Rare non surgically correctable form of primary aldosteronism

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Overview

Rare non-surgically correctable forms of primary aldosteronism encompass a group of conditions in which the adrenal glands produce excessive amounts of the hormone aldosterone, but unlike aldosterone-producing adenomas, these forms cannot be treated with surgical removal of a single adrenal gland. This category includes conditions such as bilateral adrenal hyperplasia (also called idiopathic hyperaldosteronism) and familial forms of hyperaldosteronism (including familial hyperaldosteronism types I, II, III, and IV). The excess aldosterone acts on the kidneys, causing the body to retain sodium and water while losing potassium, leading to high blood pressure (hypertension) that is often resistant to standard treatments, low potassium levels (hypokalemia), metabolic alkalosis, and associated symptoms such as muscle weakness, fatigue, headaches, and in some cases cardiac complications. The condition primarily affects the cardiovascular system, renal system, and electrolyte balance. Because surgical correction is not an option for these bilateral or diffuse forms, management relies on medical therapy, most notably mineralocorticoid receptor antagonists such as spironolactone or eplerenone, which block the effects of excess aldosterone. Additional antihypertensive medications may be required to achieve adequate blood pressure control. In familial hyperaldosteronism type I (glucocorticoid-remediable aldosteronism), low-dose glucocorticoids such as dexamethasone can suppress excess aldosterone production. Dietary sodium restriction and potassium supplementation may also be part of the management plan. Long-term monitoring is essential to prevent cardiovascular complications including stroke, heart failure, and kidney damage associated with chronic aldosterone excess.

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

FDA & Trial Timeline

1 event
Jul 1993

Orlaam: FDA approved

For the management of opiate dependence.

FDAcompleted

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

1 available

Orlaam

Levomethadyl acetate hydrochloride· Biodevelopment CorporationOrphan Drug

For the management of opiate dependence.

No actively recruiting trials found for Rare non surgically correctable form of primary aldosteronism at this time.

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No specialists are currently listed for Rare non surgically correctable form of primary aldosteronism.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Rare non surgically correctable form of primary aldosteronism.

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Common questions about Rare non surgically correctable form of primary aldosteronism

What is Rare non surgically correctable form of primary aldosteronism?

Rare non-surgically correctable forms of primary aldosteronism encompass a group of conditions in which the adrenal glands produce excessive amounts of the hormone aldosterone, but unlike aldosterone-producing adenomas, these forms cannot be treated with surgical removal of a single adrenal gland. This category includes conditions such as bilateral adrenal hyperplasia (also called idiopathic hyperaldosteronism) and familial forms of hyperaldosteronism (including familial hyperaldosteronism types I, II, III, and IV). The excess aldosterone acts on the kidneys, causing the body to retain sodium

What treatment and support options exist for Rare non surgically correctable form of primary aldosteronism?

1 patient support program are currently tracked on UniteRare for Rare non surgically correctable form of primary aldosteronism. See the treatments and support programs sections for copay assistance, eligibility, and contact details.