Background: There is a lack of studies on outcomes in cystic fibrosis
(CF) in children from developing countries like India. Identifying risk
factors for mortality may help identify the high-risk group and plan
policy management of such patients. Objective: To determine the factors
associated with outcomes among Indian children with CF. Design:
Retrospective analysis of data collected from January 2010 to Dec 2020.
Setting: Tertiary care hospital in Northern India. Participants:
Children diagnosed with CF during the study period. Methods: We
extracted data related to demography, clinical features, laboratory data
and outcome from children’s medical records with CF. Bivariate and
multivariate analysis was performed to identify variables associated
with mortality. Results: We enrolled 178 children, and there were 32
(18.0%) deaths. Significant factors associated with mortality included
history of neonatal complications; hazard ratio (HR): 8.5 (95% CI, 3.0
- 23.9, p < 0.001), low Z-scores for body mass index (BMI) at
the time of diagnosis; HR: 7.1 (95% CI 2.3 - 22.0, p <
0.001), FEV1/FVC at the time of diagnosis; HR: 5.1 (95% CI, 1.65 -
15.4, p-value < 0.004), and FEV1 25-75; HR: 3.6 (95% CI, 1.1-
11.8, p-value = 0.03). Conclusions: Factors associated with increased
risk of mortality included presence of neonatal complications, low BMI
and lower pulmonary function test results. Low BMI and low PFT indices
are modifiable and possibly can be improved by early diagnosis. A
new-born screening test may help in early diagnosis and identification
of the neonatal problem of CF.
Improved early diagnosis of Pseudomonas aeruginosa by real-time PCR to prevent chronic colonisation in a paediatric cystic fibrosis population
