midnight salivary cortisol level)
  • Dexamethasone suppression test

TREATMENT:

  • Removal of the cause: Surgery of the adrenal tumour OR pituitary tumour
  • Drugs that block steroidogenesis.e.g. Ketaconazole
  • Drugs that inhibit ACTH secretion.e.g. Serotonin antagonists
  • Partial or total adrenalectomy
slide16

DEFINITION:

Occasionally, a small tumor of the zonaglomerulosa cells occurs & secretes large amounts of aldosterone; the resulting condition is called “Primary aldosteronism” or “ Conn’s disease”.

CAUSES:

  • Small tumour of ZonaGlomerulosa cells that secretes large amounts of Aldosterone.
  • Sometimes even hyperplastic adrenal cortices secrete aldosterone instead of cortisol.
signs symptoms3
Signs & Symptoms
  • Severe hypokalemia causing muscle paralysis
  • Increased blood and ECF volume
  • Slight increase in Na conc.
  • Hypertension

Usually, diagnosed by decreased plasma renin and increased aldosterone concentration.

slide19

A syndrome that is caused by occasional adrenocortical tumour that secretes excessive quantities of androgens.

signs symptoms4
Signs & Symptoms
  • In Females:
  • Virile characteristics
  • Growth of a beard
  • Deeper voice
  • Occasionally baldness
  • Masculine distribution of hair on the body
  • Deposition of proteins in a masculine manner
slide21

In Males:

  • In prepubertal male, a virilizing adrenal tumour causes same characteristics as in the female plus rapid development of the male sex organs
  • In adult male, the virilising characteristics are often masked by the virilising effects of Testosterone
  • Diagnosis is made by the presence of excess 17-ketosteroids in the urine.