Conn syndrome wiki

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Conn's Syndrome

The adrenal glands are found above each kidney. They are triangle-shaped, and measure about half an inch in height and 3 inches in length. Each adrenal gland has 2 layers.

  • The adrenal medulla (inner part) makes epinephrine (also called adrenaline).
  • The adrenal cortex (outer part) makes steroid hormones (such as cortisone and aldosterone).

The adrenal glands control many processes in the body. Their job is to keep the body in balance by making various hormones that are critical for maintaining good health.

These hormones do many important things. For example, they help regulate fluid and salt levels in the body that affect blood volume and blood pressure. They also help the body react to stress and change. They cause a faster heart rate and boost other systems that help you to react quickly with a burst of energy when needed. Problems in the cortex or the medulla, then, can result in high blood pressure.

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Hyperaldosteronism

Hormonal disorder

Medical condition

Hyperaldosteronism is a medical condition wherein too much aldosterone is produced by the adrenal glands, which can lead to lowered levels of potassium in the blood (hypokalemia) and increased hydrogen ion excretion (alkalosis).

This cause of mineralocorticoid excess is primary hyperaldosteronism reflecting excess production of aldosterone by adrenal zona glomerulosa. Bilateral micronodular hyperplasia is more common than unilateral adrenal adenoma.

Signs and symptoms[edit]

It can be asymptomatic, but these symptoms may be present:[2]

Cause[edit]

The causes of primary hyperaldosteronism are adrenal hyperplasia and adrenal adenoma (Conn's syndrome). These cause hyperplasia of aldosterone-producing cells of the adrenal cortex resulting in primary hyperaldosteronism. The causes of secondary hyperaldosteronism are Accessory renal veins, FibroMucular Dysplasia, Reninoma, Renal Tubular acidosis, Nutcracker Syndrome, Ectopic tumours, massive ascites, left ventricular failure, and cor pulmonale. These act either by decreasing circulating fluid volume or by decreasing cardiac output, with resulting increase in renin release leading to secondary hyperaldosteronism. Secondary hyperaldosteronism can also be caused by Proximal renal tubular acidosis[4]

Secondary Hyperaldosteronism can also be a symptom of Genetic conditions Bartter's Syndrome and Gitelman's Syndrome.[citation needed]

Diagnosis[edit]

When taking a blood test, the aldosterone-to-renin ratio is abnormally increased in primary hyperaldosteronism, and decreased or normal but with high renin in secondary hyperaldosteronism.[5]

Types[edit]

In endocrinology, the terms 'primary' and 'secondary' are used to describe the abnormality (e.g., elevated aldosterone) in relation to the defect, i.e., the tumor's location. It also refers to causes that are genetic (primary) or due to another condition or influence (secondary).[citation needed]

Primary[edit]

Main article: Primary aldosteronism

Primary aldosteronism (hyporeninemic hyperaldosteronism) was previously thought to be most commonly caused by an adrenal adenoma, termed Conn's syndrome. However, recent studies have shown that bilateral idiopathic adrenal hyperplasia is the cause in up to 70% of cases. Differentiating between the two is important, as this determines treatment. Also, see congenital adrenal hyperplasia. Adrenal carcinoma is an extremely rare cause of primary hyperaldosteronism.[citation needed]

Two familial forms have been identified: type I (dexamethasone suppressible), and type II, which has been linked to the 7p22 gene.[6]

Features

Investigations

Management

  • Adrenal adenoma: surgery
  • Bilateral adrenocortical hyperplasia: aldosterone antagonist, e.g., spironolactone

Secondary[edit]

Secondary refers to an abnormality that indirectly results in pathology through a predictable physiologic pathway, i.e., a renin-producing tumor leads to increased aldosterone, as the body's aldosterone production is normally regulated by renin levels. One cause is a juxtaglomerular cell tumor. Another is renal artery stenosis, in which the reduced blood supply across the juxtaglomerular apparatus stimulates the production of renin. Likewise, fibromuscular dysplasia may cause stenosis of the renal artery, and therefore secondary hyperaldosteronism. Other causes can come from the tubules: low reabsorption of sodium (as seen in Bartter and Gitelman syndromes) will lead to hypovolemia/hypotension, which will activate the renin–angiotensin system (RAAS).[7]

Secondary hyperaldosteronism can also be caused by excessive ingestion of licorice or other members of the Glycyrrhiza genus of plants that contain the triterpenoidsaponinglycoside known as glycyrrhizin. Licorice and closely related plants are perennial shrubs, the roots of which are used in medicine as well as making candies and in cooking other desserts because of the sweet taste. Through inhibition of 11-beta-hydroxysteroid dehydrogenase type 2 (11-beta-HSD2), glycyrrhizin allows cortisol to activate mineralocorticoid receptors in the kidney. This severely potentiates mineralocorticoid receptor-mediated renal sodium reabsorbtion, due to much higher circulating concentrations of cortisol compared to aldosterone. This, in turn, expands the extracellular volume, increases total peripheral resistance and increases arterial blood pressure. The condition is termed pseudohyperaldosteronism.[8]

Secondary hyperaldosterone can also be caused by a genetic mutation in the kidneys which causes sodium and potassium wasting. These conditions can be referred to syndromes such as Bartter Syndrome and Gitelman Syndrome.[9]

Treatment[edit]

Treatment includes removing the causative agent (such as licorice), a high-potassium, low-sodium diet (for primary) and high-sodium diet (for secondary), spironolactone and eplerenone, potassium-sparing diuretics that act as aldosterone antagonists, and surgery, depending on the cause.[10] Secondary hyperaldosteronism may also be treated with cox2 inhibitors which cause water retention, sodium retention and potassium retention as well as raising blood pressure. Bartter and Gitleman syndrome tend to cause low blood pressure in significant populations and treatment with blood pressure medications tend to lower the blood pressure even more.[citation needed]

Other animals[edit]

Main article: Feline hyperaldosteronism

Cats can be affected by hyperaldosteronism. The most common signs in cats are muscle weakness and loss of eyesight, although only one of these signs may be present.[11] Muscle weakness is due to low potassium concentrations in the blood, and signs of muscle weakness, such as being unable to jump, may be intermittent.[11] High blood pressure causes either detachment of the retina, or blood inside the eye, which leads to loss of vision.[11] Hyperaldosteronism caused by a tumor is treated by surgical removal of the affected adrenal gland.[11]

See also[edit]

References[edit]

