Abstract
Fanconi anemia is a rare disease, which is characterized by decreased production of all blood cell types. Fanconi anemia is the most common inherited form of aplastic anemia. Congenital abnormalities of the eyes, ears, and heart, malformed or absent kidney, urogenital system involvement are common. There is a delay in physical development. Intelligence in patients with Fanconi anemia is usually normal. The most serious problems associated with Fanconi anemia include the gradual development of bone marrow disorders. Many patients with Fanconi anemia develop leukemia or myelodysplastic syndrome, as well as other oncological diseases. Oral manifestations in patients with Fanconi anemia can be classified as gingivitis, periodontitis, dental caries, dental anomalies, soft tissue lesions, oral cancer, and lesions of the tongue. Patients with Fanconi anemia have increased predisposition to squamous cell carcinoma of the head and neck and oral cancer. The interdisciplinary team of medical and dental specialists must be included in the medical and dental treatment of patients with Fanconi anemia. For proper dental care of patients with Fanconi anemia, the close cooperation of dental specialists, including orthodontists, pedodontists, prosthetists, oral surgeons, as well as specialists in periodontology and oral diseases, is of particular importance.
References
Deaconu A, Vodă D, Bulucea D. Fanconi anemia — case report of rare aplastic anemia at child. Acta Medica Marisiensis. 2014;60(3):125–128. Available from: https://doi.org/10.2478/amma-2014-0027
Nepal M, Che R, Zhang J, Ma C, Fei P. Fanconi anemia signaling and cancer. trends in cancer. 2017;3(12):840–856. Available from: https://doi.org/10.1016/j.trecan.2017.10.005
Zareifar S, Dastsooz H, Shahriari M, Faghihi MA, Shekarkhar G, Bordbar M, et al. A novel frame-shift deletion in FANCF gene causing autosomal recessive Fanconi anemia: a case report. BMC Medical Genetics. 2019;20(1). Available from: https://doi.org/10.1186/s12881-019-0855-2
Yang X, Zhang X, Jiao J, Zhang F, Pan Y, Wang Q, et al. Rare variants in FANCA induce premature ovarian insufficiency. Human Genetics. 2019;138(11-12):1227–1236. Available from: https://doi.org/10.1007/s00439-019-02059-9
Van den Hondel D, Wijers CHW, van Bever Y, de Klein A, Marcelis CLM, de Blaauw I, et al. Patients with anorectal malformation and upper limb anomalies: genetic evaluation is warranted. European Journal of Pediatrics. 2015;175(4):489–497. Available from: https://doi.org/10.1007/s00431-015-2655-9
Savage SA, Walsh MF. Myelodysplastic syndrome, acute myeloid leukemia, and cancer surveillance in Fanconi anemia. Hematology/Oncology Clinics of North America. 2018;32(4):657–668. Available from: https://doi.org/10.1016/j.hoc.2018.04.002
Furquim CP, Pivovar A, Amenábar JM, Bonfim C, Torres-Pereira CC. Oral cancer in Fanconi anemia: review of 121 cases. Critical Reviews in Oncology/Hematology. 2018;125:35–40. Available from: https://doi.org/10.1016/j.critrevonc.2018.02.013
Touil D, Bouhouch R, Chebil RB, Oualha L, Douki N. Gingival bleeding in a child with Fanconi anemia: a case report and literature review. Case Reports in Dentistry. 2020;2020:3161053. Available from: https://doi.org/10.1155/2020/3161053
Gaitán-Fonseca C, Frías-Muñoz M, Guerrero-de la Torre LE, et al. Pediatric dental care in Fanconi anemia: a case report. Odovtos-Int J Dent Sc. 2018;20(3):25-31. Available from: https://www.medigraphic.com/cgi-bin/new/resumenI.cgi?IDARTICULO=82172
Ballardin BS, Mobile RZ, Coracin FL, Ribeiro LL, Bonfim CMS, Schussel JL, et al. A case series of medication‐related fibrovascular hyperplasia following hematopoietic stem cell transplantation for Fanconi anemia. Pediatric Transplantation. 2020. Available from: https://doi.org/10.1111/petr.13947
Toptan T, Brusadelli MG, Turpin B, Witte DP, Surrallés J, Velleuer E, et al. Limited detection of human polyomaviruses in Fanconi anemia related squamous cell carcinoma. PLOS ONE. 2018;13(12):e0209235. Available from: https://doi.org/10.1371/journal.pone.0209235
Pivovar A, Furquim CP, Bonfim C, Torres-Pereira CC. Mouth examination performance by children’s parents and by adolescents in Fanconi anemia. Pediatric Blood & Cancer. 2017;64(11):e26622. Available from: https://doi.org/10.1002/pbc.26622
