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Arterial Hypertension in Turner Syndrome

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Disorders of Blood Pressure Regulation

Part of the book series: Updates in Hypertension and Cardiovascular Protection ((UHCP))

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Abstract

Turner syndrome (TS) is a rare chromosomal disorder in women with complete or partial absence of one X chromosome. The phenotype is highly variable, but congenital and acquired cardiovascular diseases are frequently associated disorders that add significantly to the increased morbidity and mortality in TS patients. The prevalence of arterial hypertension (AHT) is increased compared to the general population and can present earlier as from childhood on. The exact pathophysiology is poorly understood and probably multifactorial, but increased activity of the sympathetic nervous system seems to play a major role. Oestrogen deficiency due to premature ovarian failure may contribute to the development of AHT through sympathetic stimulation and predisposition to the metabolic syndrome. Secondary forms of AHT are rare in TS patients but may occur in the setting of aortic arch anomalies, including aortic coarctation, or kidney disease. AHT adds to the increased morbidity and mortality of TS and should be carefully diagnosed and rigorously treated, both in adult and paediatric patients.

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Author information

Authors and Affiliations

  1. Department of Paediatrics, Paediatric Cardiology and Turner Clinic, Ghent University Hospital, Ghent, Belgium

    Katya De Groote

  2. Department of Cardiology and Turner Clinic, Ghent University Hospital, Ghent, Belgium

    Laurent Demulier & Julie De Backer

  3. Department of Cardiology and Clinical Pharmacology, Ghent University Hospital, Ghent, Belgium

    Tine De Backer

Authors
  1. Katya De Groote
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  2. Laurent Demulier
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  3. Julie De Backer
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  4. Tine De Backer
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Corresponding author

Correspondence to Katya De Groote .

Editor information

Editors and Affiliations

  1. Internal Medicine, American University of Beirut Medical Center, Beirut, Lebanon

    Adel E. Berbari

  2. Emeritus Professor of Medicine, University of Milano-Bicocca, Monza, Italy

    Giuseppe Mancia

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De Groote, K., Demulier, L., De Backer, J., De Backer, T. (2018). Arterial Hypertension in Turner Syndrome. In: Berbari, A., Mancia, G. (eds) Disorders of Blood Pressure Regulation. Updates in Hypertension and Cardiovascular Protection. Springer, Cham. https://doi.org/10.1007/978-3-319-59918-2_12

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  • DOI: https://doi.org/10.1007/978-3-319-59918-2_12

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  • Online ISBN: 978-3-319-59918-2

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