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==What are the symptoms of Primary hyperaldosteronism==
==What are the symptoms of Primary hyperaldosteronism==
==Common Symptoms==
==Common Symptoms==
Common symptoms of primary hyperaldosteronism (PA) include:<ref name="pmid4714286">{{cite journal |vauthors=Rubidge CJ, O'Dowd PB, Powell SJ |title=Difetarsone in the treatment of Trichuris trichiura infections |journal=S. Afr. Med. J. |volume=47 |issue=23 |pages=991–2 |year=1973 |pmid=4714286 |doi= |url=}}</ref><ref name="pmid16932426">{{cite journal |vauthors=Mattsson C, Young WF |title=Primary aldosteronism: diagnostic and treatment strategies |journal=Nat Clin Pract Nephrol |volume=2 |issue=4 |pages=198–208; quiz, 1 p following 230 |year=2006 |pmid=16932426 |doi=10.1038/ncpneph0151 |url=}}</ref><ref name="pmid3216243">{{cite journal |vauthors=Di Tullio M, Alli C, Avanzini F, Bettelli G, Colombo F, Devoto MA, Marchioli R, Mariotti G, Radice M, Taioli E |title=Prevalence of symptoms generally attributed to hypertension or its treatment: study on blood pressure in elderly outpatients (SPAA) |journal=J Hypertens Suppl |volume=6 |issue=1 |pages=S87–90 |year=1988 |pmid=3216243 |doi= |url=}}</ref><ref name="pmid21278718">{{cite journal |vauthors=Unwin RJ, Luft FC, Shirley DG |title=Pathophysiology and management of hypokalemia: a clinical perspective |journal=Nat Rev Nephrol |volume=7 |issue=2 |pages=75–84 |year=2011 |pmid=21278718 |doi=10.1038/nrneph.2010.175 |url=}}</ref><ref name="pmid546663">{{cite journal |vauthors=Bautista J, Gil-Neciga E, Gil-Peralta A |title=Hypokalemic periodic paralysis in primary hyperaldosteronism. Subclinical myopathy with atrophy of the type 2A muscle fibers |journal=Eur. Neurol. |volume=18 |issue=6 |pages=415–20 |year=1979 |pmid=546663 |doi= |url=}}</ref><ref name="pmid12908077">{{cite journal |vauthors=Bortolotto LA, Cesena FH, Jatene FB, Silva HB |title=Malignant hypertension and hypertensive encephalopathy in primary aldosteronism caused by adrenal adenoma |journal=Arq. Bras. Cardiol. |volume=81 |issue=1 |pages=97–100, 93–6 |year=2003 |pmid=12908077 |doi= |url=}}</ref>
Common symptoms of primary hyperaldosteronism (PA) include:


