A Anemia de Fanconi (AF) é unha doenza xenética que afecta a nenos e adultos de e leucemias, insuficiencia da medula ósea progresiva (anemia aplásica). Keywords: Fanconi anemia, Body composition, Hematopoietic stem cell .. Medeiros L.A., Pasquini R. Anemia aplásica adquirida e anemia de. PDF | A Anemia de Fanconi é uma desordem genética recessiva e rara Anemia aplásica pela falência progressiva da medula óssea.

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When the phase angle was analyzed, the majority of the adult patients had normal values. Considering anthropometric data and BMI of all patients, Caries There are fandoni reports in the literature, about the aaplasica of caries in this population. As FA patients are more prone to malnutrition not only due to treatment but also because of the metabolic burden related to the disease, and as they might have higher risk of fanconj, this study investigated whether changes in body composition are evident after transplantation and whether there are differences between patients submitted to transplant and those who are not.

Table 1 Demographic characteristics of patients with Fanconi anemia. Fanconi’s anemia with squamous cell carcinoma: It was applied according to the instructions provided by the guide for bioelectrical impedance analysis. The clinical oral findings described in the literature include periodontal changes, such as gingivitis and aggressive periodontitis, recurrent aphthous ulcers and traumatic lesions.

Consequences of malnutrition include increased complications after surgeries and prolonged hospitalization resulting in higher exposure to infectious agents, a reduced response to treatment, a poorer quality of life and ultimately a worse prognosis.

Fanconi anemia diagnosis and the diepoxybutane DEB fahconi.

Periodontal disease, caries lesions and soft tissue, as well as dental alterations such as agenesias and supernumerary teeth, are the most common oral manifestations in this population. Tongue lesions There are many alterations in the tongue of individuals with FA.

Body composition of Fanconi anemia patients after hematopoietic stem cell transplantation

Molecular biology of Fanconi anemia: The mean phase angle was 6. Aplsaica occurs due to the low tolerance of individuals with FA to chemotherapy and radiotherapy, due to the defective DNA repair mechanism Senescence, together with apoptosis, may constitute a major mechanism of haemopoietic cell depletion occurred in bone marrow failure. Demographic data are presented as percentages, the corresponding number of the population, mean, and standard deviation.

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Squamous cell carcinoma of the tongue in a year-old girl with Fanconi anemia.

Fanconi Anemia: main oral manifestations

However, a few cases have occurred in which older patients have died without ever developing them. However, myelomonocytic and acute monocytic are the most common subtypes observed. Fanconi Anemia is a recessive and rare genetic disorder, characterized by chromosomal instability that induces congenital alterations in individuals. Results Demographic characteristics Sixty-eight patients were recruited but aaplasica were excluded due to the exclusion criteria thus the total study population was 63 patients — 32 male and 31 female.

Recent studies have demonstrated a mean frequency of 1: J Clin Endocrinol Metab. Bone marrow transplantation is a good treatment alternative for the correction of hematological alterations in Fanconi Anemia Aggressive periodontitis associated with Fanconi’s anemia: Early diagnosis and prospective evaluation of patients may contribute to a better analysis of the development of SCC. Oral findings in Fanconi’s anemia: Introduction Fanconi anemia FA anemi a rare recessive genetic disease, usually inherited in an autosomal fanconl manner, linked to bone marrow failure and an increased risk of developing a tumor.

Squamous cell carcinoma of the tongue in a child with Fanconi anemia: Dental anomalies In radiographic studies, diverse dental anomalies have been observed in this population.

Fanconi anemia is a rare fancini disease linked to bone marrow failure; a possible treatment is hematopoietic stem cell transplantation.

Anemia de Fanconi – Wikipedia, a enciclopedia libre

Pediatr Pathol Lab Med. It is important to emphasize that changes in electrical properties of tissues and membrane ion conductivity reduce the phase angle in the post-HSCT period, however, the electrical properties tend to be restored with nutritional recovery. Fanconi Anemia FA is a recessive genetic disorder, in which individuals present congenital alterations associated with consanguinity.

The last major haematological complication associated with FA is bone marrow failure, defined as inadequate blood cell production.


Anemia de Fanconi

Patients who did not receive HSCT and were older than 16 ane,ia old did not present short stature. The deficiencies cause increased risk of hemorrhage and recurrent infectionsrespectively. Comorbidities were present in The anthropometric data of all groups are shown in Figure 2. The continuous and daily consumption of sucrose, presence of specific cariogenic microbiota, low socio-economic condition and reduced access to dental care are relevant factors for the development of caries, a multifactorial disease Fanconi anemia, Body composition, Hematopoietic stem cell transplant.

These procedures associated with myelosuppression and complications arising from post-BMT, such as graft-versus-host disease GVHD and infections, are factors that significantly elevate the risk for development of neoplasias, mainly squamous cell carcinoma in the region of the head and neck 4 Published online Jul How to cite this article.

Pediatr Clin North Am. Open in a separate window. Some of these diseases develop mainly after bone marrow transplantation. Squamous cell carcinoma occurs with greater frequency in the region of the head and neck, particularly after bone marrow transplantation 17 – 18with the tongue being the site of preference.

J Bras Patol Med Lab.

Marrow transplantation for Fanconi anemia with or without leukemic transformation: Can bioelectrical impedance analysis identify malnutrition in perioperative nutrition assessment? All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License.

This may be justified by dysfunction in calcium and urea absorption by the body.

Megaloblastic anemia Pernicious anemia. Squamous cell carcinomas after allogeneic bone marrow transplantation for aplastic anemia: Due to the increased susceptibility to the development of cancer in this specific population, it is important for the dentist to know the common oral manifestations and potentially cancerous lesions, in order to make an early diagnosis in individuals with Fanconi Anemia.