Cystic fibrosisCystic fibrosis (mucoviscidosis) is a hereditary disease affecting the exocrine (mucus) glands of the lungs, liver, pancreas and intestines, causing progressive disability due to multisystem failure. It has an autosomal recessive pattern of inheritance.Pathogenesis The basic defect in CF is a mutation in the cystic fibrosis transmembrane regulator (CFTR) gene that regulates chloride conductance channel in the outer membrane of exocrine glands. Mutation in the CFTR gene                  ↓ Dysfunction of chloride conductance channel of exocrine glands                 ↓ Lack of chloride conduction                 ↓ Secretion of glands becomes more viscous and thick                ↓ Blockade of respiratory tract and ducts of exocrine glands by thick viscid mucus                ↓ Manifestations of CF Clinical manifestations 1) Lungs and sinus diseaseSinusitis       Bronchiectasis   Lower respiratory tract infection Nasal polyps   Chronic lung disease   Pulmonary hypertension → can cause heart failure            Allergic bronchopulmonary aspergillosis           2)    GIT, Liver and pancreasMeconium ileus Rectal prolapse Malabsorption Pancreatitis      Constipation    Intussusception    Meconium peritonitis   Bile duct obstruction and biliary cirrhosis           3) Endocrine and growthDiabetes       Osteoporosis     Poor growth                                         4) InfertilityIn men due to the absence of vas deference   In women due to thick cervical mucus.