  1. ^"aldosteronism" at Dorland's Medical Dictionary
  2. ^"Hyperaldosteronism". The Lecturio Medical Concept Library. Retrieved 23 July 2021.
  3. ^"Hyperaldosteronism: eMedicine Pediatrics: General Medicine". Retrieved 2009-06-16.
  4. ^Rodriguez Soriano J, Boichis H, Stark H, Edelmann CM (1967). "Proximal renal tubular acidosis. A defect in bicarbonate reabsorption with normal urinary acidification". Pediatr. Res. 1 (2): 81–98. doi:10.1203/00006450-196703000-00001. PMID 6029811.
  5. ^"Hyperaldosteronism". The Lecturio Medical Concept Library. Retrieved 25 July 2021.
  6. ^Lafferty AR, Torpy DJ, Stowasser M, et al. (November 2000). "A novel genetic locus for low renin hypertension: familial hyperaldosteronism type II maps to chromosome 7 (7p22)". J. Med. Genet. 37 (11): 831–5. doi:10.1136/jmg.37.11.831. PMC 1734468. PMID 11073536.
  7. ^Dominguez A, Muppidi V, Gupta S. "Hyperaldosteronism". National Center for Biotechnology Information, U.S. National Library of Medicine. Retrieved 23 July 2021.
  8. ^Sabbadin C, Armanini D (September 2016). "Syndromes that mimic an excess of mineralocorticoids". High Blood Press Cardiovasc Prev. 23 (3): 231–5. doi:10.1007/s40292-016-0160-5. PMID 27251484. S2CID 207495149.
  9. ^Seyberth, Hannsjörg W.; Schlingmann, Karl P. (October 2011). "Bartter- and Gitelman-like syndromes: salt-losing tubulopathies with loop or DCT defects". Pediatric Nephrology. 26 (10): 1789–1802. doi:10.1007/s00467-011-1871-4. PMC 3163795. PMID 21503667.
  10. ^Harvey AM (June 2014). "Hyperaldosteronism: diagnosis, lateralization, and treatment". Surg Clin North Am. 94 (3): 643–56. doi:10.1016/j.suc.2014.02.007. PMID 24857581.
  11. ^ abcdKooistra, Hans S. (September 2020). "Primary hyperaldosteronism in cats". Veterinary Clinics of North America: Small Animal Practice. 50 (5): 1053–1063. doi:10.1016/j.cvsm.2020.05.007. PMID 32653266.

External links[edit]

Sours: https://en.wikipedia.org/wiki/Hyperaldosteronism
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Columbia Adrenal Center

Adrenal adenoma on CTAldosterone helps control blood pressure by holding onto salt and losing potassium from the blood. The increased salt increases the blood pressure. Hyperaldosteronism is a disease in which the adrenal gland(s) make too much aldosterone which leads to hypertension (high blood pressure) and low blood potassium levels.

Primary hyperaldosteronism can be caused by either hyperactivity in one adrenal gland (unilateral disease) or both (bilateral disease). Unilateral disease is usually caused by an aldosterone producing adenoma (benign tumor) and less commonly by adrenal cancer or hyperplasia (when the whole gland is hyperactive). Bilateral disease is usually caused by bilateral hyperplasia (when both glands are hyperactive). There are rare genetic syndromes like familial hyperaldosteronism type I and II which may cause both glands to be hyperactive.

Signs and Symptoms

High blood pressure is often the only sign of hyperaldosteronism. The hypertension is usually hard to control and patients are often on 4 or more blood pressure medications. The hypertension may cause headaches, blurred vision, and dizziness. While patients with hyperaldosteronism may have normal potassium levels, many patients may have low potassium levels. The hypokalemia (low potassium level) can cause symptoms like fatigue, numbness, increased urination, increased thirst, muscle cramps, and muscle weakness. Hyperaldosteronism leads to an increased risk for heart attacks, heart failure, strokes, kidney failure, and early death.

Diagnosis

Primary hyperaldosteronism is diagnosed by measuring the blood levels of aldosterone and renin (a hormone made by the kidney). To best measure these hormones, blood samples should be drawn in the morning. In primary hyperaldosteronism, the aldosterone level will be high while renin will be low or undetectable. The potassium level may be low or normal. If these tests are positive, then patients may have another test to confirm the diagnosis. These tests try to lower the amount of aldosterone produced either by giving a medication or giving extra salt through the diet or an intravenous fluid. If the aldosterone level is high and the renin level is low after these tests, the diagnosis is confirmed. Some medications, especially blood pressure medications, can interfere with these tests. Your physician will review your medications and decide if any need to be stopped for 4 to 6 weeks prior to testing. In general, patients with a long-standing history of hypertension or difficult to control hypertension should be screened for hyperaldosteronism.

  • PAC:PRA ratio

    This blood test is a screening test, meaning that it is performed if hyperaldosteronism is suspected but not yet diagnosed. A high ratio of PAC to PRA suggests primary hyperaldosteronism, but additional testing may be needed to confirm the diagnosis.

  • Captopril Suppression Test

    This blood test measures the body's response to captopril, a medication used to treat high blood pressure. Results of this test can confirm whether a person has primary hyperaldosteronism.

  • 24-hour Urinary Excretion of Aldosterone Test

    Patients eat a high-salt diet for five days, and then undergo urine tests during a 24-hour period. If the level of aldosterone in the urine is high, such results can confirm that a patient has primary hyperaldosteronism.

  • Saline Suppression Test

    In this test, the patient receives a salt solution through an IV. A blood test then measures the patient's levels of aldosterone and renin. Primary hyperaldosteronism can be confirmed if the level of aldosterone in the blood remains high and renin is low after this salt loading.

Localization

Dr. Morrissey demonstrates the catheter used in Adrenal Venous Sampling. Click here to see the full article.For patients with proven primary hyperaldosteronism, the next step is to figure out if this is unilateral or bilateral disease. This is critical because the treatment for each is different. A computed tomography (CT or CAT) scan or magnetic resonance imaging (MRI) scan will be done to see if there is a tumor in either adrenal gland. If the patient is younger than 40 years old and there is a tumor in only one adrenal (especially if it is between 1 and 2 cm), then the patient may go directly to treatment. If the patient is older than 40 and/or there is either no tumor seen or tumors in both adrenals, then the patient will have a special test called selective venous sampling. In this test, a surgeon or radiologist will draw blood directly from the veins draining both adrenal glands to determine if one or both are making too much hormone. Significantly higher hormone levels on one side only clinches the diagnosis of unilateral disease. Selective venous sampling is usually done as an outpatient procedure.

Treatment

The treatment for hyperaldosteronism depends on the underlying cause. In patients with a single benign tumor (adenoma), surgical removal (adrenalectomy) is curative. This operation is usually performed laparoscopically, through several very small incisions. (See Adrenal Surgery) After successful adrenalectomy, approximately 95% of patients notice significant improvement in their hypertension. Of this 95%, one third are cured of high blood pressure and the rest are on fewer medications or lower dosages. For patients with bilateral hyperplasia, the best treatment is a medication called an aldosterone-antagonist (spironolactone, eplerenone) which blocks the effect of aldosterone. In addition, patients are maintained on a low salt diet.

Without proper treatment, patients with hyperaldosteronism often suffer from poorly controlled high blood pressure and are at increased risk for heart attacks, heart failure, strokes, kidney failure, and early death. However, with appropriate treatment, this disease is treatable and has an excellent prognosis. Patients with difficult to control or long-standing hypertension should be screened for primary hyperaldosteronism.

Next Steps

If you are dealing with an adrenal issue, our team at the Columbia Adrenal Center is here to help. Call (212) 305-0444 or request an appointment online.

