Primary hyperaldosteronism resident survival guide: Difference between revisions

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{{familytree | | | | | |I01| | | | | | | | | |I02| | | |I03|I01=Subtype classification|I02=• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)<br>• Licorice ingestion<br>•Glucocorticoid resistance|I03=Liddle's syndrome)|}}
{{familytree | | | | | |I01| | | | | | | | | |I02| | | |I03|I01=Subtype classification|I02=• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)<br>• Licorice ingestion<br>•Glucocorticoid resistance|I03=Liddle's syndrome)|}}
{{familytree | | | | | |J01| | | | | | | | | | | | | | | | | |J01=Adrenal CT scan}}

Revision as of 18:43, 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
•Deoxycorticosterone producing tumor
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Do confirmatory tests:
•Fludrocortisone supression test (Positive test= Aldosterone > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour)
OR
•Intravenous saline load testing (Positive test= Aldosterone more than 10 ng / dl)
OR
•Oral sodium loading test (Positive test= 24-hour urinary aldosterone excretion > 12 μg / day
OR
•Captopril challenge test (Positive test= PAC / PRA > 30, measured two hours after the administration of 25 mg or 50 mg of captopril)
 
 
 
 
 
 
 
 
 
 
 
 
Add Mineralocrticoid antagonist for 8 weeks
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
BP response
 
 
 
No BP response
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Subtype classification
 
 
 
 
 
 
 
 
 
• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)
• Licorice ingestion
•Glucocorticoid resistance
 
 
 
Liddle's syndrome)
 
 
 
 
 
Adrenal CT scan