=== Hypertension related symptoms ===
=== Hypertension related symptoms ===
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== Less Common Symptoms ==
== Less Common Symptoms ==
Less common symptoms of Conn's syndrome (primary hyperaldosteronism) include:<ref name="pmid5596496">{{cite journal |vauthors=Moeller J, Muniz B |title=[Hypokalemic ileus and aldosteronism] |language=German |journal=Med Klin |volume=62 |issue=52 |pages=2019–24 |year=1967 |pmid=5596496 |doi= |url=}}</ref><ref name="pmid15024897">{{cite journal |vauthors=Failor RA, Capell PT |title=Hyperaldosteronism and pheochromocytoma: new tricks and tests |journal=Prim. Care |volume=30 |issue=4 |pages=801–20, viii |year=2003 |pmid=15024897 |doi= |url=}}</ref>
Less common symptoms of Conn's syndrome (primary hyperaldosteronism) include:
* Paralysis
* Paralysis
* Racing of the heart(Palpitations)
* Racing of the heart(Palpitations)
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==What causes Primary hyperaldosteronism==
==What causes Primary hyperaldosteronism==
=== Common Causes ===
=== Common Causes ===
Common causes of primary hyperaldosteronism (PA) may be divided into:<ref name="urlPrimary aldosteronism: renaissance of a syndrome - Young - 2007 - Clinical Endocrinology - Wiley Online Library">{{cite web |url=http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2265.2007.02775.x/full |title=Primary aldosteronism: renaissance of a syndrome - Young - 2007 - Clinical Endocrinology - Wiley Online Library |author= |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref><ref name="pmid24944753">{{cite journal |vauthors=Aronova A, Iii TJ, Zarnegar R |title=Management of hypertension in primary aldosteronism |journal=World J Cardiol |volume=6 |issue=5 |pages=227–33 |year=2014 |pmid=24944753 |pmc=4062125 |doi=10.4330/wjc.v6.i5.227 |url= |issn=}}</ref>
Common causes of primary hyperaldosteronism (PA) may be divided into:
*[[Adrenal gland|Adrenal]] causes:
*[[Adrenal gland|Adrenal]] causes:
**[[Aldosterone]]-secreting [[Adrenal gland|adrenal]] [[adenoma]] (APA-[[benign tumor]], 50-60%)
**[[Aldosterone]]-secreting [[Adrenal gland|adrenal]] [[adenoma]] (APA-[[benign tumor]], 50-60%)
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**[[Ectopic]] secretion of [[aldosterone]] ([[ovaries]] and [[kidneys]])
**[[Ectopic]] secretion of [[aldosterone]] ([[ovaries]] and [[kidneys]])
===Less Common Causes===
===Less Common Causes===
Less common causes of primary hyperladosteronism include:<ref name="urlPrimary aldosteronism: renaissance of a syndrome - Young - 2007 - Clinical Endocrinology - Wiley Online Library2">{{cite web |url=http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2265.2007.02775.x/full |title=Primary aldosteronism: renaissance of a syndrome - Young - 2007 - Clinical Endocrinology - Wiley Online Library |author= |authorlink= |coauthors= |date= |format= |work= |publisher= |pages= |language= |archiveurl= |archivedate= |quote= |accessdate=}}</ref><ref name="pmid16003173">{{cite journal |vauthors=So A, Duffy DL, Gordon RD, Jeske YW, Lin-Su K, New MI, Stowasser M |title=Familial hyperaldosteronism type II is linked to the chromosome 7p22 region but also shows predicted heterogeneity |journal=J. Hypertens. |volume=23 |issue=8 |pages=1477–84 |year=2005 |pmid=16003173 |doi= |url= |issn=}}</ref><ref name="pmid22707896">{{cite journal |vauthors=Song MS, Seo SW, Bae SB, Kim YJ, Kim SJ |title=Aldosterone-producing adrenocortical carcinoma without hypertension |journal=Korean J. Intern. Med. |volume=27 |issue=2 |pages=221–3 |year=2012 |pmid=22707896 |pmc=3372808 |doi=10.3904/kjim.2012.27.2.221 |url= |issn=}}</ref>
Less common causes of primary hyperladosteronism include:
*Familial hyperaldosteronism type I ([[glucocorticoid]]-remediable aldosteronism [GRA])
*Familial hyperaldosteronism type I ([[glucocorticoid]]-remediable aldosteronism [GRA])
*Familial hyperaldosteronism II (the familial occurrence of APA or bilateral idiopathic hyperplasia or both)
*Familial hyperaldosteronism II (the familial occurrence of APA or bilateral idiopathic hyperplasia or both)
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==Diagnosis==
==Diagnosis==
Laboratory findings consistent with the diagnosis of primary hyperaldosteronism include plasma aldosterone to renin activity ratio (PAC/PRA) of >30<ref name="urlThe 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">{{cite web |url=http://press.endocrine.org/doi/10.1210/jc.2015-4061 |title=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 |format= |work= |accessdate=}}</ref>, serum aldosterone value of > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour after fludrocortisone supression test, or a plasma aldosterone more than 10 ng / dl on saline infusion test or on oral sodium loading test, the post-test 24-hour urinary aldosterone excretion less than 12 μg / day and a urinary sodium excretion of more than 200 mmol / day. The adrenal venous sampling test is gold standard for subtype classification of primary hyperaldosteronism.
Laboratory findings consistent with the diagnosis of primary hyperaldosteronism include plasma aldosterone to renin activity ratio (PAC/PRA) of >30, serum aldosterone value of > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour after fludrocortisone supression test, or a plasma aldosterone more than 10 ng / dl on saline infusion test or on oral sodium loading test, the post-test 24-hour urinary aldosterone excretion less than 12 μg / day and a urinary sodium excretion of more than 200 mmol / day. The adrenal venous sampling test is gold standard for subtype classification of primary hyperaldosteronism.


=== Plasma Aldosterone to Renin Ratio (PAC/PRA) ===
=== Plasma Aldosterone to Renin Ratio (PAC/PRA) ===
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==== Protocol ====
==== Protocol ====
* Drugs that affect the renin–angiotensin-aldosterone axis should be stopped before testing, such as: beta-blockers, ACE inhibitors, ARBs (angiotensin receptor blockers), renin inhibitors, dihydropyridine calcium channel blockers, and central alpha2-agonists, for about fourteen days, and spironolactone, eplerenone, amiloride, and triamterene, and loop diuretics for about twenty eight days.
* Drugs that affect the renin–angiotensin-aldosterone axis should be stopped before testing, such as: beta-blockers, ACE inhibitors, ARBs (angiotensin receptor blockers), renin inhibitors, dihydropyridine calcium channel blockers, and central alpha2-agonists, for about fourteen days, and spironolactone, eplerenone, amiloride, and triamterene, and loop diuretics for about twenty eight days.
* The test should be conducted between 8 a.m. and 10 a. m. The patient is advised to stay upright for 2 hours prior to testing, and then sit for about 10 minutes before testing.<ref name="pmid11881117">{{cite journal |vauthors=Stowasser M, Gordon RD, Rutherford JC, Nikwan NZ, Daunt N, Slater GJ |title=Diagnosis and management of primary aldosteronism |journal=J Renin Angiotensin Aldosterone Syst |volume=2 |issue=3 |pages=156–69 |year=2001 |pmid=11881117 |doi=10.3317/jraas.2001.022 |url= |issn=}}</ref>
* The test should be conducted between 8 a.m. and 10 a. m. The patient is advised to stay upright for 2 hours prior to testing, and then sit for about 10 minutes before testing.


