Introduction
Cystic fibrosis (CF) is an autosomal recessive monogenic disorder with a prevalence of around 3000 per 10,000 in the western world (1). CF, the commonest inherited life-limiting illness, was initially considered to be affecting the Caucasians only. With increasing awareness, many cases are being recognized in India; however, its exact prevalence is unknown. This condition is not recognised at many peripheral centres due to a lack of awareness and inaccessibility to diagnostic facilities. The children with CF in India are usually diagnosed late. Indian patients differ from their counterparts from the developed world in being frequently malnourished, having clinical evidence of fat-soluble vitamin deficiencies and more chances of being colonized with Pseudomonas (2,3). The mutation profile is also different, with a lower prevalence of ΔF508 (4). Management of CF in India is complex due to inadequately trained manpower, lack of financial support, limited availability and high cost of pharmacologic agents. The determinants of early death in Indian children with CF from limited small studies may include severe malnutrition, colonization with Pseudomonas at the time of diagnosis, more than four episodes of lower respiratory infection per year and age of onset of symptoms before two months of age (5,6).
Data are scarce regarding determinants of mortality in children with cystic fibrosis from developing countries like India. Identifying risk factors for mortality may help identify the high-risk group and plan management of such patients, specifically in a limited resource setting. We determined the factors associated with outcomes among Indian children with cystic fibrosis.