Primary hyperaldosteronism resident survival guide: Difference between revisions
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*Unilateral adrenal hyperplasia | *Unilateral adrenal hyperplasia | ||
==Diagnosis and Treatment== | ==Diagnosis and Treatment== | ||
{{familytree/start}}{{familytree | | | | | | | | | A01 | | | | | |A01=Preferred screening population:<br> | {{familytree/start}}{{familytree | | | | | | | | | | | | A01 | | | | | |A01=Preferred screening population:<br> | ||
• Blood pressure > 160 / 100 particularly (< 50 years)<br> | • Blood pressure > 160 / 100 particularly (< 50 years)<br> | ||
• Resistant hypertension or refractory hypertension (use of > 3 anti-hypertensives and poor control of blood pressure)<br> | • Resistant hypertension or refractory hypertension (use of > 3 anti-hypertensives and poor control of blood pressure)<br> | ||
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• Hypertension with a family history of early-onset hypertension (< 20 years) or cerebrovascular accident at age less than 40 years<br> | • Hypertension with a family history of early-onset hypertension (< 20 years) or cerebrovascular accident at age less than 40 years<br> | ||
• Hypertensive first-degree relatives of patients with PA}} | • Hypertensive first-degree relatives of patients with PA}} | ||
{{familytree | | | | | | | | | |!| | | | | | | | }} | {{familytree | | | | | | | | | | | | |!| | | | | | | | }} | ||
{{familytree | | | | | | | | | B01 | | | | | |B01=Plasma Renin Activity/Aldosterone Ratio}} | {{familytree | | | | | | | | | | | | B01 | | | | | |B01=Plasma Renin Activity/Aldosterone Ratio}} | ||
{{familytree | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }} | {{familytree | | | | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }} | ||
{{familytree | | C01 | | | | | | | | | | | |C02|C01=Normal or High Renin (Plasma Renin/Aldosterone ratio <10|C02=Suppressed Renin (Plasma Renin/Aldosterone ratio >20}} | {{familytree | | | | | C01 | | | | | | | | | | | |C02|C01=Normal or High Renin (Plasma Renin/Aldosterone ratio <10|C02=Suppressed Renin (Plasma Renin/Aldosterone ratio >20}} | ||
{{familytree | | |!| | | | | | | | | | | | | |!| }} | {{familytree | | | | | |!| | | | | | | | | | | | | |!| }} | ||
{{familytree | | D01 | | | | | | | | | | | |D02|D01=•Renin-secreting tumors<br>•Diuretic use<br>•Renovascular hypertension<br>•Coarctation of aorta<br>•Malignant phase hypertension|D02=Urinary aldosterone}} | {{familytree | | | | | D01 | | | | | | | | | | | |D02|D01=•Renin-secreting tumors<br>•Diuretic use<br>•Renovascular hypertension<br>•Coarctation of aorta<br>•Malignant phase hypertension|D02=Urinary aldosterone}} | ||
{{familytree | | | | | | |,|-|-|-|-|-|-|-|-|-|+|-|-|-|-|.|}} | {{familytree | | | | | | | | | |,|-|-|-|-|-|-|-|-|-|+|-|-|-|-|.|}} | ||
{{familytree | | | | | |E01| | | | | | | |E02| | |E03|E01=Elevated|E02=Normal|E03=Low|}} | {{familytree | | | | | | | | |E01| | | | | | | |E02| | |E03|E01=Elevated|E02=Normal|E03=Low|}} | ||
{{familytree | | | | | | |!| | | | | | | | | |!| | | | |!| }} | {{familytree | | | | | | | | | |!| | | | | | | | | |!| | | | |!| }} | ||
{{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> •Deoxycorticosterone producing tumor|}} | {{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> •Deoxycorticosterone producing tumor|}} | ||
{{familytree | | | | | | |!| | | | | | | | | | | | | | |!| | | | }} | {{familytree | | | | | | | | | |!| | | | | | | | | | | | | | |!| | | | }} | ||
