Glucocorticoid-remediable aldosteronism
ICD-10 E26.02 is a billable code used to indicate a diagnosis of glucocorticoid-remediable aldosteronism.
Glucocorticoid-remediable aldosteronism (GRA) is a rare genetic disorder characterized by the overproduction of aldosterone due to a mutation in the gene responsible for the regulation of aldosterone synthase. This condition is often caused by a chimeric gene formed by the fusion of the 11β-hydroxylase gene (CYP11B1) and the aldosterone synthase gene (CYP11B2). Patients with GRA typically present with hypertension and hypokalemia, which can lead to various cardiovascular complications if left untreated. The condition is termed 'glucocorticoid-remediable' because the administration of glucocorticoids can suppress the excessive aldosterone production, thereby normalizing blood pressure and potassium levels. Diagnosis is often confirmed through genetic testing and measurement of plasma aldosterone levels, particularly in response to ACTH stimulation. Management includes the use of glucocorticoids to control symptoms and prevent complications associated with hyperaldosteronism.
Detailed documentation of hormone levels, genetic testing results, and treatment plans.
Patients presenting with resistant hypertension and hypokalemia.
Endocrinologists must ensure comprehensive evaluations to differentiate GRA from other adrenal disorders.
Genetic testing results and family history documentation.
Patients with a family history of hypertension and unexplained electrolyte imbalances.
Geneticists should document the implications of findings for family members.
Used to confirm diagnosis of GRA.
Document the reason for testing and results.
Endocrinologists should ensure proper interpretation of results in the context of GRA.
Glucocorticoid-remediable aldosteronism is a genetic disorder that leads to excessive production of aldosterone, causing hypertension and electrolyte imbalances. It can be managed effectively with glucocorticoid therapy.