Sours: https://columbiasurgery.org/conditions-and-treatments/primary-hyperaldosteronism-conns-syndrome
Hyperaldosteronism - causes, symptoms, diagnosis, treatment, pathology

Hyperaldosteronism

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Resident
Survival
Guide

Editor-In-Chief:C. Michael Gibson, M.S., M.D.[1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D[2]Syed Hassan A. Kazmi BSc, MD[3]

This page contains general information about hyperaldosteronism. For more information on specific types, please visit the pages on primary hyperaldosteronism.

Synonyms and Keywords: Aldosteronism

Overview

Hyperaldosteronism is a clinical scenario of mineralocorticoid excess with resistant hypertension, hypokalemia, and metabolic alkalosis due to increased hydrogen ion excretion. Aldosteronism may be classified into three types, primary hyperaldosteronism (Conn's syndrome), secondary hyperaldosteronism, and pseudohyperaldosteronism. Primary hyperaldosteronism can caused by aldosterone-secreting adenoma, bilateral hyperplasia of the adrenal glands, and ectopic secretion of aldosterone from ovaries and kidneys. Primary hyperaldosteronism features overproduction of aldosterone despite suppressed plasma renin activity (PRA). The resulting Na+ retention produces hypertension, and increased K+ excretion may cause hypokalemia. Secondary hyperaldosteronism is caused by high renin and subsequently aldosterone level, such as renovascular causes and reninoma. Pseudohyperaldosteronism is the clinical presentation of hyperaldosteronism such as resistant hypertension, hypokalemia, and metabolic alkalosis due to factors other than renin and aldosterone. The treatment should be prescribed for the blockade of aldosterone effects, or based on underlying disease.

Classification

Aldosteronism and mineralocorticoid excess may be classified into three types, primary hyperaldosteronism (conn's syndrome), secondary hyperaldosteronism, and pseudohyperaldosteronism. The different types of mineralocorticoid excess are described below.

Primary hyperaldosteronism (conn's syndrome)

Primary hyperaldosteronism causes categorizes as below table: [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]

Secondary hyperaldosteronism

Secondary hyperaldosteronism causes are categorized as below, and each specific disease is described in the related micro-chapter. [16][17][18][19][20][21]

Pseudohyperaldosteronism causes (low renin)

Pseudohyperaldosteronism causes are classified as below: [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]

Pseudohyperaldosteronism causes Disease Etiology Clinical features Labratory Treatment
Elevated mineralocorticoid Renin Aldosterone Other
Endogenous causes 17 alpha-hydroxylase deficiencyMutations in the CYP17A1 gene Deoxycorticosterone (DOC) Cortisol ↓ Corticosteroids
11β-hydroxylase deficiencyMutations in the CYP11B1 gene Cortisol ↓
Apparent mineralocorticoid excess syndrome (AME) Genetic or acquired defect of 11-HSD gene Cortisol has mineralocorticoid effects Urinary free cortisone ↓↓ Dexamethasone and/or mineralocorticoid blockers
Liddle’s syndrome (Pseudohyperaldosteronism type 1) Mutation of the epithelial sodium channels (ENaC) gene in the distal renal tubulesNo extra mineralocorticoid presents, and mutations in Na channels mimic aldosterone mechanism Cortisol ↓ Amiloride or triamterene
Cushing’s syndrome
  • The main pathogenetic mechanism is linked to the excess

of cortisol which saturates 11-HSD2 activity,

Rapid weight gain, particularly of the trunk and face with limbs sparing (central obesity) Cortisol has mineralocorticoid effects
  • ↓ if excess cortisol saturates 11-HSD2 enzyme activity
Urinary free cortisol markedly ↑↑
Insensitivity to glucocorticoids (Chrousos syndrome) Mutations in glucocorticoid receptor (GR) gene Deoxycorticosterone (DOC) CortisolDexamethasone
Cortisol-secreting adrenocortical carcinomaMultifactorial

Rapid weight gain, particularly of the trunk and face with limbs sparing (central obesity)

Cortisol has mineralocorticoid effects
  • ↓ if excess cortisol saturates 11-HSD2 enzyme activity
Urinary free cortisol markedly ↑↑ Surgery
Geller’s syndrome Mutation of mineralocorticoid (MR) receptor that alters its specificity and allows progesterone to bind MR Severe hypertension particularly during pregnancyProgesterone has mineralocorticoid effects - mineralocorticoid blockers
Gordon’s syndrome (Pseudohypoaldosteronism type 2) Mutations of at least four genes have been identified, including WNK1 and WNK4 No excess mineralocorticoid; an increased activity of the thiazide-sensitive Na–Cl co-transporter in the distal tubule Normal Hyperkalemia thiazide diuretics and/or dietary sodium restriction
Exogenous causes Corticosteroids with mineralocorticoid activity Fludrocortisone or fluoroprednisolone can mimic the action of aldosterone, Medications such as fludrocortisone - Change the treatment
Licorice ingestion Glycyrrhetinic acid that binds mineralocorticoid receptor and blocks 11-HSD2 at the level of classical target tissues of aldosterone- Urinary free cortisol Moderate ↑ Discontinue licorice
Grapefruit High assumption of naringenin, a component of grapefruit, can also block 11-HSD - - Discontinue grapefruit
EstrogensEstrogens can retain sodium and water by different mechanisms, causing:
  • Increased blood pressure values and suppressing the reninaldosterone system, on the other side inducing secondary hyperaldosteronism due to the stimulation of the synthesis of angiotensinogen
- - Discontinue estrogens

Other less common causes of pseudohyperaldosteronism are:

Differentiating Diagnosis

Hyperaldosteronism should be differentiated from other diseases causing hypertension and hypokalemia for example:[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15]

 

 

 

 

 

 

 

 

Hypertension and Hypokalemia

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Plasma renin activity

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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)

History and symptoms

History

Hyperaldosteronism may be suspected in the following scenarios:

Patients with profound hypokalemia report fatigue, muscle weakness, cramping, headaches, and palpitations. They can also have polydipsia and polyuria from hypokalemia-induced nephrogenic diabetes insipidus. Long-standing HTN may lead to cardiac, retinal, renal, and neurologic problems, with all the associated symptoms and signs. Patients with primary hyperaldosteronism may have subclinical systolic dysfunction, more bradycardia, higher blood pressure and vascular resistance values than those with the secondary hyperaldosteronism. Plasmarenin activity has been found to be lower in primary than in secondary hyperaldosteronism.

