MOHAMMED RIZWANUL AHSAN1, HOSSAIN SHAHID KAMRUL ALAM2, AHMED RASHEDUL HASAN3,MIRZA ZIAUL ISLAM4, AHMED HOSSAIN5
Abstract
Cystic fibrosis (CF) is said to be the most common lethal inherited disease of the
white population. It affects the exocrine glands of body, primarily of the gastrointestinal
and respiratory system. CF is caused by one of a large numbers of mutations of the
gene for a protein called the cystic fibrosis transmembrane conductance regulator
(CFTR). This CFTR regulates chloride and sodium transport across epithelial
membranes. The main complications involve in the lungs, with damage to the small
and large airways by chronic and recurrent bacterial infections. Other major
consequences include pancreatic malfunction, leading to malabsorption of nutrients
and vitamins with consequent impaired growth and development and in older patient
diabetes. Diagnosis is usually done by sweat test or identification of two CF-causing
mutations in patients with characteristics symptoms. Treatment is supportive through
aggressive multidisciplinary care. The prognosis of CF has improved due to earlier
diagnosis through screening, better treatment and access to health care. Although
CF is a rare disease in Bangladesh, but its possibility should be kept in mind in
appropriate circumstances.
Key words: Cystic fibrosis, inherited disease, cystic fibrosis transmembrane
conductance regulator (CFTR), Sweat test