n South African Journal of Child Health - Rate of pulmonary function decline in South African children with cystic fibrosis

Volume 3, Issue 3
  • ISSN : 1994-3032
  • E-ISSN: 1999-7671



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

. To investigate the average annual rates of pulmonary function decline in South African children with CF from 1999 to 2006.
: The medical records and best PFT over 3-monthly intervals of children attending the CF clinic at Red Cross War Memorial Children's Hospital, Cape Town, were retrospectively reviewed and analysed using the mixed model regression method.
A total of 1 139 PFT were recorded on 79 patients, with a median (interquartile range) of 14 (6 - 21) PFTs per patient. The mean (standard error) forced expiratory volume in 1 second (FEV) at age 6 years was estimated at 73.83 (3.34) per cent predicted with an FEV decline of 0.23 (0.43)% per annum. FEV at age 6 was affected by age at CF diagnosis, genotype, and year of birth. Rate of FEV decline was significantly affected by colonisation and genotype.
. Although FEV at age 6 years was low compared with developed countries, the annual rate of FEV decline in South African children with CF was minimal, setting the scene for improved survival in this population.

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