Literature DB >> 11410307

Normal cellular radiosensitivity in an adult Fanconi anaemia patient with marked clinical radiosensitivity.

Y Marcou1, A D'Andrea, P A Jeggo, P N Plowman.   

Abstract

BACKGROUND: Fanconi anaemia is a rare disease associated with cellular sensitivity to chemicals (e.g. mitomycin C and diepoxybutane); variable but mild cellular radiosensitivity has also been reported.
MATERIALS AND METHODS: A 32-year-old patient with Fanconi anaemia and tonsillar carcinoma, treated by radiotherapy, was found to exhibit profound clinical radiosensitivity. Confluent, ulcerating oropharyngeal mucositis developed after a conventionally fractionated dose of 34Gy and healing was incomplete by 2 months after cessation of therapy.
RESULTS: Cellular radiosensitivity assays and RPLD studies from this patient did not suggest any major detectable radiosensitivity.
CONCLUSION: There is a discrepancy between the observed clinical radiosensitivity and the usual "predictive" radiosensitivity assays in this patient with Fanconi anaemia.

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Year:  2001        PMID: 11410307     DOI: 10.1016/s0167-8140(01)00370-x

Source DB:  PubMed          Journal:  Radiother Oncol        ISSN: 0167-8140            Impact factor:   6.280


  16 in total

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Journal:  Arch Otolaryngol Head Neck Surg       Date:  2011-09

Review 2.  Unravelling the web of DNA repair disorders.

Authors:  A R Gennery; M O'Driscoll
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Review 3.  Fanconi anaemia.

Authors:  M D Tischkowitz; S V Hodgson
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Review 4.  Squamous cell carcinomas of the head and neck in Fanconi anemia: risk, prevention, therapy, and the need for guidelines.

Authors:  K Scheckenbach; M Wagenmann; M Freund; J Schipper; H Hanenberg
Journal:  Klin Padiatr       Date:  2012-04-13       Impact factor: 1.349

Review 5.  Clinical radiation sensitivity with DNA repair disorders: an overview.

Authors:  Julianne M Pollard; Richard A Gatti
Journal:  Int J Radiat Oncol Biol Phys       Date:  2009-08-01       Impact factor: 7.038

6.  Hypersensitivity to chemoradiation in FANCA carrier with cervical carcinoma-A case report and review of the literature.

Authors:  Igor Sirák; Zuzana Šinkorová; Mária Šenkeříková; Jiří Špaček; Jan Laco; Hana Vošmiková; Stanislav John; Jiří Petera
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Review 7.  The Fanconi anemia protein interaction network: casting a wide net.

Authors:  Meghan A Rego; Frederick W Kolling; Niall G Howlett
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Review 8.  Radioprotection.

Authors:  Joel S Greenberger
Journal:  In Vivo       Date:  2009 Mar-Apr       Impact factor: 2.155

9.  FANCD2-deficient human fibroblasts are hypersensitive to ionising radiation at oxygen concentrations of 0% and 3% but not under normoxic conditions.

Authors:  Verena M Kuhnert; Lisa A Kachnic; Li Li; Martin Purschke; Liliana Gheorghiu; Richard Lee; Kathryn D Held; Henning Willers
Journal:  Int J Radiat Biol       Date:  2009-06       Impact factor: 2.694

10.  Fanconi anemia deficiency stimulates HPV-associated hyperplastic growth in organotypic epithelial raft culture.

Authors:  E E Hoskins; T A Morris; J M Higginbotham; N Spardy; E Cha; P Kelly; D A Williams; K A Wikenheiser-Brokamp; S Duensing; S I Wells
Journal:  Oncogene       Date:  2008-11-17       Impact factor: 9.867

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