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 hyper-bilirubinemia and DIOS are morbidities observed in adolescents with CF in India. The data support the need for early screening of CF-associated morbidities.