Primary hyperaldosteronism resident survival guide
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Primary hyperaldosteronism Microchapters |
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Differentiating Primary Hyperaldosteronism from other Diseases |
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Diagnosis |
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Treatment |
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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
The Management of Primary hyperaldosteronism including case detection, diagnosis, and treatment according to the "Endocrine Society Clinical Practice Guidlines" is as follows:[1][2][3]
- 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
- Confirmatory tests:
- Fludrocortisone supression test (Positive test= Aldosterone > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour)- Gold standard
- Intravenous saline load testing (Positive test= Aldosterone more than 10 ng / dl)
- Oral sodium loading test (Positive test= 24-hour urinary aldosterone excretion > 12 μg / day)
- Captopril challenge test (Positive test= PAC / PRA > 30, measured two hours after the administration of 25 mg or 50 mg of captopril)
| Preferred screening population | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 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 | 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 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Normal, micornodularity, bilateral masses or unilateral atypical mass | Unilateral hypodense nodule >1cm and <2cm in the setting of marked primary hyperaldosteronism | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Surgery not desired | Surgery desired | Surgery desired | Surgery not desired | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| > 35 years consider | < 35 years consider | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| Glucocorticoid-remediable aldosteronism (GRA) OR Idiopathic hyperaldosteronism (bilateral hyperplasia): • Preferred regimen-Spironolactone 12.5mg to 25mg QD or Eplerenone 25mg BID • Alternative regimen-Amiloride 5mg BID plus chlorthalidone/hydrochlorothiazide 12.5mg to 25mg QD | No Lateralization with Adrenal Venous Sampling | Lateralization with Adrenal Venous Sampling | Aldosterone Producing Adenoma (APA) OR Primary Adrenal Hyperplasia (PAH): Unilateral laproscopic adrenalectomy | • Preferred regimen-Spironolactone 12.5mg to 25mg QD or Eplerenone 25mg BID • Alternative regimen-Amiloride 5mg BID plus chlorthalidone/hydrochlorothiazide 12.5mg to 25mg QD | |||||||||||||||||||||||||||||||||||||||||||||||||||||||
References
- ↑ "The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline: The Journal of Clinical Endocrinology & Metabolism: Vol 101, No 5".
- ↑ Romero DG, Yanes Cardozo LL (2016). "Clinical Practice Guideline for Management of Primary Aldosteronism: What is New in the 2016 Update?". Int J Endocrinol Metab Disord. 2 (3). PMC 5175479. PMID 28018978.
- ↑ Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, Stowasser M, Young WF (2016). "The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline". J. Clin. Endocrinol. Metab. 101 (5): 1889–916. doi:10.1210/jc.2015-4061. PMID 26934393.