Nocturnal hypertension and impaired sympathovagal tone in Turner syndrome

Gravholt, Claus Højbjerg; Hansen, Klavs Würgler; Erlandsen, Mogens; Ebbehøj, Eva; Christiansen, Jens Sandahl

doi:10.1097/01.hjh.0000200509.17947.0f

Cited by 77 publications

(81 citation statements)

References 45 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…28 The use of ␤-blockade in TS may be particularly appropriate because it is a relatively benign treatment and because resting tachycardia is common in TS. 37,38 Likewise, aggressive blood pressure control, frequent imaging, and exercise guidelines for those with aortic dilatation is a very rationale approach. 39 A major limitation of our study was a lack of correlative information on aortic root anatomy either by more extended magnetic resonance or by echocardiography.…”

Section: Discussionmentioning

confidence: 99%

Aortic Dilatation and Dissection in Turner Syndrome

et al. 2007

View full text Add to dashboard Cite

Background-The risk for aortic dissection is increased among relatively young women with Turner syndrome (TS). It is unknown whether aortic dilatation precedes acute aortic dissection in TS and, if so, what specific diameter predicts impending deterioration. Methods and Results-Study subjects included 166 adult volunteers with TS (average age, 36.2 years) who were not selected for cardiovascular disease and 26 healthy female control subjects. Ascending and descending aortic diameters were measured by magnetic resonance imaging at the right pulmonary artery. TS women were on average 20 cm shorter, yet average aortic diameters were identical in the 2 groups. Ascending aortic diameters normalized to body surface area (aortic size index) were significantly greater in TS, and Ϸ32% of TS women had values greater than the 95th percentile of 2.0 cm/m 2 . Ascending diameter/descending diameter ratios also were significantly greater in the TS group. During Ϸ3 years of follow-up, aortic dissections occurred in 3 women with TS, for an annualized rate of 618 cases/100 000 woman-years. These 3 subjects had ascending aortic diameters of 3.7 to 4.8 cm and aortic size indices Ͼ2.5 cm/m 2 . Conclusions-The risk for aortic dissection is greatly increased in young women with TS. Because of their small stature, ascending aortic diameters of Ͻ5 cm may represent significant dilatation; thus, the use of aortic size index is preferred. Individuals with a dilated ascending aorta defined as aortic size index Ͼ2.0 cm/m 2 require close cardiovascular surveillance. Those with aortic size index Ն2.5 cm/m 2 are at highest risk for aortic dissection.

show abstract

Section: Discussionmentioning

confidence: 99%

Aortic Dilatation and Dissection in Turner Syndrome

et al. 2007

View full text Add to dashboard Cite

show abstract

“…15,100 -102 Half of Turner syndrome women have hypertension. 92,103 Careful auscultation and lifelong blood pressure monitoring are essential, and for women who have had coarctation repair, the blood pressure cuff should be placed on the right arm. A variety of electrocardiographic and repolarization abnormalities have been reported which seem to expand the spectrum of cardiac involvement in Turner syndrome.…”

Section: Cardiovascular Abnormalities In Adulthoodmentioning

confidence: 99%

Adults with genetic syndromes and cardiovascular abnormalities: clinical history and management

Lin

Basson

Goldmuntz

et al. 2008

Genetics in Medicine

131

View full text Add to dashboard Cite

Cardiovascular abnormalities, especially structural congenital heart defects, commonly occur in malformation syndromes and genetic disorders. Individuals with syndromes comprise a significant proportion of those affected with selected congenital heart defects such as complete atrioventricular canal, interrupted arch type B, supravalvar aortic stenosis, and pulmonary stenosis. As these individuals age, they contribute to the growing population of adults with special health care needs. Although most will require longterm cardiology follow-up, primary care providers, geneticists, and other specialists should be aware of (1) the type and frequency of cardiovascular abnormalities, (2) the range of clinical outcomes, and (3) guidelines for prospective management and treatment of potential complications. This article reviews fundamental genetic, cardiac, medical, and reproductive issues associated with common genetic syndromes that are frequently associated with a cardiovascular abnormality. New data are also provided about the cardiac status of adults with a 22q11.2 deletion and with Down syndrome. Genet Med 2008:10(7):469 -494.

show abstract

“…1996/3561). Analysis of beat-to-beat variation and 24 h ambulatory blood pressure measurements from this study has previously been presented (11).…”

Section: Subjects and Designmentioning

confidence: 99%

Quantitative liver functions in Turner syndrome with and without hormone replacement therapy

et al. 2007

Self Cite

View full text Add to dashboard Cite

Background: Studies have documented elevated levels of liver enzymes in many females with Turner syndrome (TS). Histology has shown a range of changes. Treatment with female hormone replacement therapy (HRT) reduces liver enzymes. Aim: To study quantitative liver functions in TS in detail with and without HRT. Design: Randomized crossover study with active treatment (HRT in TS and P-pill in controls) or no treatment. Subjects: Women with TS (nZ8, age 29.7G5.6 (meanGS.D.) years), verified by karyotype, and agematched controls (C; nZ8, age 27.3G4.9 years). Methods: We determined liver enzymes in blood, used the galactose elimination capacity to assess hepatocyte cytosol activity, plasma clearance of indocyanine green to assess excretory function, antipyrine clearance to estimate microsomal activity, and the functional hepatic nitrogen clearance (FHNC) to assess mitochondrial-cytosolic metabolic capacity for conversion of amino-nitrogen. Results: Liver enzymes were elevated in untreated TS and reduced by HRT. The hepatic capacities for conversion of galactose, indocyanine green, and antipyrine were normal and did not change by HRT.

show abstract

Nocturnal hypertension and impaired sympathovagal tone in Turner syndrome

Cited by 77 publications

References 45 publications

Aortic Dilatation and Dissection in Turner Syndrome

Aortic Dilatation and Dissection in Turner Syndrome

Adults with genetic syndromes and cardiovascular abnormalities: clinical history and management

Quantitative liver functions in Turner syndrome with and without hormone replacement therapy

Contact Info

Product

Resources

About