CF was a relatively overlooked problem in Bangladesh. Only recently was a multicenter study of 94 cases over a period of 17 years between 2000 and 2017 published by Kabir et al  in 2020. Given the scarcity of information regarding children with CF in the country, it was reasonable to continue the exploration of demographic and clinical features of CF patients. Additionally, an examination of the patients’ characteristics across outcomes would identify important conditions to be addressed during management. Hence, this study was aimed.
The average age at diagnosis of our participants was lower than that found by Kabir et al  in Bangladesh, Kabra et al  in India, and Aziz et al  in Pakistan. Increased availability of diagnostic facilities might have lowered the age of diagnosis of CF. We found a slightly higher proportion of males, in conformity with previous studies[13, 15, 16]. However, unlike previous studies , we did not note any increased risk of case fatality among female patients.
Out of 50 children with CF included in the study, 16% died. This is higher than the previous finding by Kabir et al , who reported 11 deaths out of 95 patients (case fatality 11.5%). Additionally, this figure of childhood case fatality is much higher than in Western countries equipped with better management resources .
As an autosomal recessive genetic disorder , a family history of CF and consanguinity of marriage between parents are common findings in CF patients. We noted a family history in nearly three-fifths (57.1%) of patients and consanguinity of marriage in more than two-fifths (41.7%). In contrast, consanguinity was found in 22%, 55.8%, and 15% of cases by Kabir et al , Aziz et al , and Kabra et al , respectively. These differences in distribution agree with the global distribution of consanguinity, where an important cluster of countries shows a high level of consanguinity and includes mostly Muslim majority areas . However, the distribution might vary due to other factors, including religion, race, ethnicity, and sociocultural factors.
We noted a very high prevalence of malnutrition in the form of underweight, stunting, and wasting among our children with CF, which conforms with other studies [15, 16]. All age groups and both alive and dead children shared a statistically similar proportion of malnutrition. Malnutrition is common in cystic fibrosis, and the cause of growth failure and malnutrition is multifactorial, including chronic inflammation .
The most frequent clinical features were cough with purulent sputum or hemoptysis and failure to thrive among the study participants. Additionally, malnutrition, short stature, digital clubbing, and bronchiectasis were predominant signs. Cough occurs mainly due to repeated infection of the airway. Hence, recurrent respiratory infections and failure to thrive are the most commonly reported symptoms in studies involving CF[15, 16, 19–21]. Children with CF frequently pass bulky offensive stools due to fat malabsorption from the gut, and malnutrition occurs due to inadequate absorption of proteins, fats, and fat-soluble vitamins because of pancreatic insufficiency . All the symptoms occur across all age groups. Interestingly, however, we noted an association of case fatality with the presence of digital clubbing, bronchiectasis, pulmonary hypertension, allergic bronchopulmonary aspergillosis, pancreatic insufficiency, abnormal liver tests, and honeycomb appearance on chest X-ray. All these features are suggestive of advanced disease . In particular, the presence of bronchiectasis and honeycombing indicates underlying chronic inflammation and repeated infection. Management of pancreatic insufficiency requires multidisciplinary, carefully tailored strategies, which are often not possible in resource-poor settings. Hence, a combination of explored and unexplored factors might be responsible for premature deaths in CF among children in our setting.
Our study was limited by the fact that it was a single-center study with a small sample of cases. Genetic studies were not available for most of the patients due to economic problems or a lack of adequate facilities. Long-term follow-up of the patients was beyond the scope of the study. However, the study presented a detailed examination of sociodemographic and clinical features of children with CF in the country with a comparison across different outcomes, which will aid in the diagnosis and management of CF cases.