==== Interpretation ====
==== Interpretation ====
* Primary hyperaldosteronism (Conn's syndrome) is associated with an increased aldosterone levels (PAC) in plasma along with suppressed renin concentration (PRA) due to feedback inhibition of aldosterone on renin levels in the plasma.  
* Primary hyperaldosteronism (Conn's syndrome) is associated with an increased aldosterone levels (PAC) in plasma along with suppressed renin concentration (PRA) due to feedback inhibition of aldosterone on renin levels in the plasma.  
* A PAC/PRA ratio of >30 is a strong evidence of primary hyperladosteronism and value >50 is considered diagnostic in the presence of resistant hypertension, hypokalemia and metabolic alkalosis.<ref name="urlThe 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">{{cite web |url=http://press.endocrine.org/doi/10.1210/jc.2015-4061 |title=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 |format= |work= |accessdate=}}</ref><ref name="pmid3230101">{{cite journal |vauthors=Horsley MG, Bailie GR |title=Effectiveness of theophylline monitoring by the use of serum assays |journal=J Clin Pharm Ther |volume=13 |issue=5 |pages=359–64 |year=1988 |pmid=3230101 |doi= |url= |issn=}}</ref><ref name="pmid22167725">{{cite journal |vauthors=Ríos MC, Izquierdo A, Sotelo M, Honnorat E, Rodríguez Cuimbra S, Catay E, Popescu BM |title=[Aldosterone/renin ratio in the diagnosis of primary aldosteronism] |language=Spanish; Castilian |journal=Medicina (B Aires) |volume=71 |issue=6 |pages=525–30 |year=2011 |pmid=22167725 |doi= |url= |issn=}}</ref><ref name="pmid24526370">{{cite journal |vauthors=Pilz S, Kienreich K, Gaksch M, Grübler M, Verheyen N, Bersuch LA, Schmid J, Drechsler C, Ritz E, Moosbrugger A, Stepan V, Pieber TR, Meinitzer A, März W, Tomaschitz A |title=Aldosterone to active Renin ratio as screening test for primary aldosteronism: reproducibility and influence of orthostasis and salt loading |journal=Horm. Metab. Res. |volume=46 |issue=6 |pages=427–32 |year=2014 |pmid=24526370 |doi=10.1055/s-0034-1367033 |url= |issn=}}</ref><ref name="urlThe Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline: The Journal of Clinical Endocrinology & Metabolism: Vol 101, No 52">{{cite web |url=http://press.endocrine.org/doi/10.1210/jc.2015-4061 |title=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 |format= |work= |accessdate=}}</ref><ref name="pmid16617310">{{cite journal |vauthors=Doi SA, Abalkhail S, Al-Qudhaiby MM, Al-Humood K, Hafez MF, Al-Shoumer KA |title=Optimal use and interpretation of the aldosterone renin ratio to detect aldosterone excess in hypertension |journal=J Hum Hypertens |volume=20 |issue=7 |pages=482–9 |year=2006 |pmid=16617310 |doi=10.1038/sj.jhh.1002024 |url= |issn=}}</ref><ref name="pmid245263702">{{cite journal |vauthors=Pilz S, Kienreich K, Gaksch M, Grübler M, Verheyen N, Bersuch LA, Schmid J, Drechsler C, Ritz E, Moosbrugger A, Stepan V, Pieber TR, Meinitzer A, März W, Tomaschitz A |title=Aldosterone to active Renin ratio as screening test for primary aldosteronism: reproducibility and influence of orthostasis and salt loading |journal=Horm. Metab. Res. |volume=46 |issue=6 |pages=427–32 |year=2014 |pmid=24526370 |doi=10.1055/s-0034-1367033 |url= |issn=}}</ref>
* A PAC/PRA ratio of >30 is a strong evidence of primary hyperladosteronism and value >50 is considered diagnostic in the presence of resistant hypertension, hypokalemia and metabolic alkalosis.<ref name="urlThe 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">{{cite web |url=http://press.endocrine.org/doi/10.1210/jc.2015-4061 |title=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 |format= |work= |accessdate=}}</ref>


