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      Liddle Syndrome in Association with Aortic Dissection

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          Abstract

          Liddle syndrome is a rare form of autosomal dominant monogenic hypertension manifested as an early onset of resistant hypertension with either no response or suboptimal response to conventional antihypertensive therapy. If there is a delay in diagnosis, uncontrolled hypertension can lead to end organ damage. To our knowledge, aortic dissection has not been reported in association with this disease. We report a case of a dissecting aortic aneurysm occurring in association with Liddle syndrome.​

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          Prevalence of Liddle Syndrome Among Young Hypertension Patients of Undetermined Cause in a Chinese Population.

          Xian-Liang Zhou, Ya-Xin Liu, Ru-Tai Hui … (2015)
          Liddle syndrome, an autosomal dominant form of monogenic hypertension, has been regarded as a rare disorder, which leads to many Liddle syndrome patients being misdiagnosed and experiencing severe complications at an early age. Little is known about the prevalence of Liddle syndrome. In this study, the authors investigated the prevalence of Liddle syndrome confirmed by genetic testing among young hypertension patients of undetermined causes in China. A total of 330 hypertensive patients aged 14 to 40 years after exclusion of common secondary causes of hypertension were enrolled and serum potassium concentrations were measured. Patients with hypokalemia underwent genetic testing of the 13th exon of genes encoding β and γ subunits of the epithelial sodium channel (ENaC). Diagnosis was established by identification of mutations that destroy the PY motif of ENaC. Five patients were diagnosed with Liddle syndrome (prevalence, 1.52%), as well as 12 of their relatives. These patients with Liddle syndrome presented with an earlier onset of hypertension, a stronger family history of hypertension, and higher blood pressure than those with essential hypertension. All patients had hypokalemia and suppressed plasma renin activity. The results demonstrated that Liddle syndrome is an important etiology of hypertension in this young population. Screening of Liddle syndrome should focus on young hypertension patients, particularly those with early penetrance, hypokalemia, and low renin levels after exclusion of common secondary causes.
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            A familial renal disorder simulating primary aldosteronism but with negligible aldosterone secretion

            GW Liddle, T Bledsoe, WSJ Coppage … (1963)
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              Liddle syndrome: genetics and mechanisms of Na+ channel defects.

              Neil Warnock (2001)
              Our current understanding of Na+ transport defects has been greatly expanded over the last several years and has provided new insights into unusual clinical syndromes resulting from mutations of specific ion transporters. These genetic disorders affect Na+ balance, with both Na+ retaining and Na+ wasting conditions being the consequence. A major focus of these studies has been the epithelial sodium channel (ENaC), which can be directly affected by mutations (eg, Liddle syndrome, autosomal recessive pseudohypoaldosteronism, type I) or by changes in the response to (autosomal recessive pseudohypoaldosteronism, type I), or production of mineralocorticoids (apparent mineralocorticoid excess syndrome, glucocorticoid-remediable aldosteronism). As a result, we now have clearly defined syndromes in which ENaC activity is "dysregulated" with subsequent development of disorders of systemic blood pressure that can be attributed to a primary renal mechanisms. The focus of the current review is on Liddle syndrome ("pseudoaldosteronism").
              Article 0 comments Cited 16 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 4 May 2017
                Publication date (Electronic, collection): May 2017
                Volume: 9
                Issue: 5
                Electronic Location Identifier: e1225
                Affiliations
                [1 ] Internal Medicine Residency, Florida Hospital-Orlando
                [2 ] Internal Medicine, Florida Hospital-Orlando
                [3 ] Diagnostic Radiology, Florida Hospital-Orlando
                [4 ] Orlando Cardiac & Vascular Specialists, Florida Hospital-Orlando
                Author notes
                Article
                DOI: 10.7759/cureus.1225
                PMC ID: 5453742
                SO-VID: 3c5abe24-0f54-4913-addd-cd4e0edc8e51
                Copyright © Copyright © 2017, Abbass et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date received : 2 April 2017
                Date accepted : 4 May 2017
                Categories
                Subject: Nephrology
                Subject: Cardiac/Thoracic/Vascular Surgery
                Subject: Cardiology

                Keywords: aortic dissection,liddle syndrome,premature hypertension
                Data availability:
                Keywords: aortic dissection, liddle syndrome, premature hypertension

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