Low renin forms of monogenic hypertension: review of the evidence

Ugochi Chinenye Okorafor 1 * , Uchechi Chioma Okorafor 2
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1 Department of Cardiology, Meridian Cardiac Center, Festac Town, Lagos, Nigeria
2 Department of Medicine and Surgery, College of Medicine, University of Ibadan, University Of Ibadan, Ibadan, Nigeria
* Corresponding Author
J CLIN MED KAZ, Volume 21, Issue 1, pp. 14-20. https://doi.org/10.23950/jcmk/14269
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Author Contributions
Conceptualization, Ug. C. O.; methodology, Ug. C. O.; validation – not applicable; formal analysis, – not applicable; investigation, Ug. C. O. and Uch. C. O; resources research, Ug. C. O., Uch. C. O.; data curation – not applicable; writing – original draft preparation, Ug. C. O.; writing – review and editing, Ug. C. O.; visualization, – not applicable; supervision – not applicable; project administration – not applicable; funding acquisition – not applicable. The authors have read and agreed to the published version of the manuscript.

ABSTRACT

Background: Monogenic hypertension syndromes result from a single genetic mutation and present with severe, refractory hypertension, distinct laboratory abnormalities, and a positive family history. These syndromes are often unrecognized or misdiagnosed as essential hypertension, thus preventing proper treatment. The rise of molecular genetics has brought these conditions to the limelight, and physicians must be kept abreast of the latest in this field. This paper aims to educate doctors to recognize and institute appropriate management early to prevent end-organ damage.
Discussion: These syndromes all affect sodium transport in the distal nephron of the kidneys. However, they are divided based on the location of the primary disorder, i.e., the adrenal glands or the distal nephron and discussed in that manner. Tables provide an overview of the different syndromes and provide essential information in a snapshot.
Conclusion: The widespread availability of genetic testing facilities will aid in the earlier diagnosis of these conditions to prevent morbidity.
Keywords: Apparent Mineralocorticoid Excess, Congenital Adrenal Hyperplasia, Familial Hyperaldosteronism, Gordon Syndrome, Hypertension, Liddle Syndrome

CITATION

Okorafor UC, Okorafor UC. Low renin forms of monogenic hypertension: review of the evidence. J CLIN MED KAZ. 2024;21(1):14-20. https://doi.org/10.23950/jcmk/14269

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