Primary hyperaldosteronism resident survival guide
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) • 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 (Plasma Renin/Aldosterone ratio <10 | Suppressed (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) | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||