Fanconi Anemia: main oral manifestations

D'Agulham, Anna Clara Duszczak; Chaiben, Cassiano Lima; Lima, Antônio Adílson Soares de; Torres‐Pereira, Cassius Carvalho; Machado, Maria Ângela Naval

doi:10.1590/1981-8637201400030000082275

Cited by 7 publications

(4 citation statements)

References 33 publications

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“…5 Another reason is that leukocyte deficiency, reducing the immunity of these individuals, leads to the development of ulcerated lesions. 21 Traumatic nonulcerated lesions and petechiae are common in these individuals and are normally related to low platelet counts.…”

Section: Discussionmentioning

confidence: 99%

Dental Perspective of Rare Disease of Fanconi Anemia: Case Report with Review

Goswami

Bhushan

Goswami³

2016

Clin Med Insights Case Rep

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Fanconi anemia is an extremely rare genetic disease characterized by chromosomal instability that induces congenital alterations in individuals. It causes defective hemopoiesis ultimately leading to bone marrow failure. Patients are susceptible to recurrent infections and increased risk of hemorrhage, as well as delayed and poor wound healing. Herein, we report a case of Fanconi anemia in which various classical signs of the disease were present. The patient has been on regular follow-up since three and a half years for management of dental problems. The different aspects of this rare disorder are discussed with emphasis on oral manifestations and their influence on the general health of affected patients. Due to an increased susceptibility to developing cancers in this specific population, it is imperative for pediatric dentists to know about the common oral manifestations and potentially cancerous lesions, in order to make an early diagnosis and provide comprehensive care and maintenance of oral health in affected individuals.

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Section: Discussionmentioning

confidence: 99%

Dental Perspective of Rare Disease of Fanconi Anemia: Case Report with Review

Goswami

Bhushan

Goswami³

2016

Clin Med Insights Case Rep

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“…The use of corticosteroids and androgens provide a partial answer to these hematological complications; however, transfusions and antiinfective therapies are necessary. Curative and definitive treatment in these patients comprises bone-marrow transplantation (2,3).…”

Section: Introductionmentioning

confidence: 99%

Pediatric Dental Care in Fanconi Anemia: A Case Report

Gaitán-Fonseca

Frías-Muñoz

Torre

et al. 2018

Odovtos - Int J Dent Sc

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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.

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“…El diagnóstico preciso se lleva a cabo generalmente entre los 5 y 10 años de edad, debido a que un tercio de los pacientes afectados no presenta ninguna anormalidad evidente al nacer y durante la primera infancia. 6 Los hallazgos hematológicos anormales y las alteraciones físicas características sugieren la presencia de la enfermedad, la que se confirma mediante el estudio de fragilidad o rotura cromosómica, que es característico de la pancitopenia de Fanconi. 7,8 Para el diagnóstico prenatal de la enfermedad, es posible realizar una prueba de fragilidad cromosómica a partir de las 8-12 semanas de gestación, en las vellosidades coriónicas, o a las 16 semanas, en las células de líquido amniótico.…”

Section: Introductionunclassified

“…Los tejidos blandos y mucosas se encontraban bien hidratadas y con buena coloración, y una buena implantación de los frenillos. Oclusalmente, existía una relación molar y canina clase I, con alineación, resalte y entrecruzamiento normales, aunque con espacios en el sector antero-superior, y líneas medias coincidentes(Figura 3,4,5,6). No se reportó ningún hábito oral pernicioso.…”

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Manejo estomatológico de Anemia de Fanconi en Odontología Pediátrica. Reporte de caso.

Rosales

Ruiz

Pozos

et al. 2018

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Resumen (español):La anemia de Fanconi (FA) es una enfermedad autosómica recesiva que se caracteriza por presentar anemia aplásica, además de malformaciones congénitas renales, cardíacas, esqueléticas, dermatológicas, y aumento en la incidencia de neoplasias malignas. Los pacientes con FA son más susceptibles de presentar sangrado e infecciones, síntomas relacionados con trombocitopenia y neutropenia. El propósito del presente reporte es describir el manejo estomatológico proporcionado a un niño de 9 años y 9 meses de edad con diagnóstico de Anemia de Fanconi, en la Clínica del Posgrado en Estomatología Pediátrica. [ABSTRACT FROM AUTHOR] Abstract Fanconi Anemia (FA) is an autosomal recessive disorder, characterized by aplastic anemia, congenital malformations in kidney, heart, skeletal and skin structures, and with increased incidence to malignancies. FA patients are likely to have bleeding and infections, which are associated with thrombocytopenia and neutropenia symptoms. The purpose of this paper is to report the stomatological management delivered to a child of 9 years and 9 months of age, diagnosed with Fanconi Anemia, in the clinic of Pediatric Dentistry. [ABSTRACT FROM AUTHOR]

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Fanconi Anemia: main oral manifestations

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Cited by 7 publications

References 33 publications

Dental Perspective of Rare Disease of Fanconi Anemia: Case Report with Review

Dental Perspective of Rare Disease of Fanconi Anemia: Case Report with Review

Pediatric Dental Care in Fanconi Anemia: A Case Report

Manejo estomatológico de Anemia de Fanconi en Odontología Pediátrica. Reporte de caso.

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