Pediatric Dental Care in Fanconi Anemia: A Case Report
Fanconi Anemia (FA) is an autosomal-recessive genetic disease that is linked to Chromosome X, which is reported in studies with pancytopenia, congenital malformations, and a predisposition to develop cancer. FA 1,360,000 births, and is a condition that occurs in heterozygous subjects in 0.5% of the...
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| Veröffentlicht in: | Odovtos 2018-01, Vol.20 (3), p.25-31 |
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| creator | Gaitán-Fonseca DDS, MSc, PhD, César Frías-Muñoz DDS, Maribel Guerrero-de la Torre DDS, Luis Enrique Lemus-Rojero DDS, MSc, Obed Aguilera-Galavíz DDS, MSc, PhD, Luis Alejandro |
| description | Fanconi Anemia (FA) is an autosomal-recessive genetic disease that is linked to Chromosome X, which is reported in studies with pancytopenia, congenital malformations, and a predisposition to develop cancer. FA 1,360,000 births, and is a condition that occurs in heterozygous subjects in 0.5% of the population. In Mexico, there are, to our knowledge, no epidemiological data on FA, and it is thought that many cases are underdiagnosed. This document reports the clinical case of a patient diagnosed with FA who the cardinal signs of this rare pathology. The pediatric approach involved was performed with a preventive and restorative approach, in addition to the design and placement of a palatal shutter. After a follow-up of more than 12 months, a significant reduction in the recurrence of infections, such as otitis, tonsillitis, and pharyngitis, was observed, suggesting a positive influence of the use of the obturator. In turn with the latter, there was a lower need for transfusions, which may also be related to control of the foci of the infection. The pediatric approach employed in to patients with FA may have significant repercussions on both quality of life and on their patients’ general systemic condition, although this is scarcely verifiable due to the rarity of this pathology. |
| doi_str_mv | 10.15517/ijds.2018.33350 |
| format | Article |
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FA 1,360,000 births, and is a condition that occurs in heterozygous subjects in 0.5% of the population. In Mexico, there are, to our knowledge, no epidemiological data on FA, and it is thought that many cases are underdiagnosed. This document reports the clinical case of a patient diagnosed with FA who the cardinal signs of this rare pathology. The pediatric approach involved was performed with a preventive and restorative approach, in addition to the design and placement of a palatal shutter. After a follow-up of more than 12 months, a significant reduction in the recurrence of infections, such as otitis, tonsillitis, and pharyngitis, was observed, suggesting a positive influence of the use of the obturator. In turn with the latter, there was a lower need for transfusions, which may also be related to control of the foci of the infection. The pediatric approach employed in to patients with FA may have significant repercussions on both quality of life and on their patients’ general systemic condition, although this is scarcely verifiable due to the rarity of this pathology.</description><identifier>ISSN: 1659-1046</identifier><identifier>ISSN: 2215-3411</identifier><identifier>EISSN: 2215-3411</identifier><identifier>DOI: 10.15517/ijds.2018.33350</identifier><language>eng</language><publisher>San Jose: Universidad de Costa Rica, Facultad de Odontologia</publisher><subject>Anemia ; Anemia de Fanconi ; Autosomal recessive inheritance ; Cancer ; Case reports ; Congenital defects ; Dental care ; Dentistry ; Diagnosis ; Diagnóstico ; Epidemiology ; Fanconi anemia ; Fanconi syndrome ; Hematology ; Hemoglobin ; Mutation ; Odontología pediátrica ; Otitis ; Pancytopenia ; Patients ; Pediatric dentistry ; Pediatrics ; Pharyngitis ; Quality of life ; Tonsillitis ; Transplants & implants ; Tratamiento ; Treatment</subject><ispartof>Odovtos, 2018-01, Vol.20 (3), p.25-31</ispartof><rights>2018. This work is published under https://creativecommons.org/licenses/by-nc-sa/4.0/deed.es (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>LICENCIA DE USO: Los documentos a texto completo incluidos en Dialnet son de acceso libre y propiedad de sus autores y/o editores. Por tanto, cualquier acto de reproducción, distribución, comunicación pública y/o transformación total o parcial requiere el consentimiento expreso y escrito de aquéllos. Cualquier enlace al texto completo de estos documentos deberá hacerse a través de la URL oficial de éstos en Dialnet. Más información: https://dialnet.unirioja.es/info/derechosOAI | INTELLECTUAL PROPERTY RIGHTS STATEMENT: Full text documents hosted by Dialnet are protected by copyright and/or related rights. This digital object is accessible without charge, but its use is subject to the licensing conditions set by its authors or editors. 