Hostname: page-component-7c8c6479df-fqc5m Total loading time: 0 Render date: 2024-03-29T12:33:03.487Z Has data issue: false hasContentIssue false

Mortality and cancer incidence in persons with numerical sex chromosome abnormalities: a cohort study

Published online by Cambridge University Press:  17 May 2001

A. J. SWERDLOW
Affiliation:
Section of Epidemiology, D Block, Institute of Cancer Research, Cotswold Road, Sutton, Surrey SM2 5NG, UK
C. HERMON
Affiliation:
ICRF Cancer Epidemiology Unit, University of Oxford, Institute of Health Sciences, Old Road, Headington, Oxford OX3 7LF, UK
P. A. JACOBS
Affiliation:
Wessex Regional Genetics Laboratory, Salisbury District Hospital, Salisbury, Wilts SP2 8BJ, UK
E. ALBERMAN
Affiliation:
3 Millfield Place, Highgate, London N6 6JP, UK
V. BERAL
Affiliation:
ICRF Cancer Epidemiology Unit, University of Oxford, Institute of Health Sciences, Old Road, Headington, Oxford OX3 7LF, UK
M. DAKER
Affiliation:
Department of Cytogenetics, Guy's Hospital, 8th Floor, Guy's Tower, St. Thomas Street, London SE1 9RT, UK Current address: 34 Gibb Green, Edgware, Middx HA8 9RN, UK
A. FORDYCE
Affiliation:
MRC Human Genetics Unit, Western General Hospital, Edinburgh EH4 2XU, UK
S. YOUINGS
Affiliation:
Wessex Regional Genetics Laboratory, Salisbury District Hospital, Salisbury, Wilts SP2 8BJ, UK
Get access

Abstract

Mortality and cancer incidence were assessed in a cohort of 1373 patients with numerical sex chromosome abnormalities diagnosed at three cytogenetics centres in Britain during 1959–90, and were compared with expectations from national rates. Four hundred patients with Turner's syndrome were followed, of whom 62 died, with a relative risk (RR) of death of 4.16 (95% confidence interval (CI) 3.22–5.39). Turner's syndrome patients had greatly raised risks of death from diseases of the nervous, cardiovascular, respiratory, digestive and genitourinary systems. One hundred and sixty three deaths occurred among 646 patients with Klinefelter's syndrome with a 47,XXY constitution, giving an RR of 1.63 (1.40–1.91). Mortality in these patients was significantly raised from diabetes and diseases of the cardiovascular, respiratory and digestive systems. There was also significantly increased mortality for patients with X polysomy (RR = 2.11 (1.43–3.02)) and Y polysomy (RR = 1.90 (1.20–2.85)), the former with significantly increased mortality from cardiovascular disease and the latter from respiratory disease. The only significantly raised risks of cancer incidence or mortality in the cohort were for lung cancer and breast cancer in patients with Klinefelter's syndrome with a 47,XXY constitution, and non-Hodgkin's lymphoma in men with more than three sex chromosomes.

Type
Research Article
Copyright
University College London 2001

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)