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.
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 melaninskin pigmentation, skin vascular and ocular anomalies. Melaninpigmentation 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.
Authors: Ruby Khoury; Sharon Sauter; Melinda Butsch Kovacic; Adam S Nelson; Kasiani C Myers; Parinda A Mehta; Stella M Davies; Susanne I Wells Journal: Viruses Date: 2018-01-20 Impact factor: 5.048