Common Symptoms

Common symptoms of Hyperaldosteronism include:[22][23][24][25][26][27]

Hypertension related symptoms

Hypokalemia related symptoms

Less Common Symptoms

Less common symptoms of hyperaldosteronism include:[28][29]

References

  1. 1.01.11.2Wada N, Jin S, Hui SP, Yanagisawa K, Kurosawa T, Chiba H (2014). "[Differential diagnosis of primary aldosteronism by measurement of hybrid steroids using mass spectrometry]". Rinsho Byori (in Japanese). 62 (3): 276–82. PMID 24800505.
  2. 2.02.12.2Nielsen ML, Pareek M, Andersen I (2012). "[Liquorice-induced hypertension and hypokalaemia]". Ugeskr. Laeg. (in Danish). 174 (15): 1024–5. PMID 22487411.
  3. 3.03.13.2Chow KM, Ma RC, Szeto CC, Li PK (2012). "Polycystic kidney disease presenting with hypertension and hypokalemia". Am. J. Kidney Dis. 59 (2): 270–2. doi:10.1053/j.ajkd.2011.08.020. PMID 21962616.
  4. 4.04.14.2Sarafidis PA, Georgianos PI, Germanidis G, Giavroglou C, Nikolaidis P, Lasaridis AN, Madias NE (2012). "Hypertension and symptomatic hypokalemia in a patient with simultaneous unilateral stenoses of intrarenal arteries and mesangioproliferative glomerulonephritis". Am. J. Kidney Dis. 59 (3): 434–8. doi:10.1053/j.ajkd.2011.11.001. PMID 22154539.
  5. 5.05.15.2Khosla N, Hogan D (2006). "Mineralocorticoid hypertension and hypokalemia". Semin. Nephrol. 26 (6): 434–40. doi:10.1016/j.semnephrol.2006.10.004. PMID 17275580.
  6. 6.06.16.2Weiner ID (2013). "Endocrine and hypertensive disorders of potassium regulation: primary aldosteronism". Semin. Nephrol. 33 (3): 265–76. doi:10.1016/j.semnephrol.2013.04.007. PMC 3748390. PMID 23953804.
  7. 7.07.17.2Martell-Claros N, Abad-Cardiel M, Alvarez-Alvarez B, García-Donaire JA, Pérez CF (2015). "Primary aldosteronism and its various clinical scenarios". J. Hypertens. 33 (6): 1226–32. doi:10.1097/HJH.0000000000000546. PMID 25715092.
  8. 8.08.18.2Franse LV, Pahor M, Di Bari M, Somes GW, Cushman WC, Applegate WB (2000). "Hypokalemia associated with diuretic use and cardiovascular events in the Systolic Hypertension in the Elderly Program". Hypertension. 35 (5): 1025–30. PMID 10818057.
  9. 9.09.19.2Rossi E, Farnetti E, Nicoli D, Sazzini M, Perazzoli F, Regolisti G, Grasselli C, Santi R, Negro A, Mazzeo V, Mantero F, Luiselli D, Casali B (2011). "A clinical phenotype mimicking essential hypertension in a newly discovered family with Liddle's syndrome". Am. J. Hypertens. 24 (8): 930–5. doi:10.1038/ajh.2011.76. PMID 21525970.
  10. 10.010.110.2Ruecker B, Lang-Muritano M, Spanaus K, Welzel M, l'Allemand D, Phan-Hug F, Katschnig C, Konrad D, Holterhus PM, Schoenle EJ (2015). "The Aldosterone/Renin Ratio as a Diagnostic Tool for the Diagnosis of Primary Hypoaldosteronism in Newborns and Infants". Horm Res Paediatr. 84 (1): 43–8. doi:10.1159/000381852. PMID 25968592.
  11. 11.011.111.2Ardhanari S, Kannuswamy R, Chaudhary K, Lockette W, Whaley-Connell A (2015). "Mineralocorticoid and apparent mineralocorticoid syndromes of secondary hypertension". Adv Chronic Kidney Dis. 22 (3): 185–95. doi:10.1053/j.ackd.2015.03.002. PMID 25908467.
  12. 12.012.112.2Iglesias P, Tajada P, Martínez I, Díez JJ (2009). "[Salt-wasting congenital adrenal hyperplasia associated to hyperreninemic hyperaldosteronism]". Med Clin (Barc) (in Spanish; Castilian). 132 (2): 80–1. doi:10.1016/j.medcli.2008.09.002. PMID 19174076.
  13. 13.013.113.2Kikuta Y, Sanjo K, Nakajima K, Ashizawa I, Ojima M (1988). "Primary aldosteronism in childhood due to primary adrenal hyperplasia". Tohoku J. Exp. Med. 155 (1): 57–70. PMID 3413779.
  14. 14.014.114.2Hassan-Smith Z, Stewart PM (2011). "Inherited forms of mineralocorticoid hypertension". Curr Opin Endocrinol Diabetes Obes. 18 (3): 177–85. doi:10.1097/MED.0b013e3283469444. PMID 21494136.
  15. 15.015.115.2Bartter FC, Henkin RI, Bryan GT (1968). "Aldosterone hypersecretion in "non-salt-losing" congenital adrenal hyperplasia". J. Clin. Invest. 47 (8): 1742–52. doi:10.1172/JCI105864. PMC 297334. PMID 4299011.
  16. Stewart PM (2003). "Tissue-specific Cushing's syndrome, 11beta-hydroxysteroid dehydrogenases and the redefinition of corticosteroid hormone action". Eur. J. Endocrinol. 149 (3): 163–8. PMID 12943516.
  17. Quinkler M, Stewart PM (2003). "Hypertension and the cortisol-cortisone shuttle". J. Clin. Endocrinol. Metab. 88 (6): 2384–92. doi:10.1210/jc.2003-030138. PMID 12788832.
  18. Ulick S, Wang JZ, Blumenfeld JD, Pickering TG (1992). "Cortisol inactivation overload: a mechanism of mineralocorticoid hypertension in the ectopic adrenocorticotropin syndrome". J. Clin. Endocrinol. Metab. 74 (5): 963–7. doi:10.1210/jcem.74.5.1569172. PMID 1569172.
  19. Whorwood CB, Sheppard MC, Stewart PM (1993). "Licorice inhibits 11 beta-hydroxysteroid dehydrogenase messenger ribonucleic acid levels and potentiates glucocorticoid hormone action". Endocrinology. 132 (6): 2287–92. doi:10.1210/endo.132.6.8504732. PMID 8504732.
  20. CHRISTY NP, LARAGH JH (1961). "Pathogenesis of hypokalemic alkalosis in Cushing's syndrome". N. Engl. J. Med. 265: 1083–8. doi:10.1056/NEJM196111302652203. PMID 13879332.
  21. 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.
  22. Rubidge CJ, O'Dowd PB, Powell SJ (1973). "Difetarsone in the treatment of Trichuris trichiura infections". S. Afr. Med. J. 47 (23): 991–2. PMID 4714286.
  23. Mattsson C, Young WF (2006). "Primary aldosteronism: diagnostic and treatment strategies". Nat Clin Pract Nephrol. 2 (4): 198–208, quiz, 1 p following 230. doi:10.1038/ncpneph0151. PMID 16932426.
  24. Di Tullio M, Alli C, Avanzini F, Bettelli G, Colombo F, Devoto MA, Marchioli R, Mariotti G, Radice M, Taioli E (1988). "Prevalence of symptoms generally attributed to hypertension or its treatment: study on blood pressure in elderly outpatients (SPAA)". J Hypertens Suppl. 6 (1): S87–90. PMID 3216243.
  25. Unwin RJ, Luft FC, Shirley DG (2011). "Pathophysiology and management of hypokalemia: a clinical perspective". Nat Rev Nephrol. 7 (2): 75–84. doi:10.1038/nrneph.2010.175. PMID 21278718.
  26. Bautista J, Gil-Neciga E, Gil-Peralta A (1979). "Hypokalemic periodic paralysis in primary hyperaldosteronism. Subclinical myopathy with atrophy of the type 2A muscle fibers". Eur. Neurol. 18 (6): 415–20. PMID 546663.
  27. Bortolotto LA, Cesena FH, Jatene FB, Silva HB (2003). "Malignant hypertension and hypertensive encephalopathy in primary aldosteronism caused by adrenal adenoma". Arq. Bras. Cardiol. 81 (1): 97–100, 93–6. PMID 12908077.
  28. Moeller J, Muniz B (1967). "[Hypokalemic ileus and aldosteronism]". Med Klin (in German). 62 (52): 2019–24. PMID 5596496.
  29. Failor RA, Capell PT (2003). "Hyperaldosteronism and pheochromocytoma: new tricks and tests". Prim. Care. 30 (4): 801–20, viii. PMID 15024897.

