Primary hyperaldosteronism resident survival guide: Difference between revisions

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{{familytree | | | | | | | | | | | | F01 | | F02 | | | F03 |F01=Conn's syndrome (Primary aldosteronism)|F02=Profound K+ depletion|F03=• 17 alpha hydroxylase deficiency<br>• 11 beta hydroxylase deficiency<br>• Liddle's syndrome<br>• Licorice ingestion<br> •Deoxycortisone producing tumor|}}
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{{familytree | | | | | |I01| | | | | | | | | | | | | | |I02|I01=• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)<br>• Licorice ingestion<br>•Glucocorticoid resistance|I02=Liddle's syndrome)|}}

Revision as of 17:08, 17 July 2017

Template:Primary hyperladosteronism Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Causes

Common Causes

Common causes of Conn's Syndrome may be divided into:

  • Adrenal causes:
    • Aldosterone-secreting adrenal adenoma (APA-benign tumor, 50-60%)
    • Idiopathic hyperaldosteronism (IHA-Bilateral hyperplasia of the adrenal gland, 40-50%)
  • Extra-adrenal causes
    • Ectopic secretion of aldosterone (Ovaries and Kidneys)

Less Common Causes

  • Familial hyperaldosteronism type I (glucocorticoid-remediable aldosteronism [GRA])
  • Familial hyperaldosteronism II (the familial occurrence of APA or bilateral idiopathic hyperplasia or both)
  • Familial hyperaldosteronism type III (associated with the germline mutation in the KCNJ5 potassium channel)
  • Pure aldosterone-producing adrenocortical carcinomas
  • Unilateral adrenal hyperplasia

Diagnosis and Treatment

 
 
 
 
 
 
 
 
Preferred screening population:

• Blood pressure > 160 / 100 particularly (< 50 years)
• Resistant hypertension or refractory hypertension (use of > 3 anti-hypertensives and poor control of blood pressure)
• Hypokalemia (provoked by diuretic therapy or unprovoked)
• Hypertension and incidentally discovered adrenal adenoma
• Hypertension with a family history of early-onset hypertension (< 20 years) or cerebrovascular accident at age less than 40 years

• Hypertensive first-degree relatives of patients with PA
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Plasma Renin Activity/Aldosterone Ratio
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Normal or High Renin (Plasma Renin/Aldosterone ratio <10
 
 
 
 
 
 
 
 
 
 
 
Suppressed Renin (Plasma Renin/Aldosterone ratio >20
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
•Renin-secreting tumors
•Diuretic use
•Renovascular hypertension
•Coarctation of aorta
•Malignant phase hypertension
 
 
 
 
 
 
 
 
 
 
 
Urinary aldosterone
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Elevated
 
Normal
 
 
Low
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Conn's syndrome (Primary aldosteronism)
 
Profound K+ depletion
 
 
• 17 alpha hydroxylase deficiency
• 11 beta hydroxylase deficiency
• Liddle's syndrome
• Licorice ingestion
•Deoxycortisone producing tumor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Add Mineralocrticoid antagonist for 8 weeks
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
No BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)
• Licorice ingestion
•Glucocorticoid resistance
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Liddle's syndrome)