=== Confirmatory Tests ===
=== Confirmatory Tests ===
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'''''1. Fludrocortisone suppression test (FST)''''' 
'''''1. Fludrocortisone suppression test (FST)''''' 
* This is the gold standard test for confirmation of primary hyperaldosteronism.<ref name="pmid24627898">{{cite journal |vauthors=Djajadiningrat-Laanen SC, Galac, Boevé MH, Boroffka SA, Naan EC, IJzer J, Kooistra HS |title=Evaluation of the oral fludrocortisone suppression test for diagnosing primary hyperaldosteronism in cats |journal=J. Vet. Intern. Med. |volume=27 |issue=6 |pages=1493–9 |year=2013 |pmid=24627898 |doi= |url=}}</ref><ref name="pmid22689209">{{cite journal |vauthors=Willenberg HS, Vonend O, Schott M, Gao X, Blondin D, Saleh A, Rump LC, Scherbaum WA |title=Comparison of the saline infusion test and the fludrocortisone suppression test for the diagnosis of primary aldosteronism |journal=Horm. Metab. Res. |volume=44 |issue=7 |pages=527–32 |year=2012 |pmid=22689209 |doi=10.1055/s-0032-1314786 |url=}}</ref><ref name="pmid6986736">{{cite journal |vauthors=Lund JO, Nielsen MD |title=Fludrocortisone suppression test in normal subjects, in patients with essential hypertension and in patients with various forms of aldosteronism |journal=Acta Endocrinol. |volume=93 |issue=1 |pages=100–7 |year=1980 |pmid=6986736 |doi= |url=}}</ref>
* This is the gold standard test for confirmation of primary hyperaldosteronism.


* Patient is given a synthetic mineralocorticoid (9-[alpha]-fludrocortisone acetate 0.1 mg every six hours) and sodium chloride [slow-release sodium 30 mmol (1.75 g) three times daily].
* Patient is given a synthetic mineralocorticoid (9-[alpha]-fludrocortisone acetate 0.1 mg every six hours) and sodium chloride [slow-release sodium 30 mmol (1.75 g) three times daily].
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* Patient is fed a high sodium diet, of approximately 218 mmol / day, for three days.  
* Patient is fed a high sodium diet, of approximately 218 mmol / day, for three days.  
* On the third day, a 24-hour urine sample is collected.  
* On the third day, a 24-hour urine sample is collected.  
* Normal suppression is defined as post-test 24-hour urinary aldosterone excretion less than 12 μg / day and a urinary sodium excretion of more than 200 mmol / day.<ref name="pmid25630564">{{cite journal |vauthors=Weigel M, Riester A, Hanslik G, Lang K, Willenberg HS, Endres S, Allolio B, Beuschlein F, Reincke M, Quinkler M |title=Post-saline infusion test aldosterone levels indicate severity and outcome in primary aldosteronism |journal=Eur. J. Endocrinol. |volume=172 |issue=4 |pages=443–50 |year=2015 |pmid=25630564 |doi=10.1530/EJE-14-1013 |url=}}</ref><ref name="pmid25931414">{{cite journal |vauthors=Nanba K, Tsuiki M, Umakoshi H, Nanba A, Hirokawa Y, Usui T, Tagami T, Shimatsu A, Suzuki T, Tanabe A, Naruse M |title=Shortened saline infusion test for subtype prediction in primary aldosteronism |journal=Endocrine |volume=50 |issue=3 |pages=802–6 |year=2015 |pmid=25931414 |doi=10.1007/s12020-015-0615-9 |url=}}</ref>
* Normal suppression is defined as post-test 24-hour urinary aldosterone excretion less than 12 μg / day and a urinary sodium excretion of more than 200 mmol / day.
'''''4. Captopril challenge test''''' 
'''''4. Captopril challenge test''''' 
* Positive test for primary hyperaldosteronism is defined as a PAC / PRA > 30, measured two hours after the administration of 25 mg or 50 mg of captopril with patients in the sitting position.<ref name="pmid25031295">{{cite journal |vauthors=Kuo CC, Balakrishnan P, Hsein YC, Wu VC, Chueh SC, Chen YM, Wu KD, Wang MJ |title=The value of losartan suppression test in the confirmatory diagnosis of primary aldosteronism in patients over 50 years old |journal=J Renin Angiotensin Aldosterone Syst |volume=16 |issue=3 |pages=587–98 |year=2015 |pmid=25031295 |pmc=4297265 |doi=10.1177/1470320313498632 |url=}}</ref>
* Positive test for primary hyperaldosteronism is defined as a PAC / PRA > 30, measured two hours after the administration of 25 mg or 50 mg of captopril with patients in the sitting position.
* Reserved for patients with reduced cardiac or renal function.
* Reserved for patients with reduced cardiac or renal function.


