Short QTc Interval in Males with Klinefelter Syndrome-Influence of CAG Repeat Length, Body Composition, and Testosterone Replacement Therapy.

Background Klinefelter syndrome (KS) is a sex chromosomal aneuploidy (47,XXY) affecting 1/660 males. Based on findings in Turner syndrome, we hypothesized that electrocardiogram (ECG) abnormalities would be present in males with KS. Objective To investigate ECGs in males with KS and compare with con...

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Publicado en:Pacing & Clinical Electrophysiology Vol. 38; no. 4; pp. 472 - 483
Autores principales: JØRgensen, Inger Norlyk, SkakkebÆK, Anne, Andersen, Niels Holmark, Pedersen, Lisbeth NØRum, Hougaard, David Michael, Bojesen, Anders, Trolle, Christian, Gravholt, Claus HØJbjerg
Formato: research tables/charts Journal Article
Publicado: Wiley-Blackwell Apr2015
Acceso en línea:Ver este registro en EBSCOhost
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        atl: Short QTc Interval in Males with Klinefelter Syndrome-Influence of CAG Repeat Length, Body Composition, and Testosterone Replacement Therapy.
      aug:
        au:
          JØRgensen, Inger Norlyk
          SkakkebÆK, Anne
          Andersen, Niels Holmark
          Pedersen, Lisbeth NØRum
          Hougaard, David Michael
          Bojesen, Anders
          Trolle, Christian
          Gravholt, Claus HØJbjerg
        affil: Department of Endocrinology and Internal Medicine (MEA), Aarhus University Hospital
      sug:
        subj:
          Klinefelter's Syndrome Complications
          Heart Conduction System Abnormalities
          Testosterone Replacement Therapy Adverse Effects
          Electrocardiography Evaluation
          Mutation
          Human
          Male
          Descriptive Statistics
          Case Control Studies
          Intrarater Reliability
          Linear Regression
          Cross Sectional Studies
          Adolescence
          Young Adult
          Adult
          Middle Age
          Body Composition
          Karyotyping
          Blood Chemical Analysis
          Unpaired T-Tests
          Mann-Whitney U Test
          Chi Square Test
          Pearson's Correlation Coefficient
          Spearman's Rank Correlation Coefficient
          Data Analysis Software
          Funding Source
          Adolescent: 13-18 years
          Adult: 19-44 years
          Middle Aged: 45-64 years
          Male
      ab: Background Klinefelter syndrome (KS) is a sex chromosomal aneuploidy (47,XXY) affecting 1/660 males. Based on findings in Turner syndrome, we hypothesized that electrocardiogram (ECG) abnormalities would be present in males with KS. Objective To investigate ECGs in males with KS and compare with controls. Methods Case control study of 62 males with KS and 62 healthy males matched on age. The primary outcome parameter was a difference in the ECG presentation between the two groups. The ECGs were analyzed by one blinded examiner (intraobserver variability 0.2-2.1%). The QT-interval was measured using 'teach-the-tangent' method excluding the U-wave. QTc was calculated using Bazett's equation, Hodges' equation, and a linear regression model. Body mass index, abdominal fat, and muscle mass as well as sex hormone levels were secondary parameters. The prevalence of mutations in genes related to short QT syndrome was determined in participants with a QTc < 330 ms. Results Compared to controls, the QTc-interval was shorter (P = 0.02-0.06) in males with KS depending on the applied correction method. QTc was shortest among testosterone (T)-treated males with KS, while untreated and thus hypogonadal KS had QTc interval comparable to controls. No mutations in genes related to short QT syndrome were found. Conclusion We found short QTc interval in males with KS, with further shortening of the QTc interval by T. These results suggest that genes on the X chromosome could be involved in regulation of the QTc interval and that T treatment may aggravate this mechanism.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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