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Familial Hyperaldosteronism Type 1

Glucocorticoid-remediable aldosteronism (GRA).

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is Familial Hyperaldosteronism Type 1?

Familial hyperaldosteronism type 1 (FH-1) or glucocorticoid-remediable aldosteronism (GRA) is an autosomal dominant disorder caused by formation of a chimeric gene between the CYP11B1 (11-beta-hydroxylase) and CYP11B2 (aldosterone synthase) genes.

This abnormal gene structure makes aldosterone synthesis sensitive to ACTH and produces aldosterone in the zona fasciculata. ACTH suppression with dexamethasone treatment normalises blood pressure and aldosterone levels.

Symptoms

Early-onset hypertension (childhood-young adult)
Severe and treatment-resistant hypertension
Hypokalaemia (sometimes mild or absent)
Family history of hypertension and stroke
Early-onset stroke at young ages
Cerebral aneurysms (increased risk)

Risk Factors

Autosomal dominant inheritance
Family history of early-onset hypertension
Family history of haemorrhagic stroke at young age
First-degree relative with FH-1
Resistant hypertension under age 20

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Hypertension developing under age 20
  • Family history of early-onset hypertension/stroke
  • Treatment-resistant hypertension + low PRA
  • Aldosterone elevated, renin suppressed
  • Cerebral aneurysm screening request

Treatment Methods

01
Genetic testing (Long-PCR for CYP11B1/CYP11B2 chimeric gene)
02
Aldosterone-renin ratio (high)
03
Dexamethasone suppression test (aldosterone suppression)
04
Low-dose dexamethasone (0.125-0.25 mg/day - first-line)
05
Mineralocorticoid receptor antagonist (spironolactone or eplerenone)
06
Cerebral MR angiography (aneurysm screening)
07
Family screening (counselling for first-degree relatives)
08
Lifelong monitoring (potassium, blood pressure)

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.