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    Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.

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    Authors
    Evans, D Gareth R
    Ladusans, E J
    Rimmer, S
    Burnell, L D
    Thakker, N
    Farndon, P A
    Affiliation
    Department of Medical Genetics, St Mary's Hospital, Manchester.
    Issue Date
    1993-06
    
    Metadata
    Show full item record
    Abstract
    There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. We have been able to show the relative frequencies of these problems in a population based study of 84 cases in the north west of England. The major complications of basal cell carcinomas and jaw cysts occur in over 90% of patients by 40 years of age, but may both occur before 10 years of age. Less well described complications are ovarian calcification or fibroma (24%), medulloblastoma (5%), cardiac fibroma (3%), cleft palate (5%), and ophthalmic abnormalities such as squint or cataract (26%). This study more clearly defines the possible complications of the syndrome and gives clearer guidelines for counselling and screening affected and at risk persons.
    Citation
    Complications of the naevoid basal cell carcinoma syndrome: results of a population based study. 1993, 30 (6):460-4 J. Med. Genet.
    Journal
    Journal of Medical Genetics
    URI
    http://hdl.handle.net/10541/100334
    DOI
    10.1136/jmg.30.6.460
    PubMed ID
    8326488
    Type
    Article
    Language
    en
    ISSN
    0022-2593
    ae974a485f413a2113503eed53cd6c53
    10.1136/jmg.30.6.460
    Scopus Count
    Collections
    All Paterson Institute for Cancer Research

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