Results
We enrolled 178 children (114, 64.0% boys) with cystic fibrosis in the study. There was a total of 32 deaths (18.0 %). Out of these, 12 deaths happened in boys (37.5%) and 20 in girls (62.5%). Detailed demographic, clinical details and laboratory findings of the study population are mentioned in table 1. The proportion of female was more in the died group. Kaplan Meier survival analysis (Figure 1) was suggestive of girls’ shorter life span than boys. Age of onset of symptoms, registration and diagnosis was significantly high in the died group. The number of hospitalizations before diagnosis and between diagnosis and death or last follow-up were more in the dead group. Z-score for weight and BMI and SK score were not different between the groups at diagnosis, but all were low in the died group at the last follow up visit or death (Table 1). Among laboratory parameters, total leukocyte count and age at first colonization were more in the died group. History of consanguinity was present in 18 (10.1%) children; 13 in survived group and 5 in the died group. Twenty (11.2%) children had a family history of CF, and 47 (26.4%) children had a history of sibling death.
Airway was colonised in a total of 116 (65.73%) children, of which 24 (21.36%) children had died during the study. The most common aetiological agent for first colonisation was Pseudomonas in 91 (78.5%) children, followed by staphylococcus in 22 (18.9%) and Burkholderia species in 3 (2.5%) children. The most common aetiological agent at last colonisation was Pseudomonas in 64 (55.5%) children, followed by staphylococcus in 4 (3.4%) children. Around 48 (41.4%) children had no colonisation at the end of the study period.
We tested for two common mutations, and these were identified in 37 children (20.8%). The most common mutation was heterozygous delta F 508 (53.8%), followed by 3849+10 kb (25.64%) and homozygous delta F 508 in 7.7% of children.
CF-related complications are mentioned in table 2. The mean ± SD age of developing ABPA was 12.1±3.9 years. The average value of total IgE was 2369.23 IU/L, and the average value of aspergillus specific IgE was 36.78 IU/L. A total of 41 children (29 survived, 12 died) had elevated total IgE. The mean ± SD age of developing distal intestinal obstruction syndrome (DIOS) was 12.7±9.5 years. Mean ±SD age of developing haemoptysis was 15.2±1.8 years. More proportion of CF children who died had ABPA and DIOS (Table 2).