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

Mortality and cancer incidence in persons with numerical sex chromosome abnormalities: a cohort study
Mortality and cancer incidence in persons with numerical sex chromosome abnormalities: a cohort study
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.
0003-4800
177-188
Swerdlow, A.J.
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Hermon, C.
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Jacobs, P.A.
32993834-5b30-4706-a09b-640baf848c49
Alberman, E.
4138e944-3103-4a9c-8cd9-647cf0f4e654
Beral, V.
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Daker, M.
c4717474-9e84-4a43-ba8c-87e28d9df471
Fordyce, A.
76d6cd80-fae4-47d8-8e1a-d1309c202c03
Youings, S.
ba64e5f7-1005-45ca-ba04-2ea40894b08a
Swerdlow, A.J.
1e736f3c-b291-4cbd-9850-20f4a0b91a86
Hermon, C.
2e38f2b2-71f8-43fb-8090-3bde6a9b37b5
Jacobs, P.A.
32993834-5b30-4706-a09b-640baf848c49
Alberman, E.
4138e944-3103-4a9c-8cd9-647cf0f4e654
Beral, V.
6eaadbd1-f775-4dd5-8bcc-3fa4db53416c
Daker, M.
c4717474-9e84-4a43-ba8c-87e28d9df471
Fordyce, A.
76d6cd80-fae4-47d8-8e1a-d1309c202c03
Youings, S.
ba64e5f7-1005-45ca-ba04-2ea40894b08a

Swerdlow, A.J., Hermon, C., Jacobs, P.A., Alberman, E., Beral, V., Daker, M., Fordyce, A. and Youings, S. (2001) Mortality and cancer incidence in persons with numerical sex chromosome abnormalities: a cohort study. Annals of Human Genetics, 65 (2), 177-188. (doi:10.1046/j.1469-1809.2001.6520177.x).

Record type: Article

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.

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Published date: March 2001

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Local EPrints ID: 24969
URI: http://eprints.soton.ac.uk/id/eprint/24969
ISSN: 0003-4800
PURE UUID: 6cce6226-eee6-4114-a77d-148201ac1ecf

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Date deposited: 05 Apr 2006
Last modified: 15 Mar 2024 06:59

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Contributors

Author: A.J. Swerdlow
Author: C. Hermon
Author: P.A. Jacobs
Author: E. Alberman
Author: V. Beral
Author: M. Daker
Author: A. Fordyce
Author: S. Youings

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