II. Epidemiology
- Represents under 6% of Hypertension Causes
- Most common cause of drug Resistant Hypertension
- Peak age 30-50 years
- More common in women
III. Pathophysiology
- Inappropriate Aldosterone Hypersecretion
- Primary Hyperaldosteronism (See Causes below)
- Increased Aldosterone is initiating event
- Results in Sodium retention and volume increase
- Renin decreases
- Secondary Hyperaldosteronism (See Causes below)
- Decreased circulating volume is initiating event
- Results in increased renin and Aldosterone
- Results in Sodium retention
- Primary Hyperaldosteronism (See Causes below)
- Physiologic response to Aldosterone Excess
- Increased renal distal tubular Sodium reabsorption
- Increased total body Sodium content
- Increased water retention
- Escape phenomenon
- Compensatory increased ANF secretion
- Hypertension may not be solely volume expansion
- Increased Peripheral Vascular Resistance
- Hypokalemia: Potassium lost in distal renal tubule (Potassium wasting)
- Alkalosis: Ammoniagenesis
- Hydrogen Ion loss (avid Sodium retention)
- Polyuria: Decreased renal concentrating ability
- Plasma renin suppressed
- Unresponsive to intravascular volume depletion
- Increased renal distal tubular Sodium reabsorption
IV. Causes
- Primary Hyperaldosteronism (Conn's Disease)
- Solitary Adrenal Adenomas (80-90%)
- Bilateral adrenal hyperplasia (10-20%)
- Idiopathic Hyperaldosteronism
- Accounts for 50% of cases at some referral centers
- Adrenal Carcinoma (rare)
- Unilateral Adrenal Hyperplasia (very rare)
- Secondary Hyperaldosteronism
- Hypertensive States
- Primary Reninism (rare renin producing tumor)
- Secondary reninism due to decreased renal perfusion
- Edematous States
- Miscellaneous causes
- Excessive Growth Hormone (Acromegaly)
- Hypertensive States
V. Symptoms
- Often Asymptomatic
- Frontal Headache
- Muscle Weakness to Flaccid Paralysis (Hypokalemia)
- Polyuria and Polydipsia (carbohydrate intolerance)
VI. Signs
-
Hypertension
- May be severe
- Rarely malignant
- Motor Exam with decreased Muscle Strength
VII. Labs
- Serum Electrolytes
- Serum Potassium decreased (Hypokalemia)
- Hypokalemia is the most prominent feature of Hyperaldosteronism
- However, Potassium is normal in 50% of Hyperaldosteronism causes
- Serum Sodium increased (Mild)
- Metabolic Alkalosis
- Serum Potassium decreased (Hypokalemia)
- Morning Aldosterone to PRA ratio
- Ratio over 20-25 (esp if >100) suggests Hyperaldosteronism
- Aldosterone >15 ng/dl and plasma renin low
- Serum Aldosterone alone may be normal in 25% of Hyperaldosteronism patients
- Technique
- Obtain 2 hours after waking and in upright position
- Stop Spironolactone, Eplerenone, Amiloride, Triamterene, Potassium-wasting Diuretics 4 weeks before test
- Consider stopping antihypertensives and NSAIDs before test
- May use Verapamil XR, Hydralazine or Alpha Adrenergic Antagonist for Blood Pressure control
- Saline suppression
- IVF: 300-500 cc/hour for 4 hours
- Normal response
- Aldosterone usually under 0.28
- Renin usually suppressed
VIII. Differential Diagnosis: Hypertension with Hypokalemia
-
Cushing's Disease
- Low Aldosterone and Low Plasma Renin
-
Renal Artery Stenosis or other renal cause
- High Aldosterone and High Plasma Renin
IX. Management
-
Adrenal Adenoma
- Surgical excision
- Adrenal Hyperplasia
- First-Line Agents
- Alternative agents if Gynecomastia develops on Spironolactone
- Precautions
- Follow Serum Potassium and Serum Creatinine every 6 months with these agents
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Related Studies
Definition (NCI) | Overproduction of aldosterone by the adrenal glands, which may lead to hypokalemia and/or hypernatremia.(NICHD) |
Definition (MSH) | A condition caused by the overproduction of ALDOSTERONE. It is characterized by sodium retention and potassium excretion with resultant HYPERTENSION and HYPOKALEMIA. |
Definition (CSP) | abnormality of electrolyte function caused by excessive secretion of aldosterone by the adrenal cortex. |
Concepts | Disease or Syndrome (T047) |
MSH | D006929 |
ICD9 | 255.10, 255.1 |
ICD10 | E26 , E26.9 |
SnomedCT | 88213004, 190506003, 154709005, 190509005, 267484005 |
English | Aldosteronism, Hyperaldosteronism, Hyperaldosteronism, unspecified, aldosteronism, aldosteronism (diagnosis), hyperaldosteronism, Aldosteronism NOS, Hyperaldosteronism NOS, Hyperaldosteronism [Disease/Finding], Hyperaldosteronism NOS (disorder), Aldosteronism (disorder), Aldosteronism, NOS, Hyperaldosteronism, NOS |