{{familytree | | | | | |G01| | | | | | | | | | | | |G02|G01=Do confirmatory tests:<br> •Fludrocortisone supression test (Positive test= Aldosterone > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour)<br>OR<br> •Intravenous saline load testing (Positive test= Aldosterone more than 10 ng / dl)<br>OR<br> •Oral sodium loading test (Positive test= 24-hour urinary aldosterone excretion > 12 μg / day<br>OR<br> •Captopril challenge test (Positive test= PAC / PRA > 30, measured two hours after the administration of 25 mg or 50 mg of captopril)|G02=Add Mineralocrticoid antagonist for 8 weeks}} | {{familytree | | |boxstyle=text-align: left; | | | | | | |G01| | | | | | | | | | | | |G02|G01=Do confirmatory tests:<br> •Fludrocortisone supression test (Positive test= Aldosterone > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour)<br>OR<br> •Intravenous saline load testing (Positive test= Aldosterone more than 10 ng / dl)<br>OR<br> •Oral sodium loading test (Positive test= 24-hour urinary aldosterone excretion > 12 μg / day<br>OR<br> •Captopril challenge test (Positive test= PAC / PRA > 30, measured two hours after the administration of 25 mg or 50 mg of captopril)|G02=Add Mineralocrticoid antagonist for 8 weeks}} | ||
{{familytree | | | | | | |!| | | | | | | | | | | |,|-|-|^|-|-|.}} | {{familytree | | | | | | | | | |!| | | | | | | | | | | |,|-|-|^|-|-|.}} | ||
{{familytree | | | | | | |!| | | | | | | | | | |H01| | | |H02|H01=BP response|H02=No BP response}} | {{familytree | | | | | | | | | |!| | | | | | | | | | |H01| | | |H02|H01=BP response|H02=No BP response}} | ||
{{familytree | | | | | | |!| | | | | | | | | | | |!| | | | | |!|}} | {{familytree | | | | | | | | | |!| | | | | | | | | | | |!| | | | | |!|}} | ||
{{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}} | {{familytree | | | | | | | | | |!| | | | | | | | | | | | | | | | | | |}} | ||
{{familytree | | | | | | | | |J01| | | | | | | | | | | | | | | | | |J01=Adrenal CT scan}} | |||
{{familytree | | | |,|-|-|-|-|-|^|-|-|-|.|}} | |||
{{familytree | | | |K01| | | | | |K02|K01= Normal, micornodularity, bilateral masses or unilateral atypical mass|K02= Unilateral hypodense nodule >1cm and <2cm in the setting of marked primary hyperaldosteronism|}} | |||
{{familytree | |,|-|^|-|.| | | | |,|-|^|-|-|-|.}} | |||
{{familytree|L01| |L02| | |L03| | | |L04|L01= Surgery not desired|L02=Surgery desired|L03= Surgery desired|L04=Surgery not desired|}} | |||
{{familytree | |!| | | |!| | |,|-|^|-|.| | | |!| | | |}} | |||
{{familytree | |!| | | |!| | |M01| |M02| |!| | | | |M01= > 35 years consider|M02= < 35 years consider|}} | |||
{{familytree | |!| | | |!| | |!| | | |!| | | |!| | | |}} | |||
{{familytree |N01| | |N02|N03| |N04| |N05| | | |N01=• Glucocorticoid-remediable aldosteronism (GRA)<br> •Idiopathic hyperaldosteronism|N02= Adrenal Venous Sampling|N03= Adrenal Venous Sampling|N04=Aldosterone Producing Adenoma (APA) or Primary Adrenal Hyperplasia (PAH):<br> Unilateral laproscopic adrenalectomy|N05= Pharmacological therapy|}} |
Revision as of 21:07, 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) | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
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) •Idiopathic hyperaldosteronism | Adrenal Venous Sampling | Adrenal Venous Sampling | Aldosterone Producing Adenoma (APA) or Primary Adrenal Hyperplasia (PAH): Unilateral laproscopic adrenalectomy | Pharmacological therapy | |||||||||||||||||||||||||||||||||||||||||||||||||||||||