Clinical Profile of Children With Cystic Fibrosis Surviving Through Adolescence and Beyond

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.