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Mortality and cancer incidence in persons with Down's syndrome, their parents and siblings

Published online by Cambridge University Press:  17 May 2001

C. HERMON ,
E. ALBERMAN ,
V. BERAL  and
A. J. SWERDLOW
C. HERMON
Affiliation:
ICRF Cancer Epidemiology Unit, University of Oxford, Gibson Building, Radcliffe Infirmary, Oxford OX2 6HE, UK
E. ALBERMAN
Affiliation:
Department of Environmental and Preventive Medicine, Wolfson Institute of Preventive Medicine, St Bartholomews and Royal London School of Medicine and Dentistry, London EC1A 7BE, UK
V. BERAL
Affiliation:
ICRF Cancer Epidemiology Unit, University of Oxford, Gibson Building, Radcliffe Infirmary, Oxford OX2 6HE, UK
A. J. SWERDLOW
Affiliation:
Department of Epidemiology and Population Health, London School of Hygiene and Tropical Medicine, Keppel Street, London WC1E 7HT, UK
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Abstract

A cohort study of 1425 persons with Down's syndrome (DS), and of their parents (447 mothers, 435 fathers) and siblings (1176), was set up to investigate death rates from various causes and cancer incidence patterns. In individuals with DS the all-cause death rate was six times that of the national population (SMR = 622: 95% CI 559–693), the excess being attributable to many different causes. These included: leukaemia (SMR = 1304: 95% CI 651–2334); diabetes mellitus (SMR = 982: 95% CI 267–2515); Alzheimer's disease (SMR = 22028: 95% CI 7137–51326); epilepsy (SMR = 1727: 95% CI 744–3403); and congenital anomalies (SMR = 4987: 95% CI 4175–5955). The overall survival showed marked improvements for successive birth cohorts, particularly at young ages. For mothers and fathers of persons with DS, all-cause death rates were 20% lower than national rates and there were no significant excesses from any specific cause. For siblings, all-cause death rates were similar to national rates; the only condition with a significantly raised mortality ratio was colo-rectal cancer (SMR = 793: 95% CI 216–2031), but this may well be a chance finding.

Type
Research Article
Information
Annals of Human Genetics , Volume 65 , Issue 2 , March 2001 , pp. 167 - 176
Copyright
University College London 2001

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