Cystic fibrosis: predictors of accelerated decline and distribution of disease in 230 patients.

Abstract

OBJECTIVE. Thepurpose ofthisstudy wastodetermine predictors ofaccelerated deterioration in radiographic manifestations of cystic fibrosis. The incidence and distribution of fo cally accentuated disease were also studied. MATERIALSAND METHODS. From230 patients, 3038chest radiographs werescored using the Brasfield system. Scores were plotted against age, and a single age-based se verity curve was created. Specific observations (at least one episode in the first 5 years of life of air trapping, linear markings, nodular cystic lesions, or large lesions) were assessedto de termine predictors of accelerateddecline in scorescompared with the aggregatescoresplotted in the age-based severity curve. Specific observations were noted as present or absent and graded as to severity. A specific observation was counted as present if seen on at least one oc casion. (The number of occasions on which the observation was made did not affect statistical analysis.) We also evaluated the distribution of lung disease by assessing the severity and na ture of disease through specific lobar distribution. RESULTS. Malesshowed aslightly greater rateofradiologic decline. Earlydevelopmentof air trapping or bronchiectasis was associated with an accelerated rate of decline over time.Lobe-dominant diseaseoccurred in one third of all images and in two thirds of the patients. It varied with age in its incidence, location, and etiology. (Fig. 1). This curve mimics the de dine in pulmonary function studies seen in patientswith cysticfibrosis[2â€"4]. This simi larity suggests that pulmonary function stud ies and radiographic changes represent parallel physiologic and structural aspects of the evolving damage to the lung and that extrapolation of the rate of decline may be de rived from both types of studies [2â€"4].In this study we examined specific predictors of in creased rate of decline. The Brasfield system is traditionally used to follow the progression of disease in individual patients. The age based severity curve previously published [2] includes Brasfield scores of serial radiographs (n = 3038) performed on 230 patients. These data were used to develop a curve reflecting the cumulative rate of decline in Brasfield scoresfor the patientsstudied.The current study reports specific radiographic observa tions that are correlatedwith an accelerated rate of decline in that same population of pa tients with cystic fibrosis. It hasbeenreportedthatcysticfibrosisis a disease that predominately affects the upper lobes of the lungs [5â€"7].To examine this con cept, we assessed lobe-dominantdiseaseto determine the incidence and distribution of focally accentuated abnormalities. CONCLUSION. Hyperinflation orbronchiectasis thatoccurs Materials and Methods PatientsAll 456 patients in the cystic fibrosis clinic A power calculationindicatedthatto develop data with 95% confidence limits of one Brasfield point across the age range, 3000 radiographs would be needed. Patients were therefore sequen

EXPOSURE TO AIR POLLUTION IS ASSOCIATED WITH LUNG HYPERINFLATION IN HEALTHY CHILDREN AND ADOLESCENTS IN SOUTHWEST MEXICO CITY: A Pilot Study

“…Especially in children, minor amounts of edema, mucus, or inflammatory processes may markedly narrow bronchioles; this is followed by an increment in airway resistance and production of a ball-valve effect, resulting in air trapping and lung hyperexpansion (Griscom et al, 1978;Tercier, 1983). Hyperinflation has been described in premature infants (Desmond et al, 1986;Giffin et al, 1997) and cystic fibrosis children (Grum & Lynch, 1992;Cleveland et al, 1998;Desmond et al, 1986;Marchant et al, 1994;Guignon et al, 1995), in the immotile cilia syndrome (Nadel et al, 1985), in children with liver disease due to alpha-1-antitrypsin deficiency (Hird et al, 1991), in bronchopulmonar y dysplasia (Breysem et al, 1997), in infants with large left-to-right shunts (Markowitz et al, 1988), in childhood pneumonia hyperinflation-diagnosed by chest x-ray before age 5 yr-is associated with accelerated radiographic deterioration over time (Cleveland et al, 1998). In adults, chronic overdistention is associated with a reduction in the elastic properties of the lung and can lead to structural abnormalities (Gold et al, 1967).…”

“…The other radiologist was blinded to the study, but had knowledge of the country of origin of the subjects (C.J.C.). Each set of radiographs was scored by each radiologist independently.The Brasfield scoring system was used because both readers used the scoring system daily in interpretation of chest radiographs for cystic fibrosis, and reading consistency among radiologists has already been established (Brasfield et al, 1980;Grum & Lynch, 1992;Cleveland et al, 1998). For analysis of the x-ray interpretation, only the score given for hyperinflation was used.…”

Fibrosis quística en adultos: acuerdos inter e intraobservador para las escalas de puntuación de Brasfield y Chrispin-Norman en la radiografía de tórax y relación con datos clínicos y espirométricos

“…Hemos estudiado a 24 pacientes, número similar al incluido en el estudio de Terheggen et al 11 , pero éste se realizó en población pediátrica. En la bibliografía hay pocos trabajos realizados en pacientes adultos, ya que la FQ suele considerarse una enfermedad infantil 12,[18][19][20] .…”

“…Se trata de un estudio transversal, sin un seguimiento en el tiempo para comprobar cómo progresaba el daño pulmonar. Esto constituye una de las limitaciones de nuestro estudio, y sería interesante estudiar la variación de la puntuación de los sistemas de clasificación de la radiografía de tórax y de los datos funcionales espirométricos en el seguimiento de los pacientes como en el estudio realizado por Cleveland et al 19,20 .…”

Lack of correlation between pulmonary disease and cystic fibrosis transmembrane conductance regulator dysfunction in cystic fibrosis: a case report

“…Except during two endobronchial exacerbations each, FEV 1 is >80% predicted, consistent with the top quartile and normal lung function for age according to the Epidemiological Study of Cystic Fibrosis (ESCF) classification [9]. The brothers had sequential chest X-rays (CXRs) scored by the Brasfield system [10] with sequential FEV 1 and FVC, by a predictive scoring system developed from a large CF cohort [11,12] One brother had 14 CXRs over a 12.5-year period (age four months to 12 years and nine months); the other had 20 CXRs over 18 years (age one month to 18 years, three months). The brothers' aggregate decline in CXR score was 0.027%/year, FEV 1 -0.018%/year, and FVC -0.012/year, compared with aggregate declines for 57 patients homozygous for the ΔF508 CFTR mutation in the same cohort of CXR scores -0.065%/year, FEV 1 -0.045%/year and FVC -0.044%/year (unpublished data).…”