Primary hyperaldosteronism is a disorder caused by excess production of the hormone aldosterone by the adrenal glands. Primary aldosteronism hyperaldosteronism is a condition that occurs when the adrenal glands produce too much aldosterone, the hormone responsible for balancing potassium and sodium in the body. Almeida1,2 abstract primary aldosteronism pa is the most common form of secondary hypertension htn, with an estimated prevalence of 4% of hypertensive patients in primary care and around 10% of. Most cases of primary hyperaldosteronism result from a benign tumor of the adrenal gland, and occur in people between the ages of 30 and 50 years old. Primary hyperaldosteronism secondary to unilateral adrenal hyperplasia. Article information, pdf download for primary hyperaldosteronism. Not all of these cases have been reported, and of these that have, the vast majority appear in the nonsurgical literature. Hypothesis that the clinical presentations, biochemical profiles, and surgical outcomes of patients treated with laparoscopic vs open adrenalectomy for primary hyperaldosteronism are different design, settings, patients, and interventions the medical records of 80 patients with primary hyperaldosteronism who underwent open adrenalectomy between 1975 and 1986 or laparoscopic. Outcomes after adrenalectomy for unilateral primary. The diagnosis and treatment of primary hyperaldosteronism. The german conn registry was established in 2006 to evaluate the diagnosis and treatment of primary hyperaldosteronism in germany and to document comorbid conditions and the disease course.
Primary aldosteronism endocrine and metabolic disorders. Many suffer from fatigue, potassium deficiency and high blood pressure which may cause poor vision, confusion or headaches. Primary hyperaldosteronism associated with hypertensive. Please refer to table 1 for patients with hypertension to be. It is typically due to adrenal hyperplasia or adrenal adenoma. Aldosterone is the principal mineralocorticoid in man. Primary aldosteronism pa is a common form of endocrine hypertension. Primary aldosteronism is the most common form of endocrine hypertension. Diagnosis and treatment of primary hyperaldosteronism. Find a list of current medications, their possible side effects, dosage, and efficacy when used to treat or reduce. Primary aldosteronism pa is a heterogeneous group of disorders. Primary aldosteronism represents major cause of secondary hypertension, strongly associated with high cardiovascular morbidity and mortality. Hyperaldosteronism, increased secretion of the hormone aldosterone by the cells of the zona glomerulosa the outer zone of the adrenal cortex. The patient was a 33yearold male with right adrenal incidentaloma, but without any symptom.
The objective of this case report is to present a case where subtype classification of pa was challenging and repeated imaging of the adrenal glands helped establish the. An inherited form of primary hyperaldosteronism is the glucocorticoidremediable aldosteronism gra caused by an unequal crossing over between the cyp11b1 and cyp11b2. The use of aldosterone renin ratio arr for screening contributes to the increased diagnostic rate of this disease. Aldosteronoma is localized with a ct scan or mri of the abdomen prior to planning surgery. Suspected primary hyperaldosteronism is challenging to investigate and manage in pregnancy.
Conn syndrome, as originally described, refers specifically to primary aldosteronism due to the presence of an adrenal aldosteronoma aldosterone secreting benign adrenal neoplasm. As such, it is a highly treatable and potentially curable form of hypertension. The variable baseline clinical characteristics of our international cohort contributed to wide variation in clinical outcomes. However, primary aldosteronism is the main cause for second ary hypertension. Research article management of primary and secondary hyperaldosteronism. Overproduction of aldosterone causes the body to retain more sodium and lose potassium, which leads to elevated blood pressure. New advances in the diagnosis of primary aldosteronism. Prevalence and clinical manifestations of primary aldosteronism. Primary aldosteronism merck manuals professional edition. In many cases, somatic mutations in ion channels and pumps within adrenal cells initiate the pathogenesis of pa, and this mechanism might explain why pa is so common and suggests that milder and evolving forms of pa must exist. As timely diagnosis and treatment can provide a cure of hyperaldosteronism and hypertension, even when the latter is resistant to drug treatment, strategies to screen patients for pa early with a simplified diagnostic algorithm.
