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Primary unilateral adrenal hyperplasia
Disease definition
Primary unilateral adrenal hyperplasia (PUAH) is a surgically-correctable form of primary (hyper) aldosteronism (PA; see this term) characterized by renin suppression, unilateral aldosterone hypersecretion, and moderate to severe hypertension secondary to hyperplasia of the adrenal gland.
ORPHA:231580
Classification level: Disorder- Synonym(s):
- PUAH
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: All ages
- ICD-10: E26.0
- OMIM: -
- UMLS: -
- MeSH: -
- GARD: -
- MedDRA: -
Summary
Epidemiology
The prevalence of primary unilateral adrenal hyperplasia is unknown.
Clinical description
PUAH may be associated with hypokalemia, which, when present, may be symptomatic with muscular weakness, cramps, paresthesia or palpitations with or without atrial fibrillation.
Etiology
The etiology of PUAH is not known.
Diagnostic methods
Diagnostic methods include peripheral aldosterone and renin determinations, adrenal venous sampling which makes it possible to differentiate unilateral from bilateral aldosterone hypersecretion, and CT scan showing normal adrenals or unilateral adrenal hyperplasia.
Management and treatment
Unilateral adrenalectomy abolishes aldosterone hypersecretion and hypokalemia in most patients with PUAH.
Prognosis
Blood pressure is significantly improved in the majority of the patients, but hypertension is cured in only 50% of cases.
A summary on this disease is available in Deutsch (2011) Español (2011) Français (2011) Italiano (2011) Nederlands (2011) Português (2011) Greek (2011, pdf) Polski (2011, pdf)
Detailed information
Guidelines
- Clinical practice guidelines
- Français (2016) - SFE
- English (2016) - J Clin Endocrinol Metab
: produced/endorsed by ERN(s)
: produced/endorsed by FSMR(s)
Additional information