Predictors of mortality in children with cystic fibrosis in India

© 2021 Wiley Periodicals LLC..

BACKGROUND: There is a lack of studies on cystic fibrosis (CF) outcomes 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. We aimed to determine the factors associated with mortality among Indian children with CF.

METHODS: In this retrospective study, we extracted demography, clinical features, laboratory and outcome data from medical records of children 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. Median (IQR) z-score for body mass index (BMI) at last follow up was -1.5 (-2.7, -0.2) and -2.5 (-4.0, -1.6), p-value 0.039, in survived and deceased group respectively. Mean (SD) of % predicted of FEV1/FVC and FEV1 25-75 at the time of diagnosis in survived versus diseased group was 94.7 (24.1) versus 81.5 (19.1), p-value 0.063 and 56.1 (38.9) versus 45.7 (29.9), p-value 0.347, respectively. Significant factors associated with mortality included history of neonatal complications; hazard ratio (HR): 8.5 (95% confidence interval [CI]: 3.0-23.9, p < 0.001), low Z-scores for 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 < 0.004), and FEV1 25-75; HR: 3.6 (95% CI: 1.1-11.8, p = 0.03).

CONCLUSIONS: Factors associated with increased mortality risk included neonatal complications, low BMI, and lower pulmonary function test results. Low BMI and low PFT indices can be improved upon by timely treatment of respiratory infections, better nutrition, early diagnosis, and treatment. A newborn screening program may help in early diagnosis and identification of the neonatal problem of CF.

Medienart:

E-Artikel

Erscheinungsjahr:

2022

Erschienen:

2022

Enthalten in:

Zur Gesamtaufnahme - volume:57

Enthalten in:

Pediatric pulmonology - 57(2022), 3 vom: 01. März, Seite 648-654

Sprache:

Englisch

Beteiligte Personen:

Kaur, Hardeep [VerfasserIn]
Pandey, Shivam [VerfasserIn]
Jat, Kana R [VerfasserIn]
Lodha, Rakesh [VerfasserIn]
Kabra, S K [VerfasserIn]

Links:

Volltext

Themen:

Allergic bronchopulmonary aspergillosis
Asia
Children
Cystic fibrosis
India
Journal Article
Mortality predictors
Pulmonary function test
Shwachman-Kulczycki (SK) score

Anmerkungen:

Date Completed 18.04.2022

Date Revised 18.04.2022

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1002/ppul.25766

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM333634918