Endocrinepathology: endocrine diseases (E00-35, 240-259)

Thyroid

Hypothyroidism (Iodine deficiency, Cretinism, Congenital hypothyroidism, Goitre, Myxedema) - Hyperthyroidism (Graves disease, Toxic multinodular goitre, Teratoma with thyroid tissue or Struma ovarii) - Thyroiditis (De Quervain's thyroiditis, Hashimoto's thyroiditis, Riedel's thyroiditis) - Euthyroid sick syndrome

Pancreas

Diabetes mellitus (type 1, type 2, coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy) - Hypoglycemia - Hyperinsulinism - Zollinger-Ellison syndrome - insulin receptor (Rabson-Mendenhall syndrome)

Parathyroid

Hypoparathyroidism (Pseudohypoparathyroidism) - Hyperparathyroidism (Primary, Secondary, Tertiary)

Pituitary

Hyperpituitarism (Acromegaly, Hyperprolactinaemia, SIADH) - Hypopituitarism (Simmonds' disease / Sheehan's syndrome, Kallmann syndrome, Growth hormone deficiency, Diabetes insipidus) - Adiposogenital dystrophy - Empty sella syndrome - Hypophysitis

Adrenal

Cushing's syndrome (Nelson's syndrome, Pseudo-Cushing's syndrome) - CAH (Lipoid, 3β, 11β, 17α, 21α) - Hyperaldosteronism (Conn syndrome, Bartter syndrome) - Adrenal insufficiency (Addison's disease) - Hypoaldosteronism

Gonads

Ovarian dysfunction (Polycystic ovary syndrome, Premature ovarian failure) - Testicular dysfunction (5-alpha-reductase deficiency) - Testosterone biosynthesis (17-beta-hydroxysteroid dehydrogenase deficiency) - General (Hypogonadism, Delayed puberty, Precocious puberty)

Other

Androgen insensitivity syndrome - Autoimmune polyendocrine syndrome - Carcinoid syndrome - Gigantism - Short stature (Laron syndrome, Psychogenic dwarfism) - Multiple endocrine neoplasia (1, 2) - Progeria - Woodhouse-Sakati syndrome

de:Hyperaldosteronismusit:Iperaldosteronismo


Template:WikiDoc Sources

Sours: https://www.wikidoc.org/index.php/Hyperaldosteronism

Wiki conn syndrome

Primary aldosteronism

Medical condition

Primary aldosteronism
Other namesPrimary hyperaldosteronism, Conn's syndrome
Aldosterone-2D-skeletal.svg
Aldosterone
SpecialtyEndocrinology
SymptomsHigh blood pressure, poor vision, headaches, muscular weakness, muscle spasms[1][2]
ComplicationsStroke, myocardial infarction, kidney failure, abnormal heart rhythms[3][4]
Usual onset30 to 50 years old[5]
CausesEnlargement of both adrenal glands, adrenal adenoma, adrenal cancer, familial hyperaldosteronism[6][1]
Diagnostic methodBlood test for aldosterone-to-renin ratio[1]
TreatmentSurgery, spironolactone, eplerenone, low salt diet[1]
Frequency10% of people with high blood pressure[1]

Primary aldosteronism (PA), also known as primary hyperaldosteronism or Conn's syndrome, refers to the excess production of the hormonealdosterone from the adrenal glands, resulting in low renin levels and high blood pressure.[1] This abnormality is caused by hyperplasia or tumors. Many suffer from fatigue, potassium deficiency and high blood pressure which may cause poor vision, confusion or headaches.[1][2] Symptoms may also include: muscular aches and weakness, muscle spasms, low back and flank pain from the kidneys, trembling, tingling sensations, dizziness/vertigo, nocturia and excessive urination.[1] Complications include cardiovascular disease such as stroke, myocardial infarction, kidney failure and abnormal heart rhythms.[3][4]

Primary hyperaldosteronism has a number of causes. About 33% of cases are due to an adrenal adenoma that produces aldosterone, and 66% of cases are due to an enlargement of both adrenal glands.[1] Other uncommon causes include adrenal cancer and an inherited disorder called familial hyperaldosteronism.[6] Some recommend screening people with high blood pressure who are at increased risk, while others recommend screening all people with high blood pressure for the disease.[3] Screening is usually done by measuring the aldosterone-to-renin ratio in the blood (ARR) whilst off interfering medications and a serum potassium over 4, with further testing used to confirm positive results.[1] While low blood potassium is classically described in primary hyperaldosteronism, this is only present in about a quarter of people.[1] To determine the underlying cause, medical imaging is carried out.[1]

Some cases may be cured by removing the adenoma by surgery after localization with adrenal venous sampling (AVS).[1][7] A single adrenal gland may also be removed in cases where only one is enlarged.[4] In cases due to enlargement of both glands, treatment is typically with medications known as aldosterone antagonists such as spironolactone or eplerenone.[1] Other medications for high blood pressure and a low salt diet, e.g. DASH diet, may also be needed.[1][4] Some people with familial hyperaldosteronism may be treated with the steroid dexamethasone.[1]

Primary aldosteronism is present in about 10% of people with high blood pressure.[1] It occurs more often in women than men.[5] Often, it begins in those between 30 and 50 years of age.[5] Conn's syndrome is named after Jerome W. Conn (1907–1994), an Americanendocrinologist who first described adenomas as a cause of the condition in 1955.[8][9]

Signs and symptoms[edit]

People often have few or no symptoms.[1] They may get occasional muscular weakness, muscle spasms, tingling sensations, or excessive urination.[1]

High blood pressure, manifestations of muscle cramps (due to hyperexcitability of neurons secondary to low blood calcium), muscle weakness (due to hypoexcitability of skeletal muscles secondary to hypokalemia), and headaches (due to low blood potassium or high blood pressure) may be seen.[citation needed]

Secondary hyperaldosteronism is often related to decreased cardiac output, which is associated with elevated renin levels.[10]

Causes[edit]

Two slices of an adrenal gland with a cortical adenoma, from a person with Conn's syndrome

The condition is due to:[11]

