| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
The MRC Environmental Epidemiology Unit, University of Southampton, Southampton General Hospital, Southampton [E. C. P., A. J. H., J. P. B.]; and the MRC Cell Mutation Unit, University of Sussex, Falmer, Brighton [B. A. B.], England
We have documented mortality and cancer incidence in the families of 67 patients with ataxia-telangiectasia and 48 patients with xeroderma pigmentosum resident in Britain. For both diseases, parents of patients are obligate heterozygotes and grandparents have a probability of heterozygosity of 0.5.
Fourteen ataxia-telangiectasia patients had died by June 30, 1986. This was a significant excess (14 deaths observed, 1.65 expected). Only one death was from a malignancy (non-Hodgkin's lymphoma). Three parents of ataxia-telangiectasia patients had died, all from cancer. The excess from breast cancer (two deaths observed, 0.17 expected) was statistically significant, p < 0.05. However, no excess mortality from malignant neoplasms was found in the grandparents.
Five xeroderma pigmentosum patients had died, none from internal malignancies. No excess mortality from malignant neoplasms was recorded in either their parents or grandparents.
1 To whom requests for reprints should be addressed, at MRC Cell Mutation Unit, University of Sussex, Falmer, Brighton BN1 9RR, England.
Received 8/12/87. Revised 1/19/88. Accepted 2/ 8/88.
This article has been cited by other articles:
|
|
 |
|
  P. Concannon, R. W. Haile, A.-L. Borresen-Dale, B. S. Rosenstein, R. A. Gatti, S. N. Teraoka, A. T. Diep, L. Jansen, D. P. Atencio, B. Langholz, et al. Variants in the ATM Gene Associated with a Reduced Risk of Contralateral Breast Cancer Cancer Res., August 15, 2008; 68(16): 6486 - 6491. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. Offit, S. Gilad, S. Paglin, P. Kolachana, L. C. Roisman, K. Nafa, V. Yeugelewitz, M. Gonzales, M. Robson, D. McDermott, et al. Rare Variants of ATM and Risk for Hodgkin's Disease and Radiation-associated Breast Cancers Clin. Cancer Res., December 1, 2002; 8(12): 3813 - 3819. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 D. Tang, D. Wu, A. Hirao, J. M. Lahti, L. Liu, B. Mazza, V. J. Kidd, T. W. Mak, and A. J. Ingram ERK Activation Mediates Cell Cycle Arrest and Apoptosis after DNA Damage Independently of p53 J. Biol. Chem., April 5, 2002; 277(15): 12710 - 12717. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M M de Jong, I M Nolte, G J te Meerman, W T A van der Graaf, J C Oosterwijk, J H Kleibeuker, M Schaapveld, and E G E de Vries Genes other than BRCA1 and BRCA2 involved in breast cancer susceptibility J. Med. Genet., April 1, 2002; 39(4): 225 - 242. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
T. Dork, R. Bendix, M. Bremer, D. Rades, K. Klopper, M. Nicke, B. Skawran, A. Hector, P. Yamini, D. Steinmann, et al. Spectrum of ATM Gene Mutations in a Hospital-based Series of Unselected Breast Cancer Patients Cancer Res., October 1, 2001; 61(20): 7608 - 7615. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. H. Olsen, J. M. Hahnemann, A.-L. Borresen-Dale, K. Brondum-Nielsen, L. Hammarstrom, R. Kleinerman, H. Kaariainen, T. Lonnqvist, R. Sankila, N. Seersholm, et al. Cancer in Patients With Ataxia-Telangiectasia and in Their Relatives in the Nordic Countries J Natl Cancer Inst, January 17, 2001; 93(2): 121 - 127. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 Y. Su and M. Swift Mortality Rates among Carriers of Ataxia-Telangiectasia Mutant Alleles Ann Intern Med, November 21, 2000; 133(10): 770 - 778. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 K. E. Nichols, S. Levitz, K. E. Shannon, D. C.R. Wahrer, D. W. Bell, G. Chang, S. Hegde, D. Neuberg, T. Shafman, N. J. Tarbell, et al. Heterozygous Germline ATM Mutations Do Not Contribute to Radiation-Associated Malignancies After Hodgkin's Disease J. Clin. Oncol., April 1, 1999; 17(4): 1259 - 1259. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Lavin Role of the ataxia-telangiectasia gene (ATM) in breast cancer BMJ, August 22, 1998; 317(7157): 486 - 487. [Full Text]
|
|
|
|
 |
|
 M. Meyn High spontaneous intrachromosomal recombination rates in ataxia-telangiectasia Science, May 28, 1993; 260(5112): 1327 - 1330. [Abstract] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| Cancer Research | Clinical Cancer Research |
| Cancer Epidemiology Biomarkers & Prevention | Molecular Cancer Therapeutics |
| Molecular Cancer Research | Cancer Prevention Research |
| Cancer Prevention Journals Portal | Cancer Reviews Online |
| Annual Meeting Education Book | Meeting Abstracts Online |