This is the first report of eplerenone use instead of spironolactone to treat primary hyperaldosteronism in a malebearing pregnant woman. Based on older data, it was originally estimated that primary aldosteronism accounted for less than 1% of all patients with htn. A left adrenalectomy was performed and pathologic evaluation confirmed an aldosteronoma. Primary aldosteronism is caused by an adenoma, usually unilateral, of the glomerulosa cells of the adrenal cortex or, more rarely, by adrenal carcinoma or hyperplasia. In primary hyperaldosteronism, which is due to an abnormality within the adrenal gland, the initiating event is an autonomous increase in the secretion of aldosterone. Hyperaldosteronism may be classified as primary or secondary. Further, the measurement process is manual and can be affected by sample. Recent studies have demonstrated that primary aldosteronism pa is the most common form of secondary hypertension when determinations of serum aldosterone sa, plasma renin activity pra, and the sapra ratio are used as screening tools and the fludrocortisone, saline infusion, or captopril tests are used to confirm the diagnosis. Primary hyperaldosteronism genetic and rare diseases.
Primary aldosteronism and hypertensive disease hypertension. Adrenal myelolipoma associated with primary hyperaldosteronism. Primary hyperaldosteronism is induced by the overproduction of aldo by the adrenal gland. Adenomas are extremely rare in children, but primary aldosteronism sometimes occurs in childhood adrenal carcinoma or hyperplasia.
Primary hyperaldosteronism edit edit source primary aldosteronism, which is defined as a nonsuppressible hyper secretion of aldosterone from the adrenal glands, can occur through several etiologies but the two most common are. Paul margulies, md, face, facp, medical director, nadf. The initial screening test is the aldosteronerenin ratio. This disorder comprises both sporadic and familial forms. Primary hyperaldosteronism, sometimes referred to as conn syndrome, is an excess of aldosterone caused by autonomous overproduction, usually at the adrenal cortex. The initial screening test is the aldosterone renin ratio. Patient group affected cats present at a median age of years range 520 years. We report the case of a 37yearold africanamerican woman with refractory hypertension who was admitted to our hospital for palpitations, shortness of breath and headache. Laparoscopic vs open adrenalectomy for the treatment of. A ct scan of the adrenal glands showed a left 2cm lesion with characteristics typical of an adenoma. The use of aldosteronerenin ratio arr for screening contributes to the increased diagnostic rate of this disease. Eplerenone use in primary aldosteronism during pregnancy. Primary hyperaldosteronism is one of the most common causes of secondary hypertension but clear differentiation between its various subtypes can be a clinical challenge.
The syndrome of primary hyperaldosteronism is not rare. Comparison of the biochemical profile and target organ damage in the cohort of essential hypertensive patients and those with primary hyperaldosteronism. Primary aldosteronism pa is the most common form of secondary hypertension. The first page of the pdf of this article appears above. This document was downloaded for personal use only. Primary aldosteronism pa or conn syndrome is the most common secondary form of arterial hypertension, with an estimated prevalence ranging between 6% and 20% in patients with resistant hypertension.
It is considered a secondary form of hypertension caused by inappropriate secretion of aldosterone and activation of the mineralocorticoid receptor. A total of 1,672 primary care patients with hypertension 569 newly diagnosed. Primary aldosteronism is aldosteronism caused by autonomous production of aldosterone by the adrenal cortex due to hyperplasia, adenoma, or carcinoma. Flow chart for the diagnostic workup of primary aldosteronism.
The primary actions of aldosterone are to increase retention of salt and water and to increase excretion of potassium by the kidneys and to a lesser extent by the skin and intestine. Clinical associate professor of medicine, zucker school of medicine at hofstranorthwell. Primary aldosteronism hyperaldosteronism cedarssinai. Primary aldosteronism pa is a common, but frequently overlooked, cause of arterial hypertension and excess cardiovascular events, particularly atrial fibrillation. Primary hyperaldosteronism is the most common cause of secondary hypertension. The retrospective analysis presented in this article is based on data from 555 patients treated since 1990 in the participating centers. Diagnosis and management of primary aldosteronism leticia a. The classic presenting signs of primary aldosteronism are hypertension and hypokalemia, but potassium levels are often normal in modernday series of aldosteronomas. Even in the absence of hypokalaemia and especially in patients with difficult to control hypertension, arr may be useful. Consultant, division of endocrinology, metabolism, nutrition and internal medicine, mayo clinic and mayo foundation, rochester, minnesota 55905 primary aldosteronism affects 5% of patients with. Bone and mineral metabolism in patients with primary. Practical relevance primary hyperaldosteronism is probably the most.
