Articles

Rate of pulmonary function decline in South African children with cystic fibrosis.

Brenda May Morrow, Andrew C Argent, Greg Distiller, Heather J Zar, Anthony TR Westwood

Abstract


Background: Pulmonary function tests (PFT) objectively measure the extent and progression of cystic fibrosis (CF) lung disease. The rate of PFT decline has not been studied in developing countries.

Objectives: This study investigated the average annual rates of pulmonary function decline in South African children with cystic fibrosis (CF) from 1999 to 2006.

Design: The individual medical records and best PFT over three-monthly intervals were retrospectively reviewed and analysed using the mixed model regression method.

Setting: CF clinic at Red Cross War Memorial Children’s Hospital, Cape Town, South Africa – a tertiary level paediatric teaching hospital.

Subjects: 79 children over five years of age with CF.

Primary outcome measures: Estimated mean forced expiratory volume in one second (FEV1) at six years of age and average annual rate of FEV1 decline.

Results: 1139 PFT were recorded, with a median (IQR) of 14 (6 – 21) PFT per patient. With no covariates in the model, the estimated mean (SE) FEV1 at age six years was 73.83 (3.34) percent predicted and the annual FEV1 decline was 0.23 (0.43)%. FEV1 at age six was affected by age at CF diagnosis, genotype, and year of birth. Rate of FEV1 decline was significantly affected by Pseudomonas aeruginosa colonisation and genotype.

Conclusions: Although FEV1 at age six years was low compared to developed countries, the annual rate of FEV1 decline in South African children with CF was minimal setting the scene for improved survival in this population.

Authors' affiliations

Brenda May Morrow, School of Child and Adolescent Health, University of Cape Town

Andrew C Argent, School of Child and Adolescent Health, University of Cape Town and Red Cross War Memorial Children's Hospital

Greg Distiller, Department of Statistical Sciences, University of Cape Town

Heather J Zar, School of Child and Adolescent Health, University of Cape Town

Anthony TR Westwood, School of Child and Adolescent Health, University of Cape Town and Red Cross War Memorial Children's Hospital

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Keywords

cystic fibrosis, lung function, children

Cite this article

South African Journal of Child Health 2009;3(3):73.

Article History

Date submitted: 2009-07-23
Date published: 2009-11-09

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