Morbidity and mortality in Klinefelter syndrome (47,XXY).

Abstract

UNLABELLED:

Klinefelter syndrome (KS) (47,XXY) is the most common sex chromosome disorder in man and is a relatively common cause of male infertility and hypogonadism. The syndrome has been known since 1942, and many reports of different diseases associated with KS have been reported since that, but a more systematic knowledge about the long-term outcome was not described until the last decade, where nation-wide epidemiological studies were reported from Britain and Denmark. We here review the epidemiological data from two cohorts of patients with KS in Denmark and Britain, showing a significant increase in both mortality and morbidity from a variety of different causes. Mortality was increased by 50% (SMR 1.5 or HR 1.4) corresponding to a median loss of approximately 2 years. The risk of being admitted to hospital with any diagnosis was increased by 70%. The underlying reason for the poorer health in KS may be caused by interaction of genetic, hormonal and socio-economic factors.

CONCLUSION:

Both morbidity and mortality are significantly increased in Klinefelter syndrome with a 50% increase in mortality risk and a 70% increase in risk of being admitted to hospital.

• Authors: Anders Bojesen, Claus H Gravholt
• Journal: Acta paediatrica (Oslo, Norway : 1992) (Acta Paediatr) Vol. 100 Issue 6 Pg. 807-13 (Jun 2011) ISSN: 1651-2227 [Electronic] Norway
• PMID: 21414026 (Publication Type: Journal Article, Review)
• Copyright: © 2011 The Author(s)/Acta Paediatrica © 2011 Foundation Acta Paediatrica.

Topics

• Denmark (epidemiology)
• Hospitalization (statistics & numerical data)
• Humans
• Klinefelter Syndrome (epidemiology, mortality)
• Male
• Morbidity
• Neoplasms (epidemiology)
• Risk
• United Kingdom (epidemiology)