Cystic Fibrosis – a Born to Die disease: Current Status in Bangladesh

Abstract

Cystic fibrosis (CF) an autosomal recessive (genetic) disorder caused by mutations in both copies of gene encoding for CF-transmembrane conductance regulator (CFTR) protein. This life-limiting disease remain common among the Caucasian population living in America, Europe, Australia and Asia though found in non-Caucasian, ethnic minorities, too. The incidence of CF, a life limiting genetic disorder is 1 in 2500 children born in UK, but less common in African-Americans (1/15 000) and Asian-Americans (1:31 000). According to Indian experience, because of widespread belief that CF does not occur in Indo-Bangladesh subcontinent, the disease is rarely suspected, and, even if it does, the diagnosis is not always confirmed due to poorly available facilities for diagnosis like ours.  CF is an autosomal recessive disorder caused by mutations in the CFTR gene, fails to move chloride ions to the cell surface. Reduced chloride secretion coupled with increased sodium and water reabsorption, creates thick and dehydrated mucus that plugs airways and ducts. Viscous mucus in the lungs causes chronic bacterial infection, persistent inflammation, and progressive bronchiectasis. Obstruction of pancreatic ducts prevents enzymes from reaching the intestines, causing exocrine pancreatic insufficiency, fat malabsorption, and malnutrition. Inability to reabsorb chloride leads to high salt content in sweat (the basis for the sweat chloride test). The gold standard test is sweat chloride test for the diagnosis of CF. 

Medivision J. of Med. and Health Sci. Vol 1(1), Jul 2026; p 52-53