loading page

  • +2
  • Hardeep Kaur,
  • Shivam Pandey,
  • Kana Jat,
  • Sushil Kabra
Hardeep Kaur
All India Institute of Medical Sciences
Author Profile
Shivam Pandey
All India Institute of Medical Sciences
Author Profile
Kana Jat
All India Institute of Medical Sciences
Author Profile
Author Profile
Sushil Kabra
All India Institute of Medical Sciences
Author Profile


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.

Peer review status:UNDER REVIEW

07 Jun 2021Submitted to Pediatric Pulmonology
18 Jun 2021Submission Checks Completed
18 Jun 2021Assigned to Editor
20 Jun 2021Reviewer(s) Assigned
02 Jul 2021Review(s) Completed, Editorial Evaluation Pending
08 Jul 2021Editorial Decision: Revise Major
15 Sep 20211st Revision Received
16 Sep 2021Assigned to Editor
16 Sep 2021Submission Checks Completed
16 Sep 2021Reviewer(s) Assigned