  • Bilateral idiopathic (micronodular) adrenal hyperplasia: 66% of cases[1]
  • Adrenal adenoma (Conn's disease): 33% of cases[1]
  • Primary (unilateral) adrenal hyperplasia: 2% of cases
  • Aldosterone-producing adrenocortical carcinoma: <1% of cases
  • Familial Hyperaldosteronism (FH)
    • Glucocorticoid-remediable aldosteronism (FH type I): <1% of cases
    • FH type II (APA or IHA): <2% of cases
  • Ectopic aldosterone-producing adenoma or carcinoma: < 0.1% of cases

Genetics[edit]

40% of people with an adrenal aldosterone producing adenoma have somatic gain-of-function mutations in a single gene (KCNJ5).[12] This gene is mutated in inherited cases of early onset primary aldosteronism and bilateral adrenal hyperplasia, albeit less frequently.[13] These mutations tend to occur in young women with the adenoma in the cortisol secreting zona fasciculata. Adenomas without this mutation tend to occur in older men with resistant hypertension.[citation needed]

Other genes commonly mutated in aldosterone producing adenomas are ATP1A1[14][15]ATP2B3,[14]CACNA1D,[15] and CTNNB1.[16]

Pathophysiology[edit]

Aldosterone has effects on most or all cells of the body but, clinically, the most important actions are in the kidney, on cells of the late distal convoluted tubule and medullary collecting duct. In the principal cells aldosterone increases activity of basolateral membrane sodium-potassium ATPase and apical epithelial sodium channels, ENaC, as well as potassium channels, ROMK. These actions increase sodium reabsorption and potassium secretion. Since more sodium is reabsorbed than potassium secreted, it also makes the lumen more electrically negative, causing chloride to follow sodium. Water then follows sodium and chloride by osmosis. In Conn syndrome, these actions cause increased extracellular sodium and fluid volume and reduced extracellular potassium. Aldosterone also acts on intercalated cells to stimulate an apical proton ATPase, causing proton secretion that acidifies urine and alkalizes extracellular fluid.[citation needed]

In summary, hyperaldosteronism causes hypernatremia, hypokalemia, and metabolic alkalosis.[10]

Finer notes on aldosterone include the fact that it stimulates sodium-potassium ATPase in muscle cells, increasing intracellular potassium and also increases sodium reabsorption all along the intestine and nephron, possibly due to widespread stimulation of sodium-potassium ATPase. Finally, epithelial cells of sweat gland ducts and distal colon surface respond exactly the same as the principal cells of the nephron. These responses are important in climate adaptation and as a cause of constipation with elevated aldosterone[citation needed].

The sodium retention leads to plasma volume expansion and elevated blood pressure. The increased blood pressure will lead to increased glomerular filtration rate and cause a decrease in renin released from the granular cells of the juxtaglomerular apparatus in the kidney decreasing sodium reabsorption and returning sodium renal excretion to near normal levels allowing sodium to 'escape' the effect of mineralocorticoids (also known as Aldosterone escape mechanism in primary hyperaldosteronism also contributed to by increased ANP level). If there is primary hyperaldosteronism, the decreased renin (and subsequent decreased angiotensin II) will not lead to a decrease in aldosterone levels (a very helpful clinical tool in diagnosis of primary hyperaldosteronism).[10]

Diagnosis[edit]

Screening may be considered in people with high blood pressure presenting with low blood potassium, high blood pressure that is difficult to treat, other family members with the same condition, or a mass on the adrenal gland.[1]

Measuring aldosterone alone is not considered adequate to diagnose primary hyperaldosteronism. Rather, both renin and aldosterone are measured, and a resultant aldosterone-to-renin ratio (ARR) is used for case detection.[17][18] A high aldosterone-to-renin ratio suggests the presence of primary hyperaldosteronism. The diagnosis is made by performing a saline suppression test, ambulatory salt loading test, or fludrocortisone suppression test.[19]

Measuring sodium and potassium concentrations simultaneously in serum and urine specimens has been suggested for screening purposes. Calculating the serum sodium over urinary sodium to serum potassium over urinary potassium (SUSPUP) and the (serum sodium to urinary sodium to (serum potassium)2 (SUSPPUP) ratios delivers calculated structure parameters of the RAAS, which may be used as a static function test.[20][21] Its results have to be confirmed by calculating the ARR.[citation needed]

If primary hyperaldosteronism is confirmed biochemically, CT scanning or other cross-sectional imaging can confirm the presence of an adrenal abnormality, possibly an adrenal cortical adenoma (aldosteronoma), adrenal carcinoma, bilateral adrenal hyperplasia, or other less common changes. Imaging findings may ultimately lead to other necessary diagnostic studies, such as adrenal venous sampling, to clarify the cause. It is not uncommon for adults to have bilateral sources of aldosterone hypersecretion in the presence of a nonfunctioning adrenal cortical adenoma, making adrenal venous sampling (AVS) mandatory in cases where surgery is being considered.[19][7] For cases where AVS is unable to provide lateralisation of the source/sources of aldosterone hypersecretion, PET/CT using the radiotracer 11C-Metomidate is an option. Since 11C-Metomidate is unspecific for CYP11B1/CYP11B2 the patient needs pre-treatment with dexamethasone to downregulate the expression of CYP11B1.[22][23][24]

The diagnosis is best accomplished by an appropriately-trained subspecialist, though primary care providers are critical in recognizing clinical features of primary aldosteronism and obtaining the first blood tests for case detection.[citation needed]

Classification[edit]

Some people only use Conn's syndrome for when it occurs due to an adrenal adenoma (a type of benign tumor).[25] In practice, however, the terms are often used interchangeably, regardless of the underlying physiology.[1]

Differential diagnosis[edit]

Primary hyperaldosteronism can be mimicked by Liddle syndrome, and by ingestion of licorice and other foods containing glycyrrhizin. In one case report, hypertension and quadriparesis resulted from intoxication with a non-alcoholic pastis (an anise-flavored aperitif containing glycyrrhizinic acid).[26]

Treatment[edit]

The treatment for hyperaldosteronism depends on the underlying cause. In people with a single benign tumor (adenoma), surgical removal (adrenalectomy) may be curative. This is usually performed laparoscopically, through several very small incisions. For people with hyperplasia of both glands, successful treatment is often achieved with spironolactone or eplerenone, drugs that block the effect of aldosterone. With its antiandrogen effect, spironolactone drug therapy may have a range of side effects in males and females, including gynecomastia and irregular menses. These symptoms occur less frequently with eplerenone drug therapy.[27]

In the absence of treatment, individuals with hyperaldosteronism often have poorly controlled high blood pressure, which may be associated with increased rates of stroke, heart disease, and kidney failure. With appropriate treatment, the prognosis is considered good.[28]

Esaxerenone, the first non-steroidal mineralocorticoid blocker, was approved in 2019 in Japan to treat essential hypertension. Finerenone, a drug belonging to the same class, reached phase 3 clinical trial in 2020, but is not yet considered for hypertension. More importantly, next-generation Aldosterone Synthase Inhibitors have entered the research pipeline with CIN-107 undergoing Phase 2 clinical trial as of 2021[29]

Society and culture[edit]

The Primary Aldosteronism Foundation[30] is a patient-driven initiative committed to creating the paradigm shift that will lead to optimum diagnosis and treatment of primary aldosteronism by raising awareness, fostering research, and providing support to patients and healthcare professionals worldwide.[citation needed]

