Oral manifestations of Fanconi's anaemia: a case report.
BACKGROUND
Fanconi's anaemia (FA) is a rare, inherited autosomal recessive genetic disease characterized by progressive bone marrow failure, congenital abnormalities, and high predisposition to malignancies. The incidence of the disease is unusually high in the Afrikaner population of South Africa with calculated heterozygote prevalence being approximately 1 in 77. This report describes the oral clinical and radiological findings in a 16 year old male with FA.


METHODS
Medical and dental history was noted, the oral mucosa was examined and the teeth were screened for fillings and caries. Periodontal examination consisted of: measurement of probing depths, recession, mobility of teeth, furcation involvement, plaque index and gingival index. An orthopantomograph was taken.


RESULTS
There was a generalized black hyper-pigmentation on the buccal mucosa, tongue and palate. The patient had generalized gingival recession and most of the periodontal pockets were deeper than 5mm. Several teeth were mobile and the orthopantomograph showed loss of alveolar bone in the furcation areas of most posterior teeth. A diagnosis of severe generalized periodontitis associated with systemic disease was made.


CONCLUSIONS
Patients with Fanconi's anaemia are susceptible to severe dental and periodontal diseases.
Department of Oral Medicine and Periodontology, University of the Western Cape, Cape Town, South Africa. 2558431@uwc.ac.za
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Saleh
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Stephen
D000293:Adolescent; D016301:Alveolar Bone Loss / Q000209:etiology; D003774:Dental Plaque Index; D005199:Fanconi Anemia / Q000150:complications*; D017823:Furcation Defects / Q000209:etiology; D005889:Gingival Recession / Q000209:etiology; D006801:Humans; D017495:Hyperpigmentation / Q000209:etiology*; D008297:Male; D009059:Mouth Diseases / Q000209:etiology*; D010510:Periodontal Diseases / Q000209:etiology*; D010512:Periodontal Index; D010514:Periodontal Pocket / Q000209:etiology; D014086:Tooth Mobility / Q000209:etiology