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This version published online on October 4, 2005
Journal of Clinical Endocrinology & Metabolism, doi:10.1210/jc.2005-1077
A more recent version of this article appeared on December 1, 2005
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*Klinefelter's Syndrome

Submitted on May 16, 2005
Accepted on September 22, 2005

Mortality in Patients with Klinefelter Syndrome in Britain : A Cohort Study

Anthony J Swerdlow*, Craig D Higgins, Minouk J Schoemaker, Alan F Wright, Patricia A Jacobs, and on behalf of the UK Clinical Cytogenetics Group

Section of Epidemiology, Institute of Cancer Research (AJS, CDH, MJS), Sutton, Surrey SM2 5NG; Cell & Molecular Genetics Section, MRC Human Genetics Unit, Western General Hospital (AFW), Edinburgh; Wessex Regional Genetics Laboratory, Salisbury District Hospital (PAJ), Salisbury, Wilts, UK

* To whom correspondence should be addressed. E-mail: anthony.swerdlow{at}icr.ac.uk.

Context: Klinefelter syndrome is characterized by hypogonadism and infertility, consequent on the presence of extra X chromosome(s). There is limited information about long-term mortality in this syndrome because there have been no large cohort studies.

Objectives: To investigate mortality in men with Klinefelter syndrome.

Design & Setting: We obtained data about patients diagnosed with Klinefelter syndrome at almost all cytogenetics centers in Britain, as far back as records were available, and conducted a cohort study of their mortality, overall and by karyotype.

Patients: 3518 patients diagnosed since 1959, followed to mid-2003.

Outcome measure: Standardized mortality ratio (SMR).

Results: 461 deaths occurred. There was significantly raised mortality overall (SMR, 1.5; 95% confidence interval (CI), 1.4 - 1.7) and from most major causes of death including cardiovascular disease (SMR, 1.3; 95% CI, 1.1 - 1.5), nervous system disease (SMR, 2.8; 95% CI, 1.6 - 4.6) and respiratory disease (SMR, 2.3; 95% CI, 1.8 - 2.9). Mortality was particularly raised from diabetes (SMR, 5.8; 95% CI, 3.4 - 9.3), epilepsy (SMR, 7.2; 95% CI, 3.1 - 14.1), pulmonary embolism (SMR, 5.7; 95% CI, 2.5 - 11.3), peripheral vascular disease (SMR, 7.9; 95% CI, 2.9 - 17.2), vascular insufficiency of the intestine (SMR, 12.3; 95% CI, 4.0 - 28.8), renal disease (SMR, 5.0; 95% CI, 2.0 - 10.3), and femoral fracture (SMR, 39.4; 95% CI, 4.8 - 142.3). Mortality from ischemic heart disease was significantly decreased (SMR, 0.7; 95% CI, 0.5 - 0.9).

Conclusions: Patients diagnosed with Klinefelter syndrome have raised mortality from several specific causes. This may reflect hormonal and genetic mechanisms.
Key words: Klinefelter syndrome • mortality • cohort




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