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.