cystic fibrosis

Cystic fibrosis also known as mucoviscidosis because this disease cause formation of viscous mucus in lungs, pancreas and also testes.

This disease is hereditary autosomal recessive disease. cystic fibrosis can cause restriction to breathing because the mucus obstruct the lung and also fill the airspace with mucus that is very hard to remove by coughing.

Cystic fibrosis occur when genetic that codes for CFTR(cystic fibrosis transmembrane regulator) is defect causing formation of abnormal CFTR. Abnormal CFTR cannot regulate the chloride ions out the cells causing chloride ions trap inside the cells. this causing disbalance of electric charge inside and outside the cells causing movement of cation mainly sodium ions into the cells and result in formation of salt. when sodium channel is move into the cells , it cause osmotic pressure changes resulting diffusion of water into the cells resulting in dehydration and thick mucus.

when there is thick mucus in lungs, lungs cannot be cleaned properly causing recurrent infections result in chronic pneumonia and formation of lung fibrosis and finally result in cor pulmonale whic is right heart failure.