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* Limitations are similar to that of CT scan.
* Limitations are similar to that of CT scan.
'''''3. Adrenal venous sampling (AVS)'''''   
'''''3. Adrenal venous sampling (AVS)'''''   
* Gold standard test for subtype classification.<ref name="pmid26854152">{{cite journal |vauthors=Deipolyi AR, Bailin A, Wicky S, Alansari S, Oklu R |title=Adrenal Vein Sampling for Conn's Syndrome: Diagnosis and Clinical Outcomes |journal=Diagnostics (Basel) |volume=5 |issue=2 |pages=254–71 |year=2015 |pmid=26854152 |pmc=4665593 |doi=10.3390/diagnostics5020254 |url=}}</ref>
* Gold standard test for subtype classification.


* It has a high sensitivity (95%) and specificity (100%) for the detection of unilateral aldosterone excess but is highly expertise dependent.<ref name="pmid24218436">{{cite journal |vauthors=Rossi GP, Auchus RJ, Brown M, Lenders JW, Naruse M, Plouin PF, Satoh F, Young WF |title=An expert consensus statement on use of adrenal vein sampling for the subtyping of primary aldosteronism |journal=Hypertension |volume=63 |issue=1 |pages=151–60 |year=2014 |pmid=24218436 |doi=10.1161/HYPERTENSIONAHA.113.02097 |url=}}</ref>
* It has a high sensitivity (95%) and specificity (100%) for the detection of unilateral aldosterone excess but is highly expertise dependent.
* AVS can be performed using any of the three protocols:
* AVS can be performed using any of the three protocols:
**  (a) Unstimulated sequential or simultaneous bilateral AVS
**  (a) Unstimulated sequential or simultaneous bilateral AVS
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* A gradient of > 4:1, points towards unilateral aldosterone secreting adenoma and < 3 : 1 suggests bilateral adrenal hyperplasia.  
* A gradient of > 4:1, points towards unilateral aldosterone secreting adenoma and < 3 : 1 suggests bilateral adrenal hyperplasia.  
'''''4. Posture stimulation test'''''
'''''4. Posture stimulation test'''''
* May be used when AVS is equivocal.<ref name="pmid7955437">{{cite journal |vauthors=Feltynowski T, Ignatowska-Switalska H, Wocial B, Lewandowski J, Chodakowska J, Januszewicz W |title=Postural stimulation test in patients with aldosterone producing adenomas |journal=Clin. Endocrinol. (Oxf) |volume=41 |issue=3 |pages=309–14 |year=1994 |pmid=7955437 |doi= |url=}}</ref>
* May be used when AVS is equivocal.  
* It is based on the principal that PAC in patients with aldosterone producing adenoma (APA) there is a diurnal variation and is relatively unchanged by changes in the angiotensin II levels being under ACTH control, whereas, bilateral adrenal hyperplasia (IHA) is affected heavily by a small change in the angiotensin II, due to standing.  
* It is based on the principal that PAC in patients with aldosterone producing adenoma (APA) there is a diurnal variation and is relatively unchanged by changes in the angiotensin II levels being under ACTH control, whereas, bilateral adrenal hyperplasia (IHA) is affected heavily by a small change in the angiotensin II, due to standing.  
'''''5. Iodocholesterol scintigraphy (NP-59 scan)'''''
'''''5. Iodocholesterol scintigraphy (NP-59 scan)'''''
* 6 beta- 131I iodomethyl-19-norcholesterol (NP-59), was introduced in 1977 for the diagnosis for primary aldosteronism.  
* 6 beta- 131I iodomethyl-19-norcholesterol (NP-59), was introduced in 1977 for the diagnosis for primary aldosteronism.  
* The NP-59 scan is performed with dexamethasone suppression.
* The NP-59 scan is performed with dexamethasone suppression.
* It is not very useful in identifying micro adenomas of the adrenals (<1.5cm).<ref name="pmid24235884">{{cite journal |vauthors=Chen YC, Chiu JS, Wang YF |title=NP-59 SPECT/CT imaging in stage 1 hypertensive and atypical primary aldosteronism: a 5-year retrospective analysis of clinicolaboratory and imaging features |journal=ScientificWorldJournal |volume=2013 |issue= |pages=317934 |year=2013 |pmid=24235884 |pmc=3818974 |doi=10.1155/2013/317934 |url=}}</ref>
* It is not very useful in identifying micro adenomas of the adrenals (<1.5cm).
'''''6. 18-Hydroxycorticosterone levels'''''
'''''6. 18-Hydroxycorticosterone levels'''''
* Less accurate than other tests.
* Less accurate than other tests.
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* Hydroxylation of corticosterone leads to the formation of 18-hydroxycorticosterone.
* Hydroxylation of corticosterone leads to the formation of 18-hydroxycorticosterone.
* Historically, it was used to differentiate APA from bilateral adrenal hyperplasia.  
* Historically, it was used to differentiate APA from bilateral adrenal hyperplasia.  
* Supine plasma 18-hydroxycorticosterone levels > 100 ng/dl at 8 a.m., suggest aldosterone producing adenoma (APA).<ref name="pmid22238407">{{cite journal |vauthors=Mulatero P, di Cella SM, Monticone S, Schiavone D, Manzo M, Mengozzi G, Rabbia F, Terzolo M, Gomez-Sanchez EP, Gomez-Sanchez CE, Veglio F |title=18-hydroxycorticosterone, 18-hydroxycortisol, and 18-oxocortisol in the diagnosis of primary aldosteronism and its subtypes |journal=J. Clin. Endocrinol. Metab. |volume=97 |issue=3 |pages=881–9 |year=2012 |pmid=22238407 |doi=10.1210/jc.2011-2384 |url=}}</ref>
* Supine plasma 18-hydroxycorticosterone levels > 100 ng/dl at 8 a.m., suggest aldosterone producing adenoma (APA).  




