We thank Dr Spence for his interest in the 2020 International Society of Hypertension (ISH) Global Hypertension Practice Guidelines and highlighting what he considers blind spots in the new guidelines. Specifically, the author is concerned that Liddle syndrome, one of the rare monogenic causes of hypertension is not mentioned and its treatment not appropriately addressed in the 2020 ISH guidelines. Dr Spence provides insightful comments describing the central role of the epithelial sodium channel (ENaC) in Liddle syndrome which is responsible for its clinical features including early onset of salt-sensitive hypertension, hypokalemic metabolic alkalosis, and suppression of both renin and aldosterone secretion. Importantly, Liddle syndrome is associated with increased incidence of premature cardiovascular and cerebrovascular events and usually unresponsive to mineralocorticoid receptor antagonists.
Reply / Schlaich MP, Borghi C, Tomaszewski M, Stergiou GS, Schutte AE, Unger T. - In: JOURNAL OF HYPERTENSION. - ISSN 0263-6352. - STAMPA. - 38:11(2020), pp. 2339-2340. [10.1097/HJH.0000000000002612]
Reply
Borghi CSecondo
Writing – Review & Editing
;
2020
Abstract
We thank Dr Spence for his interest in the 2020 International Society of Hypertension (ISH) Global Hypertension Practice Guidelines and highlighting what he considers blind spots in the new guidelines. Specifically, the author is concerned that Liddle syndrome, one of the rare monogenic causes of hypertension is not mentioned and its treatment not appropriately addressed in the 2020 ISH guidelines. Dr Spence provides insightful comments describing the central role of the epithelial sodium channel (ENaC) in Liddle syndrome which is responsible for its clinical features including early onset of salt-sensitive hypertension, hypokalemic metabolic alkalosis, and suppression of both renin and aldosterone secretion. Importantly, Liddle syndrome is associated with increased incidence of premature cardiovascular and cerebrovascular events and usually unresponsive to mineralocorticoid receptor antagonists.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