Primary hyperaldosteronism and related hypertension. Familial hyperaldosteronism type iii journal of human. Primary aldosteronism pa, also known as primary hyperaldosteronism or conns syndrome, refers to the excess production of the hormone aldosterone from the adrenal glands, resulting in low renin levels. An inherited form of primary hyperaldosteronism is the glucocorticoidremediable aldosteronism gra caused by an unequal crossing over. Pdf primary aldosteronism practical approach to diagnosis and. Pdf primary aldosteronism pa is the most common form of secondary.
Treatment with aldo inhibitors can reduce these symptoms until the source of the excessive aldo is removed. Its classical functions include regulation of extracellular volume and potassium homeostasis through its effects on the renal distal convoluted tubule. Practical relevance primary hyperaldosteronism is probably the most common adrenocortical disorder in cats. He has no history of hypertension, and blood pressure was normal when measured at multiple time points during hospitalization stay.
Primary hyperaldosteronism pa is a serious and potentially debilitating disease. Management of primary and secondary hyperaldosteronism. Primary aldosteronism msd manual professional edition. These standardised outcome criteria are relevant for the assessment of the success of surgical treatment in individual patients and will allow the comparison of outcome data in future studies. Primary hyperaldosteronism is a disease caused by an excess production of the normal adrenal hormone aldosterone. Primary aldosteronismchanging concepts in diagnosis and treatment william f. Primary hyperaldosteronism was confirmed with urine aldosterone of 23.
Nonsuppressible primary hypersecretion of aldosterone is an underdiagnosed cause of hypertension. Recently, in a cohort of pa patients a significant increase of primary hyperparathyroidism was found. Diagnosis and management of primary aldosteronism scielo. High systemic aldosterone levels result in increased sodium reabsorption and. Hyperaldosteronism is an endocrine disorder characterized by the excessive production of aldosterone by the adrenal glands. Primary hyperaldosteronism is one of the common causes of secondary hypertension. Pdf primary aldosteronism is caused by autonomous production of aldosterone by the adrenal cortex due to hyperplasia, adenoma. Primary aldosteronism pa due to aldosteroneproducing adenoma apa is a form of surgically curable secondary hypertension, and distinguishing apa from. Primary hyperaldosteronism an overview sciencedirect topics. General characteristics of the population with primary hyperaldosteronism and comparison with essential hypertensive patients. The prevalence of primary hyperaldosteronism among general and resistant hypertensive populations ranges from 520%. Compared with primary hypertension, pa causes more endorgan.
Retrospective analysis of the following data from 82 patients with primary aldosteronism. This abnormality is caused by hyperplasia or tumors. As in humans, it is often unrecognised, which excludes a potentially large number of cats from appropriate treatment. Most patients derive clinical benefit from adrenalectomy, with younger. This study was to report a case of normotensive patient with primary aldosteronism who was admitted to our department recently. A breed or sex predilection has not been documented. The accepted screening and confirmatory tests are either contraindicated or. Symptoms and signs include episodic weakness, elevated blood pressure, and hypokalemia. There are no human data in the literature supporting or discouraging its use in pregnancy. Hyperaldosteronism an overview sciencedirect topics.
Aldosterone excess may influence mineral homeostasis, through higher urinary calcium excretion inducing secondary increase of parathyroid hormone. Detailed guidelines have been written to guide endocrinologists in establishing the diagnosis of pa as well as in subtype classification of pa. Blood and urine tests for aldosterone and renin is confirm the chemical diagnosis. When primary hyperaldosteronism is considered by a physician, tests are done to look for an excess of the hormone aldosterone in the blood and urine, and also a suppressed plasma renin. The main symptom of primary hyperaldosteronism is high blood pressure hypertension, but other symptoms may include headaches, weakness, swelling, and muscle spasms. Recent evidence suggests that the prevalence of primary hyperaldosteronism is higher than originally thought. Patient usually has uncontrollable hypertension and low potassium level in the blood. An aldosteroneproducing adrenal adenoma with typical cholesterolrich yellow appearance.
195 70 1280 330 805 80 722 424 461 805 349 1303 857 652 129 1421 1135 888 218 913 902 1392 344 564 1120 1007 440 1327 164 1334 898 1264 1431 351 356 669 383 170 1182 1303 782 606 819 675 722 501 1036