THE PROGNOSIS OF CYSTIC-FIBROSIS IN THE WESTERN CAPE REGION OF SOUTH-AFRICA

Objective: To study the prognosis of cystic fibrosis (CF) in South Afr ica. Methodology: Retrospective chart review of 102 children with CF o ver 20 years.Results: Survival at 18 years was 54% (95% confidence int ervals 30-79). Prognosis was not influenced by gender or genotype but survival was noted to be worse for Cape Coloured children than for Eur opean children. This appeared mainly to be due to the death of eight C ape Coloured infants, four of whom presented with oedema and anaemia, mimicking kwashiorkor. Infection with Pseudomonas aeruginosa occurred earlier in Cape Coloured children than European children (median, 1 vs 4 years) and they had a worse 5-year survival (56 vs 89%, P < 0.05) a fter infection. Of the 40 children born in the second decade studied, six Coloured and no European children died. Conclusions: Ethnic differ ences in prognosis exist for children with CF in South Africa and are probably related to underrecognition of CF and the socio-economic stat us of some patients.