DefinePK

Abstract

Background: Cystic fibrosis (CF) in children is  an inherited disorder that causes thick mucus to build up in body and leads  to  damage  various organs, especially the digestive system and lungs. The predicted incidence of cystic fibrosis fluctuates among ethnic groups, with Caucasians having the greatest prevalence
Objective: The objective of the study was to explore the clinical and laboratory profile of children with Cystic Fibrosis.
Methodology: This retrospective study was carried out at the Department of Paediatric Medicine, Qazi Hussain Ahmad medical complex Nowshera from August 2021 to January 2022 after obtaining permission from the ethical committee of the institute.  Admitted Participants diagnosed with cystic fibrosis based on the positive sweat chloride test and clinical characteristics from birth to fifteen years of both gender were enrolled in this study. Sweat chloride levels above 60 mmol per litre were considered CF Positive. Analyses of the identified Delta F-508 mutations were observed. Sputum culture results and HRCT chest findings from laboratory and radiographic studies were recorded. Analysis of the data was done with SPSS version 20.
Results: A total of 47 children were enrolled in the study according to the inclusion criteria. The most prevalent symptom among the study cases was chronic cough (70%). Respiratory features that were most common in our study on chest HRCT included bronchiectasis and hyperinflation (38%). Radiological examination evaluated 27% consolidation. Culture investigation showed that the most prevalent bacteria was Pseudomonas (27%). Gastroenterological features like steatorrhea were seen in 6 (12.7%) patients.
Conclusion: In children diagnosed with cystic fibrosis, respiratory symptoms were found to be more prevalent compared to gastrointestinal symptoms. When analyzing their chest CT scans, nearly half of the examined cases showed bronchiectatic changes. Among those investigated, 27% exhibited pseudomonas colonization in their airways. In the present study, the delta F-508 mutation was identified as rare.