Cancer and neurologic degeneration in xeroderma pigmentosum: long term follow-up characterises the role of DNA repair.

@article{Bradford2011CancerAN,
  title={Cancer and neurologic degeneration in xeroderma pigmentosum: long term follow-up characterises the role of DNA repair.},
  author={Porcia T Bradford and Alisa M. Goldstein and Deborah Tamura and S G Khan and Takahiro Ueda and Jennifer L Boyle and Kyu-Seon Oh and Kyoko Imoto and Hiroki Inui and Shin-ichi Moriwaki and Steffen Emmert and Kristen M. Pike and Arati Raziuddin and Teri M Plona and J J DiGiovanna and Margaret A. Tucker and Kenneth H Kraemer},
  journal={Journal of medical genetics},
  year={2011},
  volume={48 3},
  pages={168-76}
}
BACKGROUND The frequency of cancer, neurologic degeneration and mortality in xeroderma pigmentosum (XP) patients with defective DNA repair was determined in a four decade natural history study. METHODS All 106 XP patients admitted to the National Institutes of Health from 1971 to 2009 were evaluated from clinical records and follow-up. RESULTS In the 65 per cent (n=69) of patients with skin cancer, non-melanoma skin cancer (NMSC) was increased 10,000-fold and melanoma was increased 2000… CONTINUE READING