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).