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Start free with EleplanFamilial hyperaldosteronism type I
ORPHA:403Disease
Also called Dexamethasone-sensitive hypertension · FH-I · FH1 · Familial hyperaldosteronism type 1 · GRA · Glucocorticoid-remediable aldosteronism · Glucocorticoid-sensitive hypertension
What it is
A rare heritable, glucocorticoid remediable form of primary aldosteronism (PA) characterized by early-onset hypertension, hyperaldosteronism, variable hypokalemia, low plasma renin activity (PRA), and abnormal production of 18-oxocortisol and 18-hydroxycortisol.
Key facts
- Age of onset
- Adolescent, Adult, Childhood
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Signs and symptoms
Common30–79%
3These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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