Glucocorticoid-remediable aldosteronism (GRA) is a monogenic form of human hypertension that predisposes to cerebral hemorrhage. As a result of a chimeric. Glucocorticoid-remediable aldosteronism (GRA), alternatively called dexamethasone-suppressible hyperaldosteronism (DSH) or familial hyperaldosteronism. Aldosterone suppression by dexamethasone, and high hydroxycortisol and oxocortisol levels are used to differentiate glucocorticoid-remediable.
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Only comments written in English can be processed. Familial hyperaldosteronism type I FH-I is a rare heritable, glucocorticoid remediable form of primary aldosteronism PA characterized by early-onset hypertension, hyperaldosteronism, variable hypokalemia, low plasma renin activity PRAand abnormal production of oxocortisol and hydroxycortisol.
Hypertension, of varying severity even among members of the same family, manifests often before the age of It can be accompanied with symptoms of severe hypertension such as headaches and nausea. Cardiovascular anomalies, such as fibrosis, left ventricular LV dysfunction, arrhythmias, and myocardial infarction, can be associated with the disease.
Glucocorticlid adults, a high risk of cerebrovascular aneurysms is observed that causes premature hemorrhagic stroke.
Case Reports in Endocrinology
This leads to an excessive aldosterone synthase production in the zona fasciculata of the adrenal gland, regulated by the hypothalamo-pituitary axis rather than by the renin-angiotensin system.
Excessive aldosterone synthesis leads to increased sodium reabsorption, loss of potassium and subsequent increased water reabsorption. Blood and urinary tests show biological signs of primary aldosteronism, i.
Many patients are diagnosed during familial genetic screening in case of family history. Once the diagnosis adosteronism ascertained, small doses of glucocorticoids dexamethasoneat 0. In severe cases, dexamethasone can be combined with a mineralocorticoid receptor antagonist and other non-specific antihypertensive drugs.
Because of the side effects of glucocorticoids, in children eplerenone can be given instead. With treatment, hypertension can be controlled for many years, and echocardiographic parameters maintained in normal values.
A Case of Glucocorticoid Remediable Aldosteronism and Thoracoabdominal Aneurysms
Other search option s Alphabetical list. Summary and related texts. Check this box if you wish to receive a copy of your message. Disease definition Familial hyperaldosteronism type I FH-I is a rare heritable, glucocorticoid remediable form of primary aldosteronism PA characterized by early-onset hypertension, hyperaldosteronism, variable hypokalemia, low plasma renin rwmediable PRAand abnormal production of oxocortisol and hydroxycortisol.
Clinical description Hypertension, of varying severity even among members of the same family, manifests often before the age of Genetic counseling Transmission is autosomal dominant. Management and treatment Once the diagnosis is ascertained, small doses of glucocorticoids dexamethasoneat 0. Prognosis With treatment, hypertension can be controlled for many years, and glucocorticoidd parameters maintained in normal values.
Additional information Further information on this disease Classification glucocorgicoid 4 Gene s 2 Clinical signs and symptoms Publications in PubMed Other website s 4. Health care resources for this disease Expert centres Diagnostic tests 24 Patient organisations 32 Orphan drug s 0. Specialised Social Services Eurordis directory.
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