Ambarkova V. Oral cancer. Annals of Dentistry and Oral Disorders. 2019;2:110. Available from: http://gslpublishers.org/journals/current-issues/119-Article.pdf
Furquim CP, Soares GMS, Ribeiro LL, Azcarate-Peril MA, Butz N, Roach J, et al. The salivary microbiome and oral cancer risk: a pilot study in Fanconi anemia. Journal of Dental Research. 2016;96(3):292–299. Available from: https://doi.org/10.1177/0022034516678169
Velleuer E, Dietrich R, Pomjanski N, Santana Almeida Araujo IK, Silva de Araujo BE, Sroka I, et al. Diagnostic accuracy of brush biopsy–based cytology for the early detection of oral cancer and precursors in Fanconi anemia. Cancer Cytopathology. 2020;128(6):403–413. Available from: https://doi.org/10.1002/cncy.22249
Lyko K, Lemes AL, Bonfim C, Torres-Pereira CC, Amenábar JM. Oral health status in children and adolescents with Fanconi anemia. Special Care in Dentistry. 2015;36(2):71–74. Available from: https://doi.org/10.1111/scd.12151
Kaul R, Jain P, Saha S, Sarkar S. Fanconi anemia in pediatric dentistry: case report and review of literature. SRM J Res Dent Sci. 2017;8(2):92-96. Available from: https://www.srmjrds.in/text.asp?2017/8/2/92/207658
Miranda F, Garib D, Netto BA, Lucena FS de, da Silva Santos PS. Orthodontic intervention in Fanconi’s anemia: A case report. Special Care in Dentistry. 2020;40(4):382–389. Available from: https://doi.org/10.1111/scd.12487
Goswami M, Bhushan U, Goswami M. Dental perspective of rare disease of fanconi anemia: case report with review. Clinical Medicine Insights: Case Reports. 2016;9:CCRep.S37931. Available from: https://doi.org/10.4137/CCRep.S37931
Perdoncini NN, Furquim CP, Bonfim CMS, Soares GMS, Torres-Pereira CC. Self-perception of periodontal health status among individuals with Fanconi anemia. Hematology, Transfusion and Cell Therapy. 2020. Available from: https://doi.org/10.1016/j.htct.2020.07.009
De Araujo M, de Oliveira Ribas M, Koubik A, Mattioli T, de Lima A, França B. Fanconi’s anemia: clinical and radiographic oral manifestations. Oral Diseases. 2007;13(3):291–295. Available from: https://doi.org/10.1111/j.1601-0825.2006.01282.x
Tekcicek M, Tavil B, Cakar A et al. Oral and dental findings in children with Fanconi anemia. Pediatr Dent. 2007;29(3):248-252. Available from: https://www.ingentaconnect.com/openurl?genre=article&issn=0164-1263&volume=29&issue=3&spage=248&aulast=Tekcicek
Açikgöz A, Özden FO, Fisgin T, Açikgöz G, Duru F, Yarali N, et al. Oral and dental findings in Fanconi’s anemia. Pediatric Hematology and Oncology. 2005;22(6):531–539. Available from: https://doi.org/10.1080/08880010591002413
Lee HJ, Park S, Kang HJ, Jun JK, Lee JA, Lee DS, et al. A case report of Fanconi anemia diagnosed by genetic testing followed by prenatal diagnosis. Annals of Laboratory Medicine. 2012;32(5):380–384. Available from: https://doi.org/10.3343/alm.2012.32.5.380
Panovska-Stavridis I, Pivkova-Veljanovska A, Ridova N, Stojanovski Z, Cadievski L, Trajkova S, et al. Molecular monitoring in acute myeloid leukemia patients undergoing matched unrelated donor – hematopoietic stem cell transplantation: single center experience. PRILOZI. 2020;41(3):5–12. Available from: https://doi.org/10.2478/prilozi-2020-0040
Ebens CL, MacMillan ML, Wagner JE. Hematopoietic cell transplantation in Fanconi anemia: current evidence, challenges and recommendations. Expert Review of Hematology. 2016;10(1):81–97. Available from: https://doi.org/10.1080/17474086.2016.1268048
Mehta PA, Davies SM, Leemhuis T, Myers K, Kernan NA, Prockop SE, et al. Radiation-free, alternative-donor HCT for Fanconi anemia patients: results from a prospective multi-institutional study. Blood. 2017;129(16):2308–2315. Available from: https://doi.org/10.1182/blood-2016-09-743112
Quinn TJ, Ding X, Li X, Wilson GD, Buelow K, Sivananthan A, et al. Amelioration of mucositis in proton therapy of Fanconi anemia Fanca−/− mice by JP4-039. In Vivo. 2019;33(6):1757–1766. Available from: https://doi.org/10.21873/invivo.11666
Abram TJ, Pickering CR, Lang AK, Bass NE, Raja R, Meena C, et al. Risk stratification of oral potentially malignant disorders in Fanconi anemia patients using autofluorescence imaging and cytology-on-a chip assay. Translational Oncology. 2018;11(2):477–486. Available from: https://doi.org/10.1016/j.tranon.2018.01.014

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