Clinical outcome in relation to care in centres specialising in cystic fibrosis: cross sectional study

BMJ 1998;316:1771-1775 ( 13 June )

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Ravi Mahadeva, Anglia and Oxford research fellow, ^a Kevin Webb, consultant physician, ^b Roger C Westerbeek, biostatistician, ^c Nick R Carroll, senior registrar, ^d Mary E Dodd, senior physiotherapist, ^b Diana Bilton, consultant physician, ^e David A Lomas, honorary consultant physician. ^a

^a Department of Haematology, University of Cambridge, Medical Research Council Centre, Cambridge CB2 2QH, ^b Bradbury Cystic Fibrosis Unit, Wythenshawe Hospital NHS Trust, Manchester M23 9LT, ^c Medical Research Council Biostatistics Unit, University of Cambridge CB2 2QQ, ^d Addenbrooke's Hospital NHS Trust, Cambridge CB2 2QQ, ^e Cystic Fibrosis Unit, Papworth Hospital NHS Trust, Papworth Everard, Cambridge CB3 8RE

Correspondence to: Dr Bilton

Objectives: To assess the effect on clinical outcome of managing paediatric and adult patients with cystic fibrosisat specialised cystic fibrosis centres.
Design: Cross sectional study.
Setting: Two adult cystic fibrosis centres in the United Kingdom.
Subjects: Patients from an adult cystic fibrosis centre in Manchester were subdivided into those who had received continuouscare from paediatric and adult cystic fibrosis centres (groupA), and those who had received paediatric care in a centre not specialising in cystic fibrosis followed by adult care in a cystic fibrosis centre (group B). Group C were referrals to the new adult cystic fibrosis centre in Cambridge who had received neither paediatric nor adult centre care for their cystic fibrosis.
Main outcome measures: Body mass index (weight (kg)/height (m²)), lung function (forced expiratory volume in one second (FEV₁percentage of predicted)), the Northern chest x ray film score,and age at colonisation with Pseudomonas aeruginosa.
Results: A prominent stepwise increase in body mass index was associated with increasing amounts of care at a cystic fibrosis centre; 18.3, 20.2, and 21.3 for groups C, B, and A respectively(P<0.001). Improved nutritional status was correlated with a higherFEV₁ and better (lower) chest x ray film scores; r=0.52 and $-$ 0.45respectively (P<0.001 for both).
Conclusion: These findings provide the first direct evidence that management of cystic fibrosis in paediatric and adult cystic fibrosis centres results in a better clinical outcome,and strongly supports the provision of these specialist services.

Key messages

Management of patients with cystic fibrosis in paediatric and adult cystic fibrosis centres results in an improved clinical outcome
Improved clinical outcome occurred in cystic fibrosis centres despite earlier and more frequent colonisation with Pseudomonas aeruginosa
Nutritional status is an important predictor of lung disease in cystic fibrosis

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