Overview
Familial hyperreninemic hypoaldosteronism type 2 is an extremely rare inherited condition that affects the adrenal glands and how the body manages salt and water balance. In this condition, the body produces too much renin (a hormone made by the kidneys that helps regulate blood pressure) but too little aldosterone (a hormone made by the adrenal glands that helps the body hold onto sodium and get rid of potassium). This imbalance can lead to problems with electrolytes in the blood, particularly low sodium and high potassium levels. Patients may experience symptoms such as low blood pressure, dehydration, muscle weakness, fatigue, and salt craving. In severe cases, dangerously high potassium levels can affect heart rhythm. This entry is now classified as 'OBSOLETE' in the Orphanet database, meaning it may have been reclassified, merged with another condition, or its classification has been updated as scientific understanding has improved. It may now fall under a broader category of familial hypoaldosteronism or pseudohypoaldosteronism. Patients who were previously diagnosed with this condition should consult with an endocrinologist or clinical geneticist to determine the current classification and appropriate management. Treatment typically involves replacing the missing aldosterone with a medication called fludrocortisone and ensuring adequate salt intake. Monitoring electrolyte levels is essential to prevent dangerous complications.
Also known as:
Key symptoms:
Low blood pressureDehydrationHigh potassium levels in the bloodLow sodium levels in the bloodMuscle weaknessFatigue and tirednessSalt cravingPoor weight gain in infantsNausea or vomitingDizziness or lightheadednessIrregular heartbeat due to high potassiumFailure to thrive in young children
Autosomal recessive
Passed on when both parents carry the same gene change; often skips generations
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for OBSOLETE: Familial hyperreninemic hypoaldosteronism type 2.
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Specialists
View all specialists →No specialists are currently listed for OBSOLETE: Familial hyperreninemic hypoaldosteronism type 2.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to OBSOLETE: Familial hyperreninemic hypoaldosteronism type 2.
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Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What is the current classification of my condition, since this diagnosis is now considered obsolete?,What dose of fludrocortisone do I need, and how often should my electrolytes be checked?,Should I or my family members undergo genetic testing?,What are the warning signs of a dangerous potassium level, and when should I go to the emergency room?,How much extra salt should I include in my diet?,Do I need to take extra precautions during illness, surgery, or physical activity?,Are there any new treatments or clinical trials available for this condition?
Common questions about OBSOLETE: Familial hyperreninemic hypoaldosteronism type 2
What is OBSOLETE: Familial hyperreninemic hypoaldosteronism type 2?
Familial hyperreninemic hypoaldosteronism type 2 is an extremely rare inherited condition that affects the adrenal glands and how the body manages salt and water balance. In this condition, the body produces too much renin (a hormone made by the kidneys that helps regulate blood pressure) but too little aldosterone (a hormone made by the adrenal glands that helps the body hold onto sodium and get rid of potassium). This imbalance can lead to problems with electrolytes in the blood, particularly low sodium and high potassium levels. Patients may experience symptoms such as low blood pressure, d
How is OBSOLETE: Familial hyperreninemic hypoaldosteronism type 2 inherited?
OBSOLETE: Familial hyperreninemic hypoaldosteronism type 2 follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.