Fanconi's anemia: clinical and radiographic oral manifestations

Background:  Fanconi's anemia (FA) is a rare autosomal recessive disorder characterized by progressive bone marrow failure, congenital abnormalities, and predisposition to malignancies. There are 11 genetic subtypes characterized by complementation groups – FA‐ A, B, C, D1, D2, E, F, G, I, J, a...

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Veröffentlicht in:Oral diseases 2007-05, Vol.13 (3), p.291-295
Hauptverfasser: De Araujo, MR, De Oliveira Ribas, M, Koubik, ACGA, Mattioli, T, De Lima, AAS, França, BHS
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container_end_page 295
container_issue 3
container_start_page 291
container_title Oral diseases
container_volume 13
creator De Araujo, MR
De Oliveira Ribas, M
Koubik, ACGA
Mattioli, T
De Lima, AAS
França, BHS
description Background:  Fanconi's anemia (FA) is a rare autosomal recessive disorder characterized by progressive bone marrow failure, congenital abnormalities, and predisposition to malignancies. There are 11 genetic subtypes characterized by complementation groups – FA‐ A, B, C, D1, D2, E, F, G, I, J, and L. Objective:  To evaluate and describe clinical, oral and radiographic manifestations of patients with FA. Methods:  A quantitative analysis of clinical manifestations, oral lesions and panoramic radiographs was performed in 33 patients. Results:  Clinical manifestations included melanin skin pigmentation, skin vascular and ocular anomalies. Melanin pigmentation on oral mucosa, traumatic lesions, gingival bleeding, dental biofilm and gingival alterations were the main oral manifestations that were found. Oral and clinical manifestations were not dependent on patient's sex. No significant statistical difference between females and males was detected. Dental anomalies were not remarkable either at clinical or at radiographic examinations. Although several dental anomalies were observed in patients with FA, the correlation between this disease was not established from this study. Panoramic radiographs showed agenesis, taurodontism, radicular anomalies such as dilaceration, tapering, and foreshortening. Conclusion:  This study suggests that gingival alterations are associated with defective oral hygiene but not with hematologic conditions. It also helps elucidate oral manifestations of FA. These patients are living longer and need special dental care.
doi_str_mv 10.1111/j.1601-0825.2006.01282.x
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There are 11 genetic subtypes characterized by complementation groups – FA‐ A, B, C, D1, D2, E, F, G, I, J, and L. Objective:  To evaluate and describe clinical, oral and radiographic manifestations of patients with FA. Methods:  A quantitative analysis of clinical manifestations, oral lesions and panoramic radiographs was performed in 33 patients. Results:  Clinical manifestations included melanin skin pigmentation, skin vascular and ocular anomalies. Melanin pigmentation on oral mucosa, traumatic lesions, gingival bleeding, dental biofilm and gingival alterations were the main oral manifestations that were found. Oral and clinical manifestations were not dependent on patient's sex. No significant statistical difference between females and males was detected. Dental anomalies were not remarkable either at clinical or at radiographic examinations. Although several dental anomalies were observed in patients with FA, the correlation between this disease was not established from this study. Panoramic radiographs showed agenesis, taurodontism, radicular anomalies such as dilaceration, tapering, and foreshortening. Conclusion:  This study suggests that gingival alterations are associated with defective oral hygiene but not with hematologic conditions. It also helps elucidate oral manifestations of FA. 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There are 11 genetic subtypes characterized by complementation groups – FA‐ A, B, C, D1, D2, E, F, G, I, J, and L. Objective:  To evaluate and describe clinical, oral and radiographic manifestations of patients with FA. Methods:  A quantitative analysis of clinical manifestations, oral lesions and panoramic radiographs was performed in 33 patients. Results:  Clinical manifestations included melanin skin pigmentation, skin vascular and ocular anomalies. Melanin pigmentation on oral mucosa, traumatic lesions, gingival bleeding, dental biofilm and gingival alterations were the main oral manifestations that were found. Oral and clinical manifestations were not dependent on patient's sex. No significant statistical difference between females and males was detected. Dental anomalies were not remarkable either at clinical or at radiographic examinations. Although several dental anomalies were observed in patients with FA, the correlation between this disease was not established from this study. Panoramic radiographs showed agenesis, taurodontism, radicular anomalies such as dilaceration, tapering, and foreshortening. Conclusion:  This study suggests that gingival alterations are associated with defective oral hygiene but not with hematologic conditions. It also helps elucidate oral manifestations of FA. 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source MEDLINE; Wiley Online Library Journals Frontfile Complete
subjects Adolescent
Adult
Child
Child, Preschool
Dentistry
Fanconi Anemia - complications
Fanconi's anemia
Female
Gingival Hemorrhage - etiology
Humans
Male
manifestation
mouth
Mouth Diseases - etiology
oral
Pigmentation Disorders - etiology
radiography
Radiography, Panoramic
Tooth Abnormalities - diagnostic imaging
Tooth Abnormalities - etiology
title Fanconi's anemia: clinical and radiographic oral manifestations
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