==Treatment options==
==Treatment options==
=== Medical Therapy ===
The optimal therapy for primary hyperladosteronism depends on the etiology of hyperaldosteronism. Medical therapy is indicated for bilateral adrenal hyperplasia, and all ambiguous causes of primary hyperaldosteronism. The following medications may be used depending on the situation:
# Mineralocorticoid receptor antagonists (Spironolactone, Potassium Canrenoate, Eplerenone)
# Potassium sparing diuretics (Amiloride, Triamterene)
# Calium channel blockers (Amlodipine, Nifedipine)
# ACE inhibitors (Lisinopril, Captopril)
# Angiotensin receptor blockers (Valsartan, Candesartan, Losartan)
# Dexamethaspne therapy (For familial hyperaldosteronism type I)
=== Surgery ===
Surgery is the mainstay of treatment for unilateral adrenal hyperplasia, aldosterone producing adenomas (APAs), adrenal carcinoma, ectopic ACTH, renin, and deoxycorticosterone secreting tumors. Laproscopic adrenalectomy may cure the disease.
=== Post Surgical Prognosis ===
Good prognosis after adrenalectomy depends on:
* Good response to medical therapy with spironolactone
* Young age
* Decreased duration of hypertension
* Preoperative use of two or fewer antihypertensive agents


==Diseases with similar symptoms==
==Diseases with similar symptoms==
Primary hyperaldosteronism must be differentiated from other diseases that cause [[hypertension]] and [[hypokalemia]] such as:
* [[Renal artery stenosis]]
* [[Cushing's syndrome (patient information)|Cushing's syndrome]]
* [[Congenital adrenal hyperplasia]]
* [[Liddle's syndrome]]
* [[Diuretic]] use
* [[Licorice]] ingestion
* [[Renin]]-secreting tumors


==Where to find medical care for primary hyperaldosteronism==
==Where to find medical care for primary hyperaldosteronism==


==What to expect?(Outlook/Prognosis)==
==What to expect?(Outlook/Prognosis)==
The prognosis of primary hyperaldosteronism is good with treatment. Without treatment, primary hyperaldosteronism will result in hypertension with resultant hypertension-related complications, which may be a major cause of morbidity and mortality among patients.
* Adrenalectomy lowers long-term all-cause mortality from primary hyperaldosteronism.
'''Patients undergoing unilateral adrenalectomy for unilateral adenoma'''
* Adrenalectomy leads to cure of HTN in 50% to 60% of patients.
* Blood pressure typically becomes normal after 1 to 6 months of the procedure.
* Treatment leads to a significant increase in quality of life and improved cardiovascular outcomes.
'''Patients receiving aldosterone antagonist medications'''
* Hypertension is controlled in majority of the patients.
* Improvements are not as significant as after unilateral adrenalectomy for lateralizing lesions.
'''Patients with FH-I undergoing treatment with glucocorticoid medications'''
* Hypertension in familial hyperaldosteronism type I (FH-I) is usually of early onset and may be severe enough to cause early death, usually from hemorrhagic stroke, unless specifically treated.<sup>[[Primary hyperaldosteronism natural history, complications and prognosis#cite note-pmid9483237-20|[20]]]</sup>
* Treatment with glucocorticoids, given in low doses is usually effective in controlling hypertension and consequently preventing stroke.
<references />

Revision as of 12:59, 21 July 2017

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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

What are the symptoms of Primary hyperaldosteronism

Common Symptoms

Common symptoms of primary hyperaldosteronism (PA) include:

Hypertension related symptoms

  • Headaches
  • Facial flushing
  • Weakness
  • Visual impairment
  • Impaired consciousness
  • Seizures (hypertensive encephalopathy)

Hypokalemia related symptoms

  • Constipation
  • Increased urinary frequency and thirst (Polyuria and polydipsia)
  • Weakness

Less Common Symptoms

Less common symptoms of Conn's syndrome (primary hyperaldosteronism) include:

  • Paralysis
  • Racing of the heart(Palpitations)
  • Abdominal fullness/constipation(Ileus)

What causes Primary hyperaldosteronism

Common Causes

Common causes of primary hyperaldosteronism (PA) may be divided into:

Less Common Causes

Less common causes of primary hyperladosteronism include:

  • 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

When to seek urgent medical care?

