Clinico-epidemiological Characteristics of Children with Cystic Fibrosis: a Tertiary Care Experience
P K Sarker1, N Akand, S Tahura
1Professor Dr Probir Kumar Sarkar, Professor of Paediatric Respiratory Medicine (Pulmonology), Bangladesh Shishu Hospital & Institute, Dhaka, Bangladesh;
Insights
Pediatric cystic fibrosis (CF) in Bangladesh shows high rates of malnutrition and respiratory issues. Pulmonary hypertension is a common comorbidity, with mortality linked to severe complications.
Area of Science:
- Pediatrics
- Genetics
- Epidemiology
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Understanding the clinico-epidemiological profile of pediatric CF in Bangladesh is crucial for targeted interventions.
Purpose of the Study:
- To describe the clinico-epidemiological characteristics of pediatric cystic fibrosis (CF) cases in Bangladesh.
- To identify common symptoms, signs, comorbidities, and outcomes in this population.
Main Methods:
- An observational study included 50 pediatric patients (up to 14 years) with a clinical CF diagnosis.
- Data collected on demographics, medical history, symptoms, signs, and outcomes.
- Statistical analysis performed using SPSS 26.0.
Main Results:
- The majority of patients (86.0%) were stunted and underweight, with 54.0% experiencing wasting.
- Predominant symptoms included cough (100.0%), purulent sputum (100.0%), failure to thrive (98.0%), and bulky stools (86.0%).
- Pulmonary hypertension (48.0%) was the most common comorbidity; in-hospital mortality was 16.0%.
Conclusions:
- Pediatric CF in Bangladesh presents with significant undernutrition and respiratory symptoms.
- Failure to thrive is nearly universal.
- Pulmonary hypertension is a frequent complication, and severe signs correlate with higher mortality.
Abstract:
To describe the clinico-epidemiological characteristics of pediatric cystic fibrosis (CF) cases among Bangladeshi paediatric population was the objective of the study. This observational study included pediatric patients (up to 14 years of age) with a clinical diagnosis of CF. Data were collected within the period from April 2021 to October 2021. Informed assent was taken from the accompanying parent. Clinical and epidemiological characteristics were analyzed on the basis of demographic data, medical history, laboratory tests and outcome information. Collected data were analyzed statistical software, SPSS 26.0. A total of 50 patients (66.0% male) with a mean±SD age of 39.7±30.75 months were included. Twenty-eight patients (57.14%) had siblings with CF and 41.67% of parents had a history of consanguineous marriage. The majority of them were stunted (86.0%) and underweight (86.0%) and half of them had wasting (54.0%). Median disease duration was 12 months (range: 2-72). Cough (100.0%) and purulent sputum (100.0%) were the predominant respiratory symptoms, while failure to thrive (98.0%) and bulky offensive stools (86.0%) were prime gastrointestinal symptoms. Among the signs, malnutrition (94.0%), short stature (72.0%), digital clubbing (64.0%) and bronchiectasis (40.0%) were most frequent. Pulmonary hypertension (48.0%, n=24) was the most common comorbidity identified in the study participants. In hospital, mortality was 16.0% (n=8). Digital clubbing, bronchiectasis, pancreatic insufficiency and abnormal liver function tests were significantly higher in the patient who died. Children with cystic fibrosis most commonly present with under nutrition and respiratory symptoms. Failure to thrive was almost a global phenomenon. Pulmonary hypertension was the most common complication found in echocardiography.
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