Primary hyperaldosteronism

Primary hyperaldosteronism

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  Primary Aldosteronism Abdulmoein EidAl-Agha, FRCPCH(UK) Professor of Pediatric Endocrinology, Head, Pediatric Endocrinology section, King Abdulaziz University Hospital, Pediatric Department, Jeddah, Saudi Arabia E-mail: [email protected] Website: http://aagha.kau.edu.sa
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  Primary Hyperaldestronism By theend of this section of the lecture, the following points will be identified: • Knowledge on important causes of primary hyperaldestronism. • Describe signs & symptoms of primary hyperaldestronism. • Knowledge on how to investigate clinical case of hyperaldestronism.
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  Cortex Medul a > • 'lo •
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  Aldosterone • Is asteroid hormone produced exclusively in the zona glomerulosa of the adrenal cortex. • Is a mineralocorticoid hormone. • The principal site of action of aldosterone is the distal nephron. • The principal regulators of aldosterone synthesis & secretion are the renin-angiotensin system. • In distal tubules, increases reabsorption of sodium & chloride and excretion of potassium & hydrogen ions. • Hyperaldestronism is characterized by excessive secretion of aldosterone, which causes increases in sodium & chloride reabsorption and loss of potassium and hydrogen ions.
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  ACTH Cortisol Cortisol
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  • Aldosterone isessential in the homeostasis of circulating blood volume & serum potassium concentration. • Aldosterone secretion is stimulated by depletion in blood volume detected by stretch receptors & by an increase in serum potassium ion concentrations. • In contrast, it is suppressed by hypervolemia & hypokalemia.
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  Primary Hyperaldestronism Conn’s Syndrome
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  Conn’s Syndrome • Theterm primary hyperaldestronism (or primary aldosteronism) refers to a renin-independent increase in the secretion of aldosterone. • It was first described in 1955 by J. W. Conn in a patient who had anAldosterone-producing adenoma. • Is characterized by increased Aldosterone secretion from the adrenal glands. • This condition is principally a disease of adulthood, with its peak incidence in the fourth to sixth decades of life.
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  Cause of Hyperaldosteronism Aldosterone-producingadenoma : Conn’s disease Idiopathic bilateral adernal hyperplasia Adrenal carcinoma 65-70% 30% < 1%
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  Clinical Presentations • Primaryhyperaldestronism may be asymptomatic, particularly in its early stages. • When symptoms are present, they may be related to hypertension (if severe), hypokalemia, or both. • Hypertension results from hypervolemia secondary to sodium chloride retention. • The spectrum of hypertension-related symptoms includes the following: • Headache. • Facial flushing. • If hypertension is severe, weakness, visual impairment, impaired consciousness, and seizures (hypertensive encephalopathy). • Symptoms of hypokalemia include the following: • Constipation • Polyuria and polydipsia (because of impaired renal concentrating ability) • Weakness. • If the serum potassium is low enough, muscle weakness, transient paralysis and arrhythmia. • Paresthesia.
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  Workup for primaryaldosteronism • The presence of hypertension, hypokalemia, or both necessitate decision to screen. • Diagnostic investigations include: • Hypernatremia & hyperchloremia. • Hypokalemia with metabolic alkalosis. • High serum aldosterone, with low Plasma Renin Activity. • Aldosterone-to-renin ratio is sensitive means of differentiating primary from secondary causes of hyperaldestronism. • Most authors recommend ratio of 20-40 confirms diagnosis. • Computed Tomography of adrenal gland. • Adrenal Venous Sampling is the criterion standard test to differentiate unilateral from bilateral disease in patients with PA; however, it requires considerable skill.
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  Hyperaldestronism Treatment &Management • Surgical excision of the affected adrenal gland is recommended for all patients with hyperaldestronism who have a proven aldosterone-producing adenoma/ carcinoma. • Spironolactone is the most effective drug for controlling the effects of hyperaldestronism. • It is a nonselective, competitive mineralocorticoid receptor antagonist that is structurally similar to progesterone and metabolized in the liver to active metabolites.
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  unilateral Aldosterone- Producing Adenoma Tx: Unilateral adrenalectomy
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