Diagnosis

Laboratory findings consistent with the diagnosis of primary hyperaldosteronism include plasma aldosterone to renin activity ratio (PAC/PRA) of >30, serum aldosterone value of > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour after fludrocortisone supression test, or a plasma aldosterone more than 10 ng / dl on saline infusion test or on oral sodium loading test, the post-test 24-hour urinary aldosterone excretion less than 12 μg / day and a urinary sodium excretion of more than 200 mmol / day. The adrenal venous sampling test is gold standard for subtype classification of primary hyperaldosteronism.

Plasma Aldosterone to Renin Ratio (PAC/PRA)

Protocol

  • Drugs that affect the renin–angiotensin-aldosterone axis should be stopped before testing, such as: beta-blockers, ACE inhibitors, ARBs (angiotensin receptor blockers), renin inhibitors, dihydropyridine calcium channel blockers, and central alpha2-agonists, for about fourteen days, and spironolactone, eplerenone, amiloride, and triamterene, and loop diuretics for about twenty eight days.
  • The test should be conducted between 8 a.m. and 10 a. m. The patient is advised to stay upright for 2 hours prior to testing, and then sit for about 10 minutes before testing.

Interpretation

  • Primary hyperaldosteronism (Conn's syndrome) is associated with an increased aldosterone levels (PAC) in plasma along with suppressed renin concentration (PRA) due to feedback inhibition of aldosterone on renin levels in the plasma.
  • A PAC/PRA ratio of >30 is a strong evidence of primary hyperladosteronism and value >50 is considered diagnostic in the presence of resistant hypertension, hypokalemia and metabolic alkalosis.[1]

Confirmatory Tests

After preliminary testing for primary hyperaldosteronism via PAC/PRA ratio, any one of the following tests may be performed in order to confirm the diagnosis:

1. Fludrocortisone suppression test (FST) 

  • This is the gold standard test for confirmation of primary hyperaldosteronism.
  • Patient is given a synthetic mineralocorticoid (9-[alpha]-fludrocortisone acetate 0.1 mg every six hours) and sodium chloride [slow-release sodium 30 mmol (1.75 g) three times daily].
  • Plasma aldosterone level is measured in the a.m. after four days of administration.
  • A value of > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour, confirm primary hyperaldosteronism.

2. Intravenous saline load test (SLT) 

  • Patient is infused with two liters of NaCl 0.9% for fours hours.
  • Plasma aldosterone more than 10 ng / dl is confirmatory, normally aldosterone would be suppressed to below 5 ng / dl.

3. Oral sodium loading test 

  • This test has a sensitivity and specificity of >90%
  • Patient is fed a high sodium diet, of approximately 218 mmol / day, for three days.
  • On the third day, a 24-hour urine sample is collected.
  • Normal suppression is defined as post-test 24-hour urinary aldosterone excretion less than 12 μg / day and a urinary sodium excretion of more than 200 mmol / day.

4. Captopril challenge test 

  • Positive test for primary hyperaldosteronism is defined as a PAC / PRA > 30, measured two hours after the administration of 25 mg or 50 mg of captopril with patients in the sitting position.
  • Reserved for patients with reduced cardiac or renal function.

Less Common Tests

  • Frusemide upright posture test
  • 24-hour urinary aldosterone
  • Losartan test

Subtype Classification

Once the diagnosis of primary hyperaldosteronism is confirmed, a subtype classification is required as the management may vary based on the etiology.

Tests useful in assessing subtypes are:

1. Computed Tomography (CT) 

  • A high-resolution CT (HRCT) scan with contrast, has a high sensitivity (78%) and specificity (75%) for detection of adrenal masses (inluding aldosterone producing adenomas-APAs)
  • CT scan is best when used for adrenal adenomas > 2cm but accuracy decreases if the mass is < 1cm.
  • A unilateral lesion exceeding 4 cm suggests possible carcinoma
  • Moreover, it cannot distinguish between a functional APA and a non-secreting adrenal adenoma (incidentaloma).

2. Magnetic Resonance Imaging (MRI)

  • Sensitivity of 70 to 100% in detecting APA, depending on the size of the lesion, being greatest for lesions > 2 cm.
  • Limitations are similar to that of CT scan.