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More info: https://dialnet.unirioja.es/info/derechosOAI</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c1697-576f2dcce5dac5d11365e583611e849647671614e32b1155350b4d37a0629a593</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,870,27903,27904</link.rule.ids></links><search><creatorcontrib>Gaitán-Fonseca DDS, MSc, PhD, César</creatorcontrib><creatorcontrib>Frías-Muñoz DDS, Maribel</creatorcontrib><creatorcontrib>Guerrero-de la Torre DDS, Luis Enrique</creatorcontrib><creatorcontrib>Lemus-Rojero DDS, MSc, Obed</creatorcontrib><creatorcontrib>Aguilera-Galavíz DDS, MSc, PhD, Luis Alejandro</creatorcontrib><title>Pediatric Dental Care in Fanconi Anemia: A Case Report</title><title>Odovtos</title><description>Fanconi Anemia (FA) is an autosomal-recessive genetic disease that is linked to Chromosome X, which is reported in studies with pancytopenia, congenital malformations, and a predisposition to develop cancer. FA 1,360,000 births, and is a condition that occurs in heterozygous subjects in 0.5% of the population. In Mexico, there are, to our knowledge, no epidemiological data on FA, and it is thought that many cases are underdiagnosed. This document reports the clinical case of a patient diagnosed with FA who the cardinal signs of this rare pathology. The pediatric approach involved was performed with a preventive and restorative approach, in addition to the design and placement of a palatal shutter. After a follow-up of more than 12 months, a significant reduction in the recurrence of infections, such as otitis, tonsillitis, and pharyngitis, was observed, suggesting a positive influence of the use of the obturator. In turn with the latter, there was a lower need for transfusions, which may also be related to control of the foci of the infection. The pediatric approach employed in to patients with FA may have significant repercussions on both quality of life and on their patients’ general systemic condition, although this is scarcely verifiable due to the rarity of this pathology.</description><subject>Anemia</subject><subject>Anemia de Fanconi</subject><subject>Autosomal recessive inheritance</subject><subject>Cancer</subject><subject>Case reports</subject><subject>Congenital defects</subject><subject>Dental care</subject><subject>Dentistry</subject><subject>Diagnosis</subject><subject>Diagnóstico</subject><subject>Epidemiology</subject><subject>Fanconi anemia</subject><subject>Fanconi syndrome</subject><subject>Hematology</subject><subject>Hemoglobin</subject><subject>Mutation</subject><subject>Odontología pediátrica</subject><subject>Otitis</subject><subject>Pancytopenia</subject><subject>Patients</subject><subject>Pediatric dentistry</subject><subject>Pediatrics</subject><subject>Pharyngitis</subject><subject>Quality of life</subject><subject>Tonsillitis</subject><subject>Transplants & implants</subject><subject>Tratamiento</subject><subject>Treatment</subject><issn>1659-1046</issn><issn>2215-3411</issn><issn>2215-3411</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>FKZ</sourceid><recordid>eNo9kEFLAzEQhYMoWGrvHhc8b80kmeyueFmqVaGglHoO6e4UsrRJTbYH_73bVj0NzLw38-Zj7Bb4FBChuHddm6aCQzmVUiK_YCMhAHOpAC7ZCDRWOXClr9kkpY5zDpUohVIjpj-odbaPrsmeyPd2m81spMz5bG59E7zLak87Zx-yepgkypa0D7G_YVcbu000-a1j9jl_Xs1e88X7y9usXuQN6KrIsdAb0TYNYWsbbAGkRsJSagAqVaVVoQvQoEiKNQyfDNHXqpWF5VpUFis5Zo_nvUPIrafe7KPb2fhtgnXmr3fwLrrQWUPJ1MvV8T2pUHE12O_O9n0MXwdKvenCIfohsRFCImCJohhU_KxqYkgp0ub_CnBz4muOfM2RrznxlT87a2ph</recordid><startdate>20180101</startdate><enddate>20180101</enddate><creator>Gaitán-Fonseca DDS, MSc, PhD, César</creator><creator>Frías-Muñoz DDS, Maribel</creator><creator>Guerrero-de la Torre DDS, Luis Enrique</creator><creator>Lemus-Rojero DDS, MSc, Obed</creator><creator>Aguilera-Galavíz DDS, MSc, PhD, Luis Alejandro</creator><general>Universidad de Costa Rica, Facultad de Odontologia</general><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>LK8</scope><scope>M0S</scope><scope>M7P</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>AGMXS</scope><scope>FKZ</scope></search><sort><creationdate>20180101</creationdate><title>Pediatric Dental Care in Fanconi Anemia: A Case Report</title><author>Gaitán-Fonseca