A possibilidade de avaliar a evolução da doença pulmonar nos pacientes com FC, permitiu que novas intervenções terapêuticas fossem agregadas ao tratamento desta doença, o que determinou uma melhora significativa da sobrevida nos últimos vinte anos, mesmo no Brasil.
Ainda hoje o diagnóstico da FC sucinta muita dúvida, tendo em apreço a grande variabilidade clínica de sua apresentação, justificada pelo grande número de mutações identificadas no seu gene.
O estudo de uma população miscigenada agregou ao nosso trabalho uma perspectiva de trazerem dados ainda não descritos na literatura da América Latina em relação à FC.
As boas correlações encontradas entre escores de tomografia com testes de função pulmonar, sugerem que, nesta população, a monitorização da progressão da doença pulmonar poderia ser realizada por tomografia ou pela função pulmonar. A reavaliação futura destes pacientes poderá trazer contribuições no sentido de validar as observações derivadas do presente estudo.
6 CONCLUSÃO
As alterações tomográficas mais prevalentes nesta população foram o espessamento de paredes brônquicas, as bronquiectasias e o padrão de perfusão em mosaico. Uma forte correlação foi estabelecida entre escores totais de TCAR com o VEF1; o FEF25%-75% apresentou uma boa correlação com os escores de TCAR, o
mesmo não se observando com a CVF. Aspectos avaliados de forma isolada à TCAR também apresentaram boas correlações com o VEF1 e com o FEF25%-75% . Os
resultados deste trabalho sugerem que tanto a tomografia quanto a função pulmonar podem ser utilizadas como marcadores para o acompanhamento de pacientes com FC, no nosso meio.
7 ANEXOS
Anexo 3 – Informções gerais dos pacientes
Pacientes Idade Sexo Cor Escore TC VEF1 CVF FEF25%-75%
01 6 M ñ-branco 26 88 85 73 02 7 M ñ-branco 20 82 94 50 03 20 M branco 8 38 42 18 04 15 M ñ-branco 23 82 100 42 05 8 M ñ-branco 23 82 78 80 06 9 F ñ-branco 25 50 75 21 07 7 M ñ-branco 23 92 82 108 08 10 F ñ-branco 10 49 44 61 09 11 M ñ-branco 13 45 66 19 10 10 M ñ-branco 14 67 66 49 11 10 F branco 8 45 94 37 12 24 M branco 11 28 37 9 13 24 M ñ-branco 11 41 59 49 14 6 M ñ-branco 27 115 112 97 15 11 M ñ-branco 12 27 24 22 16 10 M ñ-branco 24 73 73 59 17 9 F branco 24 88 86 68 18 6 M ñ-branco 27 70 76 48 19 7 F ñ-branco 24 86 83 67 20 11 M branco 21 66 71 45 21 8 M ñ-branco 27 102 99 81 22 6 F branco 26 82 73 104 23 10 M branco 27 101 94 91 24 9 M ñ-branco 22 72 70 67 25 8 F ñ-branco 27 86 78 96 26 9 M ñ-branco 23 73 84 28 27 9 M branco 16 57 101 8 28 13 F ñ-branco 26 90 83 82 29 25 M ñ-branco 13 71 88 29 30 13 F ñ-branco 21 89 91 47 31 6 M branco 27 91 82 105 32 11 M ñ-branco 15 71 71 63 33 10 F ñ-branco 22 106 97 95 34 14 M ñ-branco 27 112 106 98 35 25 M ñ-branco 7 31 47 13
M = masculino; F = feminino; ñ = não; TC = tomografia computadorizada; VEF1 = volume expiratório forçado no
primeiro segundo; CVF = capacidade vital forçada e FEF25%-75% = fluxo expiratório forçado entre 25% e 75% da
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Abstract
Purpose: Evaluate pulmonary manifestations of Cystic Fibrosis (CF) on high resolution
computed tomography (HRCT), based on a system of scores, and correlate these results with pulmonary function tests (PFT) in patients from a specialized center in CF in Bahia, an area with a highly mixed population. Methods: A cross sectional survey from June 2004 to March 2006, prospectively examined 35 consecutive patients with CF (10 female patients, 25 male patients; mean age, 11,3; age range, 6 to 25 years) with pulmonary function tests (PFT) and HRCT. Any patient with clinical signs of an active acute pulmonary process was excluded from the study. Non-whites accounted for 77% of this serie. The computed tomography scans were analyzed by two independet radiologists. The scores of tomography were correlated with the forced expiratory volume in the first second (FEV1), forced vital capacity (FVC) and forced expiratory flow between 25% and 75% of forced vital capacity (FEF25%-75%). After an interval of three months, the radiologists made a second read for definition of intra-observer concordance. Results: Peribronchial wall thickening (57.14%), bronchiectasis (54.28%) and mosaic perfusion (51.43%) were the most frequently tomography finding in this population. A strong correlation was established between total scores of HRCT and FEV1 (r = 0.7808, p <0.0001). The FEF25% -75%had a good correlation with the scores of HRCT (r = 0.6981, p <0.00001), but not with FCV (r = 0.511, p <0.001). When evaluated separately, structural changes described by HRCT, also show good correlation with the FEV1 and with FEF25%-75%. The inter and intra-observers agreements were also excellent. Conclusions: The HRCT findings most common in this population were peribronchial wall thickening, bronchiectasis, and mosaic perfusion. A strong correlation was established between total scores of HRCT with FEV1 and FEF25%-75%, but not with FCV. Structural changes described by HRCT, also show good correlation with the FEV1 and FEF25%-75%. Our results suggest that either HRCT or PFT can be used to follow CF patients in this population.
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