3. Adrenal venous sampling (AVS) 

  • Gold standard test for subtype classification.
  • It has a high sensitivity (95%) and specificity (100%) for the detection of unilateral aldosterone excess but is highly expertise dependent.
  • AVS can be performed using any of the three protocols:
    • (a) Unstimulated sequential or simultaneous bilateral AVS
    • (b) Unstimulated sequential or simultaneous bilateral AVS followed by bolus cosyntropin-stimulated sequential or simultaneous bilateral AVS
    • (c) Continuous cosyntropin infusion with sequential bilateral AVS.
  • Plasma aldosterone collected from the adrenal veins is corrected to its respective plasma cortisol, measured as a ratio (PAC / cortisol ratio)
  • A gradient of > 4:1, points towards unilateral aldosterone secreting adenoma and < 3 : 1 suggests bilateral adrenal hyperplasia.

4. Posture stimulation test

  • May be used when AVS is equivocal.
  • It is based on the principal that PAC in patients with aldosterone producing adenoma (APA) there is a diurnal variation and is relatively unchanged by changes in the angiotensin II levels being under ACTH control, whereas, bilateral adrenal hyperplasia (IHA) is affected heavily by a small change in the angiotensin II, due to standing.

5. Iodocholesterol scintigraphy (NP-59 scan)

  • 6 beta- 131I iodomethyl-19-norcholesterol (NP-59), was introduced in 1977 for the diagnosis for primary aldosteronism.
  • The NP-59 scan is performed with dexamethasone suppression.
  • It is not very useful in identifying micro adenomas of the adrenals (<1.5cm).

6. 18-Hydroxycorticosterone levels

  • Less accurate than other tests.
  • Hydroxylation of corticosterone leads to the formation of 18-hydroxycorticosterone.
  • Historically, it was used to differentiate APA from bilateral adrenal hyperplasia.
  • Supine plasma 18-hydroxycorticosterone levels > 100 ng/dl at 8 a.m., suggest aldosterone producing adenoma (APA).


Treatment options

Medical Therapy

The optimal therapy for primary hyperladosteronism depends on the etiology of hyperaldosteronism. Medical therapy is indicated for bilateral adrenal hyperplasia, and all ambiguous causes of primary hyperaldosteronism. The following medications may be used depending on the situation:

  1. Mineralocorticoid receptor antagonists (Spironolactone, Potassium Canrenoate, Eplerenone)
  2. Potassium sparing diuretics (Amiloride, Triamterene)
  3. Calium channel blockers (Amlodipine, Nifedipine)
  4. ACE inhibitors (Lisinopril, Captopril)
  5. Angiotensin receptor blockers (Valsartan, Candesartan, Losartan)
  6. Dexamethaspne therapy (For familial hyperaldosteronism type I)

Surgery

Surgery is the mainstay of treatment for unilateral adrenal hyperplasia, aldosterone producing adenomas (APAs), adrenal carcinoma, ectopic ACTH, renin, and deoxycorticosterone secreting tumors. Laproscopic adrenalectomy may cure the disease.

Post Surgical Prognosis

Good prognosis after adrenalectomy depends on:

  • Good response to medical therapy with spironolactone
  • Young age
  • Decreased duration of hypertension
  • Preoperative use of two or fewer antihypertensive agents

Diseases with similar symptoms

Primary hyperaldosteronism must be differentiated from other diseases that cause hypertension and hypokalemia such as:

Where to find medical care for primary hyperaldosteronism

What to expect?(Outlook/Prognosis)

The prognosis of primary hyperaldosteronism is good with treatment. Without treatment, primary hyperaldosteronism will result in hypertension with resultant hypertension-related complications, which may be a major cause of morbidity and mortality among patients.

  • Adrenalectomy lowers long-term all-cause mortality from primary hyperaldosteronism.

Patients undergoing unilateral adrenalectomy for unilateral adenoma

  • Adrenalectomy leads to cure of HTN in 50% to 60% of patients.
  • Blood pressure typically becomes normal after 1 to 6 months of the procedure.
  • Treatment leads to a significant increase in quality of life and improved cardiovascular outcomes.

Patients receiving aldosterone antagonist medications

  • Hypertension is controlled in majority of the patients.
  • Improvements are not as significant as after unilateral adrenalectomy for lateralizing lesions.

Patients with FH-I undergoing treatment with glucocorticoid medications

  • Hypertension in familial hyperaldosteronism type I (FH-I) is usually of early onset and may be severe enough to cause early death, usually from hemorrhagic stroke, unless specifically treated.[20]
  • Treatment with glucocorticoids, given in low doses is usually effective in controlling hypertension and consequently preventing stroke.
  1. ↑ "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".
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