Eponym[edit]

Conn's syndrome is named after Jerome W. Conn (1907–1994), the Americanendocrinologist who first described the condition at the University of Michigan in 1955.[8]

References[edit]

  1. ^ abcdefghijklmnopqrstuvwSchirpenbach C, Reincke M (March 2007). "Primary aldosteronism: current knowledge and controversies in Conn's syndrome". Nature Clinical Practice Endocrinology & Metabolism. 3 (3): 220–7. doi:10.1038/ncpendmet0430. PMID 17315030. S2CID 23220252.
  2. ^ ab"Primary hyperaldosteronism (Conn's syndrome or aldosterone-producing adrenal tumor)". Archived from the original on 19 April 2015. Retrieved 8 April 2015.
  3. ^ abcStowasser M, Taylor PJ, Pimenta E, Ahmed AH, Gordon RD (May 2010). "Laboratory investigation of primary aldosteronism". The Clinical Biochemist. Reviews. 31 (2): 39–56. PMC 2874431. PMID 20498828.
  4. ^ abcd"Primary hyperaldosteronism (Conn's syndrome or aldosterone-producing adrenal tumor)". Archived from the original on 28 March 2015. Retrieved 8 April 2015.
  5. ^ abcHubbard JG, Inabnet WB, Heerden CL (2009). Endocrine surgery principles and practice. London: Springer. p. 367. ISBN . Archived from the original on 2016-06-30.
  6. ^ ab"Primary hyperaldosteronism (Conn's syndrome or aldosterone-producing adrenal tumor)". Archived from the original on 9 April 2015. Retrieved 8 April 2015.
  7. ^ abCusumano, Lucas R.; Mathevosian, Sipan; Sweigert, Joshua K.; Srinivasa, Ravi N.; Luhar, Aarti P.; Moriarty, John M. (January 2021). "Use of Bony Landmarks during Adrenal Venous Sampling to Guide Catheterization of the Left Adrenal Vein". The Arab Journal of Interventional Radiology. 5 (1): 25–29. doi:10.1055/s-0041-1730113. ISSN 2542-7075.
  8. ^ abConn JW, Louis LH (1955). "Primary aldosteronism: a new clinical entity". Transactions of the Association of American Physicians. 68: 215–31, discussion, 231–3. PMID 13299331.
  9. ^Williams GH (2009). Textbook of nephro-endocrinology. Amsterdam: Academic. p. 372. ISBN . Archived from the original on 2016-06-30.
  10. ^ abc"Hyperaldosteronism". The Lecturio Medical Concept Library. Retrieved 25 July 2021.
  11. ^Kronenberg HM (2008). Williams textbook of endocrinology (11th ed.). Philadelphia: Saunders/Elsevier. ISBN .
  12. ^Brown MJ (September 2012). "Platt versus Pickering: what molecular insight to primary hyperaldosteronism tells us about hypertension". JRSM Cardiovascular Disease. 1 (6): 1–8. doi:10.1258/cvd.2012.012020. PMC 3738367. PMID 24175075.
  13. ^Choi M, Scholl UI, Yue P, Björklund P, Zhao B, Nelson-Williams C, Ji W, Cho Y, Patel A, Men CJ, Lolis E, Wisgerhof MV, Geller DS, Mane S, Hellman P, Westin G, Åkerström G, Wang W, Carling T, Lifton RP (February 2011). "K+ channel mutations in adrenal aldosterone-producing adenomas and hereditary hypertension". Science. 331 (6018): 768–72. Bibcode:2011Sci...331..768C. doi:10.1126/science.1198785. PMC 3371087. PMID 21311022.
  14. ^ abBeuschlein F, Boulkroun S, Osswald A, Wieland T, Nielsen HN, Lichtenauer UD, et al. (April 2013). "Somatic mutations in ATP1A1 and ATP2B3 lead to aldosterone-producing adenomas and secondary hypertension". Nature Genetics. 45 (4): 440–4, 444e1–2. doi:10.1038/ng.2550. PMID 23416519. S2CID 205346722.
  15. ^ abAzizan EA, Poulsen H, Tuluc P, Zhou J, Clausen MV, Lieb A, et al. (September 2013). "Somatic mutations in ATP1A1 and CACNA1D underlie a common subtype of adrenal hypertension". Nature Genetics. 45 (9): 1055–60. doi:10.1038/ng.2716. PMID 23913004. S2CID 205347424.
  16. ^Åkerström T, Maharjan R, Sven Willenberg H, Cupisti K, Ip J, Moser A, Stålberg P, Robinson B, Alexander Iwen K, Dralle H, Walz MK, Lehnert H, Sidhu S, Gomez-Sanchez C, Hellman P, Björklund P (January 2016). "Activating mutations in CTNNB1 in aldosterone producing adenomas". Scientific Reports. 6: 19546. Bibcode:2016NatSR...619546A. doi:10.1038/srep19546. PMC 4728393. PMID 26815163.
  17. ^Tiu SC, Choi CH, Shek CC, Ng YW, Chan FK, Ng CM, Kong AP (January 2005). "The use of aldosterone-renin ratio as a diagnostic test for primary hyperaldosteronism and its test characteristics under different conditions of blood sampling". The Journal of Clinical Endocrinology and Metabolism. 90 (1): 72–8. doi:10.1210/jc.2004-1149. PMID 15483077.
  18. ^United Bristol Healthcare NHS Trust, the major teaching trust in South West EnglandArchived 2007-08-13 at archive.today
  19. ^ abFunder, John W.; Carey, Robert M.; Fardella, Carlos; Gomez-Sanchez, Celso E.; Mantero, Franco; Stowasser, Michael; Young, William F.; Montori, Victor M. (2008). "Case Detection, Diagnosis, and Treatment of Patients with Primary Aldosteronism". The Journal of Clinical Endocrinology & Metabolism. 93 (9): 3266–3281. doi:10.1210/jc.2008-0104. PMID 18552288.
  20. ^Willenberg, HS; Kolentini, C; Quinkler, M; Cupisti, K; Krausch, M; Schott, M; Scherbaum, WA (January 2009). "The serum sodium to urinary sodium to (serum potassium)2 to urinary potassium (SUSPPUP) ratio in patients with primary aldosteronism". European Journal of Clinical Investigation. 39 (1): 43–50. doi:10.1111/j.1365-2362.2008.02060.x. PMID 19067735. S2CID 25616329.
  21. ^Yin, GS; Zhang, SL; Yan, L; Li, F; Qi, YQ; Chen, ZC; Cheng, H (13 April 2010). "[New index of using serum sodium and potassium and urine sodium and potassium jointly in screening primary aldosteronism in hypertensive patients]". Zhonghua Yi Xue Za Zhi. 90 (14): 962–6. PMID 20646645.
  22. ^Chen Cardenas, Stanley M.; Santhanam, Prasanna (4 September 2020). "11C-metomidate PET in the diagnosis of adrenal masses and primary aldosteronism: a review of the literature". Endocrine. 70 (3): 479–487. doi:10.1007/s12020-020-02474-3. ISSN 1559-0100. PMID 32886316. S2CID 221479027.
  23. ^O'Shea, Paula M.; O'Donoghue, Darragh; Bashari, Waiel; Senanayake, Russell; Joyce, Mary B.; Powlson, Andrew S.; Browne, Darragh; O'Sullivan, Gerard J.; Cheow, Heok; Mendichovszky, Iosif; Quill, Denis (18 March 2019). "11 C-Metomidate PET/CT is a useful adjunct for lateralization of primary aldosteronism in routine clinical practice". Clinical Endocrinology. 90 (5): 670–679. doi:10.1111/cen.13942. ISSN 1365-2265. PMID 30721535. S2CID 73450281.
  24. ^Zhou, Yaqiong; Wang, Dan; Jiang, Licheng; Ran, Fei; Chen, Sichao; Zhou, Peng; Wang, Peijian (31 December 2020). "Diagnostic accuracy of adrenal imaging for subtype diagnosis in primary aldosteronism: systematic review and meta-analysis". BMJ Open. 10 (12): e038489. doi:10.1136/bmjopen-2020-038489. ISSN 2044-6055. PMC 7780716. PMID 33384386.
  25. ^Cotran RS, Kumar V, Fausto N, Nelso F, Robbins SL, Abbas AK (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. p. 1210. ISBN .
  26. ^Trono D, Cereda JM, Favre L (August 1983). "[Pseudo-Conn's syndrome due to intoxication with nonalcoholic pastis]". Schweizerische Medizinische Wochenschrift (in French). 113 (31–32): 1092–5. PMID 6623028.
  27. ^"Inspra (eplerenone) [prescribing information]". Archived from the original on 2011-08-08. Retrieved 2011-07-17.
  28. ^Columbia Adrenal Center, Hyperaldosteronism (Conn's Syndrome)Archived 2011-05-26 at the Wayback Machine
  29. ^"Spark-PA – Spark-PA is a clinical research study exploring an investigational study drug that may help people with primary aldosteronism (PA) lower their blood pressure". Retrieved 2021-03-05.
  30. ^"Welcome to the Primary Aldosteronism Foundation". Retrieved 2021-03-05.