DDS, MSc, PhD, César ; Frías-Muñoz DDS, Maribel ; Guerrero-de la Torre DDS, Luis Enrique ; Lemus-Rojero DDS, MSc, Obed ; Aguilera-Galavíz DDS, MSc, PhD, Luis Alejandro</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c1697-576f2dcce5dac5d11365e583611e849647671614e32b1155350b4d37a0629a593</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2018</creationdate><topic>Anemia</topic><topic>Anemia de Fanconi</topic><topic>Autosomal recessive inheritance</topic><topic>Cancer</topic><topic>Case reports</topic><topic>Congenital defects</topic><topic>Dental care</topic><topic>Dentistry</topic><topic>Diagnosis</topic><topic>Diagnóstico</topic><topic>Epidemiology</topic><topic>Fanconi anemia</topic><topic>Fanconi syndrome</topic><topic>Hematology</topic><topic>Hemoglobin</topic><topic>Mutation</topic><topic>Odontología pediátrica</topic><topic>Otitis</topic><topic>Pancytopenia</topic><topic>Patients</topic><topic>Pediatric dentistry</topic><topic>Pediatrics</topic><topic>Pharyngitis</topic><topic>Quality of life</topic><topic>Tonsillitis</topic><topic>Transplants & implants</topic><topic>Tratamiento</topic><topic>Treatment</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Gaitán-Fonseca DDS, MSc, PhD, César</creatorcontrib><creatorcontrib>Frías-Muñoz DDS, Maribel</creatorcontrib><creatorcontrib>Guerrero-de la Torre DDS, Luis Enrique</creatorcontrib><creatorcontrib>Lemus-Rojero DDS, MSc, Obed</creatorcontrib><creatorcontrib>Aguilera-Galavíz DDS, MSc, PhD, Luis Alejandro</creatorcontrib><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Natural Science Collection</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>ProQuest Biological Science Collection</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Biological Science Database</collection><collection>Publicly Available Content Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>Dialnet (Open Access Full Text)</collection><collection>Dialnet</collection><jtitle>Odovtos</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Gaitán-Fonseca DDS, MSc, PhD, César</au><au>Frías-Muñoz DDS, Maribel</au><au>Guerrero-de la Torre DDS, Luis Enrique</au><au>Lemus-Rojero DDS, MSc, Obed</au><au>Aguilera-Galavíz DDS, MSc, PhD, Luis Alejandro</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pediatric Dental Care in Fanconi Anemia: A Case Report</atitle><jtitle>Odovtos</jtitle><date>2018-01-01</date><risdate>2018</risdate><volume>20</volume><issue>3</issue><spage>25</spage><epage>31</epage><pages>25-31</pages><issn>1659-1046</issn><issn>2215-3411</issn><eissn>2215-3411</eissn><abstract>Fanconi Anemia (FA) is an autosomal-recessive genetic disease that is linked to Chromosome X, which is reported in studies with pancytopenia, congenital malformations, and a predisposition to develop cancer. FA 1,360,000 births, and is a condition that occurs in heterozygous subjects in 0.5% of the population. In Mexico, there are, to our knowledge, no epidemiological data on FA, and it is thought that many cases are underdiagnosed. This document reports the clinical case of a patient diagnosed with FA who the cardinal signs of this rare pathology. The pediatric approach involved was performed with a preventive and restorative approach, in addition to the design and placement of a palatal shutter. After a follow-up of more than 12 months, a significant reduction in the recurrence of infections, such as otitis, tonsillitis, and pharyngitis, was observed, suggesting a positive influence of the use of the obturator. In turn with the latter, there was a lower need for transfusions, which may also be related to control of the foci of the infection. The pediatric approach employed in to patients with FA may have significant repercussions on both quality of life and on their patients’ general systemic condition, although this is scarcely verifiable due to the rarity of this pathology.</abstract><cop>San Jose</cop><pub>Universidad de Costa Rica, Facultad de Odontologia</pub><doi>10.15517/ijds.2018.33350</doi><tpages>7</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Anemia Anemia de Fanconi Autosomal recessive inheritance Cancer Case reports Congenital defects Dental care Dentistry Diagnosis Diagnóstico Epidemiology Fanconi anemia Fanconi syndrome Hematology Hemoglobin Mutation Odontología pediátrica Otitis Pancytopenia Patients Pediatric dentistry Pediatrics Pharyngitis Quality of life Tonsillitis Transplants & implants Tratamiento Treatment |
| title | Pediatric Dental Care in Fanconi Anemia: A Case Report |
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