CYSTIC-FIBROSIS IN SAUDI-ARABIA - COMMON AND RARE PRESENTATIONS

The clinical presentations of 12 children with cystic fibrosis seen in King Khalid University Hospital are presented. Ten were of Saudi orig in and the other two were African. The mean age of onset of symptoms w as 2.3 months, and the mean age at diagnosis was 14.3 months (range 3- 48 months). Seven children were boys and five were girls. All children presented with growth failure, recurrent chest infection and chronic diarrhoea. The parents of 83% of our cases were first-degree relatives . Pseudo-Bartter syndrome was seen in eight children. Sixty-seven per cent of our cases were colonized with Pseudomonas aeruginosa by the ti me of diagnosis, despite their young age (mean 7 months). Peripheral n europathy secondary to vitamin E deficiency, meconium ileus, nasal pol yps and gall-stones were present, each in one case. On follow-up, one child died and the other 11 are still alive. We concluded that cystic fibrosis is not rare in Saudi Arabia and that increased awareness of t he disease is needed to avoid delay in diagnosis. Efforts should be ma de to prevent early colonization by Pseudomonas aeruginosa.