Clinical Profile of Children With Cystic Fibrosis Surviving Through Adolescence and Beyond
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Date
2022-01
Journal Title
Journal ISSN
Volume Title
Publisher
Indian Academy of Pediatrics
Abstract
Objective: To document morbidities in adolescents with cystic fibrosis (CF) from India. Methods: Details of children with cystic fibrosis surviving beyond 15 years of age were extracted from hospital records, and analyzed. Results: 43 children [Median (IQR) age 18.7 (17, 20.6) years, were enrolled. Median (IQR) body mass index was 15.82 (13.5, 19.05) kg/m2. Pseudomonas species were isolated from respiratory specimens of 34 (79%) adolescents. Allergic bronchopulmonary aspergillosis (ABPA) and Cystic fibrosis-related diabetes (CFRD) were seen in 12 (28%) and 11 (26%) patients, respectively. Conjugated hyperbilirubinemia and distal intestinal obstruction syndrome (DIOS) were diagnosed in 15 (35%) and 6 (14%) children, respectively. Pseudomonas species colonization (P=0.04) and multiple pulmonary exacerbations in last one year (P<0.001) were significant predictors of FEV1% predicted. Conclusion: Malnutrition, chronic airway colonization, ABPA, CFRD, conjugated hyperbilirubinemia and DIOS are morbidities observed in adolescents with CF in India. The data support the need for early screening of CF-associated morbidities.
Description
Keywords
Allergic bronchopulmonary aspergillosis, Colonization, Cystic fibrosisrelated diabetes, Pseudomonas.
Citation
Kumar Arvind, Aggarwal Bhawana, Bamal Princy, Jat Kana Ram, Lodha Rakesh, Kabra SK. Clinical Profile of Children With Cystic Fibrosis Surviving Through Adolescence and Beyond. Indian Pediatrics. 2022 Jan; 59(1): 43-45