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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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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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.</description><identifier>ISSN: 1354-523X</identifier><identifier>EISSN: 1601-0825</identifier><identifier>DOI: 10.1111/j.1601-0825.2006.01282.x</identifier><identifier>PMID: 17448211</identifier><language>eng</language><publisher>Oxford, UK: Blackwell Publishing Ltd</publisher><subject>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</subject><ispartof>Oral diseases, 2007-05, Vol.13 (3), p.291-295</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4052-e82f6da467a721ff8e4caf2abe6fcb3160f6eb8d5e18a303af5367fbc419b1213</citedby><cites>FETCH-LOGICAL-c4052-e82f6da467a721ff8e4caf2abe6fcb3160f6eb8d5e18a303af5367fbc419b1213</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fj.1601-0825.2006.01282.x$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fj.1601-0825.2006.01282.x$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27903,27904,45553,45554</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17448211$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>De Araujo, MR</creatorcontrib><creatorcontrib>De Oliveira Ribas, M</creatorcontrib><creatorcontrib>Koubik, ACGA</creatorcontrib><creatorcontrib>Mattioli, T</creatorcontrib><creatorcontrib>De Lima, AAS</creatorcontrib><creatorcontrib>França, BHS</creatorcontrib><title>Fanconi's anemia: clinical and radiographic oral manifestations</title><title>Oral diseases</title><addtitle>Oral Dis</addtitle><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.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Dentistry</subject><subject>Fanconi Anemia - complications</subject><subject>Fanconi's anemia</subject><subject>Female</subject><subject>Gingival Hemorrhage - etiology</subject><subject>Humans</subject><subject>Male</subject><subject>manifestation</subject><subject>mouth</subject><subject>Mouth Diseases - etiology</subject><subject>oral</subject><subject>Pigmentation Disorders - etiology</subject><subject>radiography</subject><subject>Radiography, Panoramic</subject><subject>Tooth Abnormalities - diagnostic imaging</subject><subject>Tooth Abnormalities - etiology</subject><issn>1354-523X</issn><issn>1601-0825</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkE1PwzAMhiME4vsvoJ7g1JKPNs2QEGKDDaRpu4DgFrlpAhn9GMkmtn9Pyia44ost-31t60EoIjghIS5nCeGYxFjQLKEY8wQTKmiy2kGHv4PdULMsjTPKXg_QkfczjEneY3QfHZA8TQUl5BDdDKFRbWMvfASNri1cRaqyjVVQhUYZOSht--Zg_m5V1LrQraGxRvsFLGzb-BO0Z6Dy-nSbj9Hz8P5p8BCPp6PHwe04VinOaKwFNbyElOeQU2KM0KkCQ6HQ3KiChacN14UoM00EMMzAZIznplAp6RWEEnaMzjd75679XIbzsrZe6aoKX7dLL3OcEkwpD0KxESrXeu-0kXNna3BrSbDs4MmZ7BjJjpHs4MkfeHIVrGfbG8ui1uWfcUsrCK43gi9b6fW_F8vp3WNXBX-88Vu_0KtfP7gPyXOWZ_JlMpK9_qTXHzIhJ-wb1xWMeA</recordid><startdate>200705</startdate><enddate>200705</enddate><creator>De Araujo, MR</creator><creator>De Oliveira Ribas, M</creator><creator>Koubik, ACGA</creator><creator>Mattioli, T</creator><creator>De Lima, AAS</creator><creator>França, BHS</creator><general>Blackwell Publishing Ltd</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>200705</creationdate><title>Fanconi's anemia: clinical and radiographic oral manifestations</title><author>De Araujo, MR ; De Oliveira Ribas, M ; Koubik, ACGA ; Mattioli, T ; De Lima, AAS ; França, BHS</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4052-e82f6da467a721ff8e4caf2abe6fcb3160f6eb8d5e18a303af5367fbc419b1213</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2007</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Dentistry</topic><topic>Fanconi Anemia - complications</topic><topic>Fanconi's anemia</topic><topic>Female</topic><topic>Gingival Hemorrhage - etiology</topic><topic>Humans</topic><topic>Male</topic><topic>manifestation</topic><topic>mouth</topic><topic>Mouth Diseases - etiology</topic><topic>oral</topic><topic>Pigmentation Disorders - etiology</topic><topic>radiography</topic><topic>Radiography, Panoramic</topic><topic>Tooth Abnormalities - diagnostic imaging</topic><topic>Tooth Abnormalities - etiology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>De Araujo, MR</creatorcontrib><creatorcontrib>De Oliveira Ribas, M</creatorcontrib><creatorcontrib>Koubik, ACGA</creatorcontrib><creatorcontrib>Mattioli, T</creatorcontrib><creatorcontrib>De Lima, AAS</creatorcontrib><creatorcontrib>França, BHS</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Oral diseases</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>De Araujo, MR</au><au>De Oliveira Ribas, M</au><au>Koubik, ACGA</au><au>Mattioli, T</au><au>De Lima, AAS</au><au>França, BHS</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Fanconi's anemia: clinical and radiographic oral manifestations</atitle><jtitle>Oral diseases</jtitle><addtitle>Oral Dis</addtitle><date>2007-05</date><risdate>2007</risdate><volume>13</volume><issue>3</issue><spage>291</spage><epage>295</epage><pages>291-295</pages><issn>1354-523X</issn><eissn>1601-0825</eissn><abstract>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.</abstract><cop>Oxford, UK</cop><pub>Blackwell Publishing Ltd</pub><pmid>17448211</pmid><doi>10.1111/j.1601-0825.2006.01282.x</doi><tpages>5</tpages></addata></record> |
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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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