WS3.6 The CF-ABLE score: a novel clinical prediction tool in cystic fibrosis

Autor: Cormac McCarthy, Noel G. McElvaney, B.D. Dimitrov, Cedric Gunaratnam
Rok vydání: 2012
Předmět:
Zdroj: Journal of Cystic Fibrosis. 11
ISSN: 1569-1993
DOI: 10.1016/s1569-1993(12)60024-1
Popis: Introduction: Adult diagnosed patients represent a unique population that differ in their clinical, genetic and prognostic characteristics compared to those diagnosed in childhood. With a different spectrum of CFTR mutations, infections and organ involvement response to standard CF treatments is unknown. Aims: To determine clinical status at diagnosis and assess response to CF centre treatment over a 4 yr period. Methods: All patients diagnosed after 16 yrs of age (2005 to 2011) were included. Clinical data at diagnosis were collected from electronic patient records (EMIS®). Best FEV1, weight and BMI for each subsequent 12 month interval recorded. Results: 20 patients (12 male, 75% PS, median (range) age at diagnosis 30(17−60) yrs, BMI 21.8(16.7–31.9), FEV1 66(43–101) and FVC 87(62–106)% predicted) were included. 10 patients were diagnosed due to bronchiectasis and 10 nonrespiratory [infertility (6), family history (3) and malabsorption (1)]. Median BMI at diagnosis was greater in those with PS vs. PI (22.0 vs. 19.2, p< 0.01). BMI increased from 21.8 at diagnosis to 24.6 at yr 4. Median weight gain over 4 yrs was 6.25 kg (5 kg PS vs. 12.3 kg PI, p< 0.005). FEV1 was greater at diagnosis in those with a non-respiratory diagnosis (93 vs. 60% predicted, p< 0.005). In patients with bronchiectasis FEV1 increased from 60 to 75% predicted at 1 yr; this increase was maintained at 4 yrs (78% predicted). No increase in FEV1 in those with a non-respiratory diagnosis. Conclusions: Adult diagnosed CF patients do demonstrate significant improvements in both nutritional and respiratory status in response to CF centre treatment and care, especially those with PI and bronchiectasis.
Databáze: OpenAIRE
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