This female patient from a consanguineous Pakistani family presented at age 9 yr. [ncbi.nlm.nih.gov]

In serum both aldosterone and renin levels are low [ citation needed ] Signs/symptoms [ edit ] This disorder presents similarly to hyperaldosteronism, leading to feedback inhibition of aldosterone. [en.wikipedia.org]

Laboratory Workup and Management The patient was started on lisinopril 10 mg daily and returned 1 week later for follow‐up, with a BP of 220/110 mm Hg. [onlinelibrary.wiley.com]

Martin Russell Pollak from Beth Israel Deaconess Medical Center at Harvard Medical School for their input and help during initial workup. F1000 recommended References 1. Garovic VD, Hilliard AA, Turner ST: Monogenic forms of low-renin hypertension. [f1000research.com]

The workup for hypertension revealed low renin and aldosterone levels. With such early clinical and biochemical presentation, monogenic hypertension is usually suspected. [thoracicmedicine.org]

The outcome of treatment in more patients may establish the efficacy of treatment. [ncbi.nlm.nih.gov]

Treatment : How to Treat "apparent mineralocorticoid excess"? Treatment for Apparent mineralocorticoid excess is primarily focused in correcting hypertension & hypokalemia. [signssymptoms.org]

Management and treatment Early diagnosis and treatment is important to prevent end-organ damage (central nervous system, kidney, heart and retina). Two main strategies can be used to treat AME. [orpha.net]

Prognosis In the absence of treatment, the prognosis for AME is severe with malignant hypertension, stroke, cardiac and renal insufficiency. However, the prognosis for patients with appropriate treatment appears to be good. [orpha.net]

In the absence of treatment, the prognosis for AME is severe with malignant hypertension, stroke, cardiac and renal insufficiency. However, the prognosis for patients with appropriate treatment appears to be good. [indianpediatrics.net]

Bartter: chronic Loop diuretic ingestion - Gitelman: chronic Thiaxide diuretic ingestion What is the prognosis for Bartter and Gitelman Syndromes? - Bartter: reduced in severe cases, prognosis is poor - Gitelman: good prognosis w/ proper treatment [cram.com]

The prognosis in these patients is ultimately dependent on progression of the underlying malignancy. Commonly, the prognosis is grave, as ectopic ACTH syndrome, like other paraneoplastic syndromes, is frequently associated with advanced disease. [medscape.com]

In addition to the low prevalence of surgically correctable PA, some patients do not undergo surgery and only one in two operated patients becomes normotensive without medication following an adrenalectomy (see Management and Prognosis below). [ojrd.biomedcentral.com]

[…] is a hereditary defect in 11β-hydroxysteroid dehydrogenase causing hypertension hypokalemia metabolic alkalosis these findings are similar to those in primary aldosteronism Epidemiology demographics early childhood onset risk factors family history Etiology [medbullets.com]

Etiology AME is caused by homozygous or compound heterozygous loss-of-function mutations or deletions in the HSD11B2 gene (16q22). [orpha.net]

[…] approximately one in three Americans 1, 2 In the United States, a majority of the current cases are classified as essential hypertension related to various behavioral and inheritable risk factors 2 However, in cases of resistant hypertension, an underlying etiology [onlinelibrary.wiley.com]

Both of these etiologies are the result of elevated production and secretion of renin leading to hyperaldosteronism. 4. Adrenal hyperfunction. [renalfellow.blogspot.com]

Clinical definition S yndrome of A pparent M ineralocorticoid E xcess ( SAME ) is a hereditary defect in 11β-hydroxysteroid dehydrogenase causing hypertension hypokalemia metabolic alkalosis these findings are similar to those in primary aldosteronism Epidemiology [medbullets.com]

Summary Epidemiology Prevalence is difficult to estimate and likely varies between populations depending on the level of consanguinity. Less than 100 cases have been reported in the literature so far. [orpha.net]

Epidemiology. AME is rare, having been identified in only approximately 40 patients worldwide in the past 20 years. [pnas.org]

Epidemiology Prevalence The prevalence of PA and its various surgically correctable subtypes in adults is not known. [ojrd.biomedcentral.com]

Obesity-related hypertension: epidemiology, pathophysiology, and clinical management. Am J Hypertens 2010; 23: 1170-1178 10 Krug AW, Ehrhart-Bornstein M. [thieme-connect.com]

Abstract Our research team and laboratories have concentrated on two inherited endocrine disorders, congenital adrenal hyperplasia (CAH) and apparent mineralocorticoid excess, in thier investigations of the pathophysiology of adrenal steroid hormone disorders [ncbi.nlm.nih.gov]

More than 2,000 disease genes have been identified in the last 20 years, providing important new insight into the pathophysiology of diseases in every field of medicine. [books.google.com]

Further studies are now required in order to evaluate the role of 11β-HSD2 activity in the pathophysiology of ‘essential’ hypertension. 1 Department of Medicine, University of Birmingham, Queen Elizabeth Hospital, Edgbaston, Birmingham, UK 2 Baker Medical [journals.lww.com]

The syndrome of AME mimics the findings of primary aldosteronism with resistant hypertension in the setting of hypokalemia, hypernatremia, and low plasma renin 4, 7 The pathophysiological basis of AME is impaired function of the enzyme 11‐beta‐hydroxysteroid [onlinelibrary.wiley.com]

Early and vigilant treatment might prevent or improve the morbidity and mortality of end-organ damage. [ncbi.nlm.nih.gov]