Dutch | aldosteronisme NAO, aldosteronisme, Hyperaldosteronisme, niet gespecificeerd, hyperaldosteronisme, Aldosteronisme, Aldosteronisme, hyper-, Hyperaldosteronisme |
French | Aldostéronisme SAI, Aldostéronisme, Hyperaldostéronisme, Hyperaldostéronisme primitif |
German | Aldosteronismus NNB, Hyperaldosteronismus, nicht naeher bezeichnet, Aldosteronismus, Hyperaldosteronismus |
Italian | Aldosteronismo NAS, Aldosteronismo, Iperaldosteronismo |
Portuguese | Aldosteronismo NE, Hiperaldosteronismo, Aldosteronismo |
Spanish | Aldosteronismo NEOM, hiperaldosteronismo, SAI (trastorno), hiperaldosteronismo, SAI, aldosteronismo con hiperplasia de la corteza suprarrenal, hiperaldosteronismo (trastorno), hiperaldosteronismo, Hiperaldosteronismo, Aldosteronismo |
Japanese | アルドステロン症NOS, アルドステロンショウ, アルドステロンショウNOS, コウアルドステロンショウ, コン症候群, アルドステロン過剰症, アルドステロン過剰, アルドステロン症, 高アルドステロン症, Conn症候群 |
Swedish | Hyperaldosteronism |
Czech | aldosteronismus, hyperaldosteronismus, Aldosteronismus, Aldosteronismus NOS, Hyperaldosteronismus |
Finnish | Hyperaldosteronismi |
Russian | KONNA SINDROM, GIPERAL'DOSTERONIZM PERVICHNYI, GIPERAL'DOSTERONIZM, AL'DOSTERONIZM, АЛЬДОСТЕРОНИЗМ, ГИПЕРАЛЬДОСТЕРОНИЗМ, ГИПЕРАЛЬДОСТЕРОНИЗМ ПЕРВИЧНЫЙ, КОННА СИНДРОМ |
Korean | 고알도스테론증, 상세불명의 고알도스테론증 |
Croatian | HIPERALDOSTERONIZAM |
Polish | Aldosteronizm, Zespół Conna, Hiperaldosteronizm pierwotny, Hiperaldosteronizm |
Hungarian | Hyperaldosteronismus k.m.n., Hyperaldosteronismus, Hyperaldosteronizmus |
Norwegian | Hyperaldosteronisme, Aldosteronisme |
Ontology: Conn Syndrome (C1384514)
Definition (NCI) | An endocrine disorder characterized by excessive production of aldosterone by the adrenal glands. Causes include adrenal gland adenoma and adrenal gland hyperplasia. The overproduction of aldosterone results in sodium and water retention and hypokalemia. Patients present with high blood pressure, muscle weakness, and headache. |
Definition (MSH) | Primary hyperaldosteronism caused by the excess production of ALDOSTERONE by an ADENOMA of the ZONA GLOMERULOSA or CONN ADENOMA. |
Definition (CSP) | overproduction of aldosterone by an adrenal cortical adenoma, characterized typically by low potassium levels, underacidity of the body, muscular weakness, excess urination, excess thirst, and high blood pressure. |
Concepts | Disease or Syndrome (T047) |
MSH | D006929 |
ICD9 | 255.12 |
ICD10 | E26.0 , E26.01 |
SnomedCT | 258117004, 13536004, 154709005, 267484005, 190506003, 190507007 |
English | Aldosteronism Primary, Conn Syndrome, primary hyperaldosteronism, primary aldosteronism (diagnosis), primary aldosteronism, Conn's syndrome (diagnosis), Idiopathic aldosteronism, Idiopathic hyperaldosteronism, Conns Syndrome, Conn's Syndrome, Syndrome, Conn's, conn syndrome, conn's syndrome, conns syndrome, Primary aldosteronism (disorder), Conn's syndrome, Conn syndrome, Primary aldosteronism, Primary hyperaldosteronism, Idiopathic aldosteronism (disorder), Primary hyperaldosteronism (disorder), hyperaldosteronism; primary, primary; hyperaldosteronism, Conn, Primary aldosteronism (disorder) [Ambiguous], Syndrome, Conn, Primary Hyperaldosteronism, Hyperaldosteronism, Primary |
Italian | Iperaldosteronismo primitivo, Sindrome di Conn |
Dutch | Conn-syndroom, hyperaldosteronisme; primair, primair; hyperaldosteronisme, Primair hyperaldosteronisme, primair hyperaldosteronisme |
French | Syndrome de conn, Syndrome de Conn, Hyperaldostéronisme primaire, Hyperaldostéronisme primaire à rénine basse |
German | Conn-Syndrom, Primaerer Hyperaldosteronismus, primaerer Hyperaldosteronismus |
Portuguese | Síndrome de Conn, Hiperaldesteronismo primário |
Spanish | Síndrome de Conn, aldosteronismo idiopático, aldosteronismo primario, hiperaldosteronismo idiopático (trastorno), hiperaldosteronismo idiopático, hiperaldosteronismo primario (concepto no activo), hiperaldosteronismo primario (trastorno), hiperaldosteronismo primario, síndrome de Conn, Hiperaldosteronismo primario |
Japanese | 原発性アルドステロン症, コーン症候群, コーンショウコウグン, ゲンパツセイアルドステロンショウ |
Czech | Primární hyperaldosteronismus, primární hyperaldosteronismus, Connův syndrom |
Korean | 원발성 고알도스테론증 |
Croatian | CONNOV SINDROM, PRIMARNI HIPERALDOSTERONIZAM |
Hungarian | Conn-syndroma, Primaer hyperaldosteronismus |