External links[edit]

  • Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, Stowasser M, Young WF (May 2016). "The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline". The Journal of Clinical Endocrinology and Metabolism. 101 (5): 1889–916. doi:10.1210/jc.2015-4061. PMID 26934393.
Sours: https://en.wikipedia.org/wiki/Primary_aldosteronism
Hyperaldosteronism and Conn's Syndrome

Primary aldosteronism or Conn's syndrome

Primary aldosteronism or Conn's syndrome is any of a number of disorders that directly cause the adrenal glandsto overproduce aldosterone. The symtoms of the condition tend to be mild and prosaic such as high blood pressure, poor vision, headaches, muscleweakness, spasms, peripheral neuropathyand excess urination.

About two-thirds of cases are caused by an enlargement of the adrenal glands, while the majority of the remainder are caused by a tumor on the adrenals. More rarely, it is caused by adrenal cancer or by a genetic condition.

About 10% of patients with high blood pressure also have Conn's. It is more common in women, and usually is first diagnosed in patients over 30 but under 50. In addition, about 40% of patients have been identified with a common gene even in non-genetic cases.

The condition can only be accurately diagnosed by measuring the ratio of aldosterone in the blood to the level of renin, an enzyme. The condition is typified by a very high ratio between the two. In most cases, medical imaging is performed to pinpoint the cause.

Treatment depends on the underlying cause. If a tumor is the cause, surgical removal is recommended. If only one adrenal gland is enlarged, it is usually removed. However, if both are enlarged, drug therapy is usually recommended.

Conn's syndrome at Wikipedia

Sours: https://house.fandom.com/wiki/Conn%27s_syndrome

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Aldosterone.svg|

Conn's syndrome is characterized by the overproduction of the mineralocorticoidhormonealdosterone by the adrenal glands. Aldosterone causes sodium and water retention and potassium excretion in the kidneys, leading to arterial hypertension (high blood pressure). It is a rare but recognised cause of hypertension.

Signs, symptoms and findings[]

Conn's syndrome is also known as primary hyperaldosteronism. Apart from high blood pressure, the symptoms may include muscle cramps (due to hyperexcitability of neurons), muscle weakness (due to hypoexcitability of skeletal muscles) and headaches (due to the low potassium), metabolic alkalosis (due to increased secretion of H+ ions by the kidney). The high pH of the blood makes calcium less available to the tissues and causes symptoms of hypocalcemia (low calcium levels).

It can be mimicked by liquorice ingestion (glycyrrhizin) and Liddle syndrome.

Diagnosis[]

Measuring aldosterone alone is not considered adequate to diagnose Conn's syndrome. Rather, both renin and aldosterone are measured, and the ratio is diagnostic.[1][2]

Usually, renin levels are suppressed, leading to a very low renin-aldosterone ratio (<0.0005). This test is confounded by antihypertensive drugs, which have to be stopped up to 6 weeks.

If there is biochemic proof of hyperaldosteronism, CT scanning can confirm the presence of an adrenal adenoma.

Causes[]

The syndrome is due to:

  • aldosterone-secreting adrenal adenoma (benign tumor, 50-60%)
  • hyperplasia of the adrenal gland (40-50%)
  • rare forms

Therapy[]

The treatment for hyperaldosteronism depends on the underlying cause. In patients with a single benign tumor (adenoma), surgical removal (adrenalectomy) is curative. This is usually performed laparoscopically, through several very small incisions. For patients with hyperplasia of both glands, successful treatment is often achieved with spironolactone or eplerenone, drugs that block the effect of aldosterone.

In the absence of proper treatment, individuals with hyperaldosteronism often suffer from poorly controlled high blood pressure, which may be associated with increased rates of stroke, heart disease, and kidney failure. With appropriate treatment, the prognosis is excellent.Cite error: Closing missing for tag

References[]

v·d·e

Endocrinepathology of psychological interest (E00-35)

thyroidHypothyroidism (Iodine deficiency, Cretinism, Congenital hypothyroidism, Goitre) - Hyperthyroidism (Graves-Basedow disease, Toxic multinodular goitre) - Thyroiditis (De Quervain's thyroiditis, Hashimoto's thyroiditis)
pancreasDiabetes mellitus (type 1, type 2, coma, angiopathy, neuropathy, retinopathy) - Zollinger-Ellison syndrome
parathyroidHypoparathyroidism - Hyperparathyroidism
pituitary hyperfunction (Acromegaly, Hyperprolactinaemia, ) - HypopituitarismKallmann syndrome, Growth hormone deficiency) - Hypothalamic-pituitary dysfunction
adrenalCushing's syndrome) - Congenital adrenal hyperplasia (due to 21-hydroxylase deficiency) - Bartter syndrome) - Adrenal insufficiency (Addison's disease)
gonads - 5-alpha-reductase deficiency - Hypogonadism - Delayed puberty - Precocious puberty
other - - Psychogenic dwarfism - Androgen insensitivity syndrome -

Sours: https://psychology.wikia.org/wiki/Conn_syndrome


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